Journal articles: 'PECome'

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Pernet, C., P. Pous, A. De La Fouchardiere, V. Laffay, J. M. Joujoux, and B. Guillot. "PECome cutané primitif." Annales de Dermatologie et de Vénéréologie 139, no. 12 (December 2012): B239. http://dx.doi.org/10.1016/j.annder.2012.10.423.

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Trotot-Voilliot, C., E. Laveine, P. Beurey, J. F. Cuny, J. L. Schmutz, and F. Truchetet. "Un PECome cutané primitif." Annales de Dermatologie et de Vénéréologie 140, no. 5 (May 2013): 373–77. http://dx.doi.org/10.1016/j.annder.2013.02.013.

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Audard, Virginie, Maryline Dorel-Le Théo, Marie-Dominique Trincard, Didier Charitanski, Valérie Barn Selmas, and Annick Vieillefond. "Un volumineux PECome para-rénal." Annales de Pathologie 24, no. 3 (June 2004): 271–73. http://dx.doi.org/10.1016/s0242-6498(04)93964-8.

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Elhaitamy, S., Z. Douhi, C. Bouhamdi, M. Soughi, S. Elloudi, H. Baybay, L. Tahiri, and F. Z. Mernissi. "Un PECome cutané primitif malin." Annales de Dermatologie et de Vénéréologie - FMC 3, no. 8 (December 2023): A170—A171. http://dx.doi.org/10.1016/j.fander.2023.09.315.

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Périgny, Martine, Olivier Larochelle, Pascal Hammel, Alain Sauvanet, Safi Dokmak, Jacques Belghiti, Philippe Ruszniewski, Valérie Vilgrain, Pierre Bedossa, and Anne Couvelard. "Tumeur des cellules épithélioïdes périvasculaires (PECome) du pancréas." Annales de Pathologie 28, no. 2 (April 2008): 138–42. http://dx.doi.org/10.1016/j.annpat.2007.11.005.

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Bléchet, Claire, Brigitte Arbeille, Anne de Muret, Julien Berhouet, Christian Bonnard, and Gonzague de Pinieux. "PECome des tissus mous : à propos d’un cas de localisation articulaire." Annales de Pathologie 27, no. 4 (September 2007): 313–16. http://dx.doi.org/10.1016/s0242-6498(07)73907-x.

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Sbrollini, Giulia, Alessandro Conti, Andrea Benedetto Galosi, Vito Lacetera, Rodolfo Montironi, Lorenzo Montesi, and Giovanni Muzzonigro. "Perivascular epithelioid cell tumor (PEC-ome) of the prostate: Ultrasound feature in case report." Archivio Italiano di Urologia e Andrologia 86, no. 4 (December 30, 2014): 393. http://dx.doi.org/10.4081/aiua.2014.4.393.

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Introduction: We describe a rare tumor arising from the prostate gland: Perivascular Epithelioid Cells tumor (PEC-ome). A 54-years old was treated for acute urinary retention with alpha-blockers at presentation due to benign prostate enlargement (65 cc) with asymmetric middle lobe and regular PSA (0.92 ng/ml). After 5 months, patient developed a second acute urinary retention episode and nodules in the left lung; he was treated with transurethral resection of the prostate and left lobectomy. Results: Histological examination of prostate and lung tissue gave the same diagnosis: leiomyosarcoma with atypical morphological features and patient was observed for 4 months. Considering the uncommon diagnosis, pathological review by the uro-pathologist at our Hospital was done. Additional immunohistochemistry was done and both tumors showed similar and typical features of metastatic PEC-ome (T1b N0 M1). Therefore a new staging showed local and distant progression with prostatic mass and small lung metastasis. Three cycles of Gemcitabine and Pazopanib were administered, but 2 months later a new urinary retention occurred, despite chemotherapy. Patient referred to our Hospital for salvage pelvic surgery with lymph node dissection. Final pathological diagnosis was PECome of the prostate stage pT4 pN0 R0 M1. Conclusions: PEC-ome is a rare but rapidly invasive mesothelial tumor with early metastatic potential. When this tumors originates from the fibromuscular stroma of the prostate it mimics benign prostatic enlargement and causes LUTS. Expert pathology aided by immunoisthochemistry is the cornerstone of diagnosis. There are no pathognomonic imaging on ultrasound or symptoms suggesting the presence of PECome in early stage. A multidisciplinary approach is necessary and radical surgery should be done to treat this aggressive cancer.

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Maran-Gonzalez, Aurélie, Pierre Baldet, and Valérie Costes. "PECome polypoïde du rectum : à propos d’un cas et revue de la littérature." Annales de Pathologie 31, no. 2 (April 2011): 102–7. http://dx.doi.org/10.1016/j.annpat.2010.07.005.

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Parfitt, Jeremy R., Anthony J. Bella, Jonathan I. Izawa, and Bret M. Wehrli. "Malignant Neoplasm of Perivascular Epithelioid Cells of the Liver." Archives of Pathology & Laboratory Medicine 130, no. 8 (August 1, 2006): 1219–22. http://dx.doi.org/10.5858/2006-130-1219-mnopec.

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Abstract Neoplasms of perivascular epithelioid cells (PEComas) have in common the coexpression of muscle and melanocytic immunohistochemical markers. Although this group includes entities with distinct clinical features, such as angiomyolipoma, clear cell sugar tumor of the lung, and lymphangioleiomyomatosis, similar tumors have been documented in an increasing diversity of locations. The term PEComa is now generally used in reference to these lesions that are not angiomyolipomas, clear cell sugar tumors, or lymphangioleiomyomatoses. While most reported PEComas have behaved in a benign fashion, malignant PEComas have occasionally been documented. We present a case of hepatic PEComa with benign histologic features, which nonetheless presented with metastases to multiple sites nearly 9 years later. This case represents the second documented malignant PEComa of the liver, as well as the longest follow-up of a surviving patient with a malignant PEComa, emphasizing both the need for criteria that more accurately predict the behavior of PEComas and the necessity of long-term follow-up of patients with PEComas.

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Tynski, Zofia, Way Chiang, and Albert Barrett. "An Inguinal Perivascular Epithelioid Cell Tumor Metastatic to the Orbit." Case Reports in Pathology 2018 (May 29, 2018): 1–5. http://dx.doi.org/10.1155/2018/5749421.

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Malignant PEComas are rare mesenchymal neoplasms. These tumors harbor distinct myomelanocytic phenotype. The PEComa family of tumors includes lymphangioleiomyomatosis, angiomyolipoma, clear cell sugar tumor of the lung, and myomelanocytic tumor of the falciparum ligament/ligamentum teres. PEComas have no known normal cell counterpart. Majority of PEComas are benign and occur predominantly in the middle-age women. These tumors are commonly encountered in the uterus. Herein, we report a 20-year-old woman with a left inguinal mass metastatic to orbit, brain, lumbar spine, and skin at presentation. To our knowledge, this is the first case of metastatic PEComa to the orbit. This is the third case of primary PEComa of the inguinal area.

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Wagner, Andrew J., Izabela Malinowska-Kolodziej, Jeffrey A. Morgan, Wei Qin, Christopher D. M. Fletcher, Natalie Vena, Azra H. Ligon, et al. "Clinical Activity of mTOR Inhibition With Sirolimus in Malignant Perivascular Epithelioid Cell Tumors: Targeting the Pathogenic Activation of mTORC1 in Tumors." Journal of Clinical Oncology 28, no. 5 (February 10, 2010): 835–40. http://dx.doi.org/10.1200/jco.2009.25.2981.

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PurposePerivascular epithelioid cell tumors (PEComas) represent a family of mesenchymal neoplasms, mechanistically linked through activation of the mTOR signaling pathway. There is no known effective therapy for PEComa, and the molecular pathophysiology of aberrant mTOR signaling provided us with a scientific rationale to target this pathway therapeutically. On this mechanistic basis, we treated three consecutive patients with metastatic PEComa with an oral mTOR inhibitor, sirolimus.Patients and MethodsPatients with advanced PEComa were treated with sirolimus and consented to retrospective collection of data from their medical records and analysis of archival tumor specimens. Tumor response was determined by computed tomography scans obtained at the clinical discretion of the treating physicians. Tumors were assessed for immunohistochemical evidence of mTORC1 activation and genetic evidence of alterations in TSC1 and TSC2.ResultsRadiographic responses to sirolimus were observed in all patients. PEComas demonstrated loss of TSC2 protein expression and evidence of baseline mTORC1 activation. Homozygous loss of TSC1 was identified in one PEComa.ConclusionInhibition of mTORC1, pathologically activated by loss of the TSC1/TSC2 tumor suppressor complex, is a rational mechanistic target for therapy in PEComas. The clinical activity of sirolimus in PEComa additionally strengthens the pathobiologic similarities linking PEComas to other neoplasms related to the tuberous sclerosis complex.

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Almousa, Maryam A., Yara A. Alnashwan, and Samir S. Amr. "Perivascular Epithelioid Cell Tumor (PEComa) of the Pancreas in a Patient with Ulcerative Colitis: A Case Report and Review of the Literature." Healthcare 11, no. 4 (February 12, 2023): 547. http://dx.doi.org/10.3390/healthcare11040547.

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Perivascular epithelioid cell tumors (PEComas) are mesenchymal tumors of peculiar cells that are focally associated with blood vessels, and generally have a distinctive bi-phenotypic expression of both smooth muscle and melanocytic markers. There are several entities in the PEComa family, including tumors that arise in the soft tissues and viscera. Frequently affected organs include the lungs (sugar tumors), uterus, broad ligament, colon, small bowel, liver, and pancreas. Ulcerative colitis (UC) has been associated with the development of tumors, especially colorectal and hepatobiliary carcinomas. Rare cases of UC have been reported in the PEComa family of tumors, but none in the pancreas. Here, we present a case study of a 27-year-old female patient with a history of UC who developed PEComa of the pancreas, a unique association that has not been previously reported. We also review reported cases of PEComas in the pancreas, as well as PEComas at all anatomic sites associated with UC.

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Anita Kartika Sari and Tri Wahjoedi. "Proactive personality literature through scientometric analysis." World Journal of Advanced Research and Reviews 15, no. 2 (August 30, 2022): 177–85. http://dx.doi.org/10.30574/wjarr.2022.15.2.0802.

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The proactive personality is designed to seize a behavioral tendency to impose, or change, a person`s environment. This take a look at goals to observe the popularity and role of the visible maps of global studies and the Scopus listed proactive character literature the usage of bibliometric positioning images. The studies turned into carried out the usage of a bibliometric technique. Data evaluation and use of the VOSViewer visualization application and the Scopus characteristic to examine seek results. In this review, the information amassed follow to 496 files posted from 1993 to 2020. The take a look at famous that Renmin University of China and United State is the maximum energetic affiliated and country institutions, scientists and countries within side the proactive character literature. In the proactive character literature, Business, Management and Accounting and Journal of Vocational Behavior are the maximum studied fields and reasserts of dissemination. There is one map of businesses round the arena with collaborative researchers. To discover the frame of expertise produced from thirty- years of publication, this take a look at constructs a convergence axis grouping inclusive of proactive character literature: Personality, Employee, Career, Organization, Manager, and Entrepreneur, abbreviated as PECOME.

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Wong, Yvonne W. Y., and Julian H. L. Kang. "Perivascular epithelioid cell tumour of the uterus: what do we know?" International Journal of Reproduction, Contraception, Obstetrics and Gynecology 9, no. 12 (November 26, 2020): 5110. http://dx.doi.org/10.18203/2320-1770.ijrcog20205255.

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Perivascular epithelioid cell neoplasm (PEComa) is a rare mesenchymal tumour characterized by distinctive histological and immunohistochemical perivascular epithelioid cells. These tumours can be found in various anatomic sites, with gynaecologic PEComas accounting for nearly one-fourth of reported cases in the literature. However, due to its non-specific clinical presentation and a lack of definitive radiological appearance, the diagnosis of PEComas remains challenging. In this case report, we describe a 45-year old lady suffering from urinary retention secondary to large uterine fibroids, who then underwent a total hysterectomy. Postoperative histopathology with immunohistochemical stains confirmed an unexpected finding of uterine PEComa. Although the treatment of gynaecologic PEComas remains controversial, complete surgical resection with negative margins is recommended. A multidisciplinary approach will be beneficial in determining the necessity of adjuvant therapy such as targeted therapy with mTOR inhibitors, especially for PEComas which exhibit aggressive and high-risk features.

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Lim, Beom Jin, Si-Gyun Roh, Jin Yong Shin, Nae-Ho Lee, Yoon Kyu Chung, and Kyu Yun Jang. "Treatment of malignant perivascular epithelioid cell tumor (PEComa) on the knee with an anterolateral thigh free flap: A case report." Medicine 102, no. 32 (August 11, 2023): e34679. http://dx.doi.org/10.1097/md.0000000000034679.

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Rationale: The World Health Organization defines a perivascular epithelioid cell tumor (PEComa) as a mesenchymal neoplasia composed of perivascular epithelioid cells with characteristic morphological and immunohistochemical features. Although PEComas have the potential to behave in a malignant fashion, malignant PEComas are extremely rare. Patient concerns: An 83-year-old man visited our clinic presented with palpable, painless, and movable mass in the right knee area. Diagnoses: Malignant PEComa was diagnosed by incisional biopsy. No metastases was confirmed by radiologic imaging including PET/CT, magnetic resonance imaging, high resolution computed tomography. Interventions: We performed wide excision of the mass and used an anterolateral thigh free flap to reconstruct the defect on the right knee. Outcomes: The permanent histopathology showed malignant PEComa was totally resected. The flap which was performed to cover the defect was survived and the patient discharge without any complications. Lessons: PEComa can metastasize to various anatomical regions. Although there is no established standardized treatment, radical resection is still considered the cornerstone of treatment. Rapid and appropriate defect coverage is important to improve the patient’s prognosis.

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Bleeker, Jonathan S., J. Fernando Quevedo, and Andrew L. Folpe. "“Malignant” Perivascular Epithelioid Cell Neoplasm: Risk Stratification and Treatment Strategies." Sarcoma 2012 (2012): 1–12. http://dx.doi.org/10.1155/2012/541626.

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Purpose. Perivascular epithelioid cell tumors (PEComas) are a rare collection of tumors characterized by a myomelanocytic phenotype, and PEComas occurring in “nonclassic” anatomic distributions are known as perivascular epithelioid cell tumor not otherwise specified (PEComa-NOS). This review aims to compile and analyze cases of PEComa-NOS in an effort to better define their natural history.Design. We evaluated all 234 cases of PEComa-NOS reported in the English literature, extracting information regarding diagnostic features, treatment approaches, and outcomes. Multivariate analysis of a number of variables evaluable on pathologic review was performed to refine preexisting risk stratification criteria. Outcomes for patients receiving nonsurgical treatment are also reported.Results. Primary tumor size ≥5 cm (P=0.02) and a high (1/50 HPF) mitotic rate (P<0.0001) were the only factors significantly associated with recurrence following surgical resection. Cytotoxic chemotherapy and radiation therapy have shown little benefit in treating PEComa-NOS; mTOR inhibition is emerging as a treatment option.Conclusion. Progress has been made in understanding the natural history and molecular biology of PEComa-NOS. This review further clarifies risk of recurrence in this disease, allowing clinicians to better risk stratify patients. Further work should focus on applying this knowledge to making treatment decisions for patients with this disease.

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Eken, Alper, and Yesim Saglican. "Primary perivascular epithelioid cell tumour (PEComa) of the prostate." Canadian Urological Association Journal 8, no. 5-6 (June 19, 2014): 455. http://dx.doi.org/10.5489/cuaj.1752.

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Perivascular epithelioid cell tumours (PEComas) are a family of rare mesenchymal tumours arising in various anatomic locations. PEComas are defined by the presence of perivascular epithelioid cells that coexpress muscle and melanotic markers, especially HMB-45. They have unpredictable biological behaviour and are mostly benign, but some tumours can become unresectable or metastatic. Surgical resection, when possible, is the best treatment option. Radiation therapy, cytotoxic chemotherapy or immunotherapy have been reported as treatment options, either alone or in combination therapy. Prostatic PEComa is extremely rare, with only 1 malignant case reported. We report the first case of prostatic PEComa, which was benign and treated with transurethral resection.

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Kloth, C., B. Boozari, H. Bösmüller, M. Haap, and M. Horger. "Multimodality Imaging of hepatic PEComa – Multimodale Bildgebung von hepatischen PEComen." RöFo - Fortschritte auf dem Gebiet der Röntgenstrahlen und der bildgebenden Verfahren 187, no. 03 (February 19, 2015): 147–50. http://dx.doi.org/10.1055/s-0034-1369612.

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Hekimoglu, Koray, and Murat Haberal. "Liver Perivascular Epithelioid Cell Tumor with an Unusual Location: Diagnostic Characteristics with Multidetector Computed Tomography and Magnetic Resonance Imaging." Journal of Clinical Imaging Science 7 (September 18, 2017): 36. http://dx.doi.org/10.4103/jcis.jcis_43_17.

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Primary perivascular epithelioid cell tumor (PEComa) of the liver is a very rare tumor that originates from mesenchyma. Gastrointestinal tract with perivascular distribution is the most common anatomic sites of these tumors. Only few cases of hepatic PEComa have been described so far. Malignant PEComas exhibit aggressive behavior with poor prognosis, making early diagnosis crucial. Hereby, we report a 79-year-old female with unusually located mass in the liver. A partial curative hepatectomy has been done, and PEComa was diagnosed histopathologically. No evidence of recurrence was observed during the 6-month follow-up.

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Al-Nahhas, Omar, Aysha Simran Haris, Yousif Basim Raad, Khaled Alaboud Alkheder, and Kenichi Miyata. "Benign Perivascular Epithelioid Cell Tumor of the Mesentery Misdiagnosed as a Uterine Fibroid: A Case Report and Review of Literature." Open Access Macedonian Journal of Medical Sciences 12, no. 1 (February 15, 2024): 136–40. http://dx.doi.org/10.3889/oamjms.2024.11842.

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BACKGROUND: Perivascular epithelioid cell tumor (PEComa) is a type of mesenchymal tumor composed of histologically and immunohistochemically distinctive perivascular epithelioid cells. Benign mesenteric PEComa is a rare entity as there has been only one case reported so far. We are presenting a case of benign mesenteric PEComa in a 30-year-old female that presented with abdominal pain and was diagnosed by microscopic morphological examination and immunohistochemical staining. The aim of this case report is to make health-care professionals aware of the diagnostic criteria in similar presentations, treatment modalities offered for this patient, and others reported over the years for malignant as well as benign PEComas and also shedding the light on a new presentation and diagnosis. CASE REPORT: We reported a case of a 30-year-old female who presented with abdominal pain radiating to the back and rectum. After a thorough physical examination which was unremarkable, a CT scan was done which showed a mesenteric mass. This mass was then resected and sent for histopathology which revealed a PEComa. We report the history, clinical findings, laboratory reports, and gross imaging of a 30-year-old female who presented to our clinic and was diagnosed with a very rare benign mesenteric PEComa. CONCLUSION: PEComa is a rare tumor, and as PEComas arising from the mesentery being one of the rarer kinds with only 10 cases reported and this case being the 11th, out of those ten cases, only two of them were found to be benign which makes the case we are reporting the second benign mesenteric PEComa reported in English Literature.

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Battistella, Enrico, Luca Pomba, Marica Mirabella, Michele Gregianin, Antonio Scapinello, Marco Volante, and Antonio Toniato. "Metastatic Adrenal PEComa: Case Report and Short Review of the Literature." Medicina 59, no. 1 (January 11, 2023): 149. http://dx.doi.org/10.3390/medicina59010149.

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PEComa has become a widely accepted entity, and increased recognition has led to descriptions of this tumor in a wide variety of anatomic sites, including the adrenal gland. PEComa (perivascular epithelioid cell tumor) is a mesenchymal tumor composed of perivascular cells, and the most frequent sites of PEComas are the uterus and retroperitoneum. The incidence is <1 per 1,000,000 people. We report a case of adrenal metastatic PEComa in a 63-year-old man discovered by a spontaneous hematoma of the rectus abdominis. In our case, PEComa of the adrenal gland was a significant diagnostic dilemma as the morphologic and immunophenotypic features of this neoplasm may easily be confused with those of other more commonly encountered lesions.

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Conlon, Niamh, Robert A. Soslow, and Rajmohan Murali. "Perivascular epithelioid tumours (PEComas) of the gynaecological tract." Journal of Clinical Pathology 68, no. 6 (March 6, 2015): 418–26. http://dx.doi.org/10.1136/jclinpath-2015-202945.

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Perivascular epithelioid tumours (PEComas) of the gynaecological tract are rare tumours which were first recognised and diagnosed within the last 20 years. They represent a unique diagnostic challenge with regard to their accurate and reproducible distinction from more common entities such as smooth muscle tumours of the uterine corpus. In this review article, we trace the development of the concept of the PEComa tumour family, highlight what is known about extra-gynaecological tract PEComa at an immunohistochemical, molecular and therapeutic level and then present a summary of all reported cases of gynaecological tract PEComa to date. In the summary, we highlight rare subtypes of gynaecological tract PEComa and compare the performances of extant prognostic classification systems for malignancy in these tumours.

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de la Fouchardiere, Arnaud, David J. Papke, Daniel Pissaloux, Eleanor Russell-Goldman, Franck Tirode, Christopher D. M. Fletcher, and John Hanna. "Melanotic PEComa." American Journal of Surgical Pathology 48, no. 1 (September 28, 2023): 88–96. http://dx.doi.org/10.1097/pas.0000000000002135.

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Perivascular epithelioid cell neoplasms (PEComas) are tumors of uncertain cell lineage that show a strong female predominance. Their hallmark is the presence of combined smooth muscle and melanocytic differentiation. In most cases, melanocytic differentiation is detectable only by immunohistochemistry, but there are rare reports of PEComa with extensive melanin accumulation (so-called “melanotic PEComa”). Here we report a clinicopathologic series of 7 melanotic PEComas that occurred across a wide patient age range of 21 to 82 years (median: 41 y) and with a wide anatomic distribution, including 2 cases in the pelvis and 1 case each in the gallbladder, cervix, eyelid, epidural space, and femur. All tumors were heavily pigmented and, like conventional PEComas, were composed of variably sized neoplastic cells with voluminous granular, or less commonly clear, cytoplasm with prominent nucleoli. All tumors expressed HMB45 by immunohistochemistry, and 6 of 7 showed nuclear TFE3 expression. Where tested, tumors were uniformly negative for Mart-1/Melan-A, S100, desmin, and smooth muscle actin. Molecular analysis identified TFE3 gene rearrangement in 5 of 7 cases, 4 of which were demonstrated by fluorescence in situ hybridization and one by whole-exome RNA sequencing which revealed a SFPQ::TFE3 fusion. The one tumor negative for TFE3 by immunohistochemistry was found instead to harbor a SFPQ::TFEB fusion, the first reported example to our knowledge of TFEB fusion in a PEComa. Clinical follow-up was available for 6 of 7 patients (median: 2.5 y: range: 0.75 to 7 y). The patient whose tumor harbored SFPQ::TFEB died of metastatic disease 9 months after diagnosis. The other tumors behaved in an indolent fashion: 4 patients were alive without evidence of disease at the most recent follow-up and 1 patient died of an unrelated cancer 4 years after diagnosis of the melanotic PEComa. Our results expand the morphologic and molecular spectrum of melanotic PEComa, and awareness of this rare but distinctive subtype is important to ensure accurate diagnosis within the broader family of heavily pigmented neoplasms.

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Fink, D., D. E. Marsden, L. Edwards, C. Camaris, and N. F. Hacker. "Malignant perivascular epithelioid cell tumor (PEComa) arising in the broad ligament." International Journal of Gynecologic Cancer 14, no. 5 (2004): 1036–39. http://dx.doi.org/10.1136/ijgc-00009577-200409000-00045.

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Malignant perivascular epithelioid cell tumor (PEComa) is an extremely rare mesenchymal neoplasm mostly composed of HMB-45-positive epithelioid cells with clear-to-eosinophilic cytoplasm, a propensity for perivascular distribution and a coexpression of smooth muscle markers. The uterus seems to be one of the most prevalent sites of involvement, although only 14 cases of uterine PEComa have been described. We report the case of a 51-year-old woman with a PEComa arising in the broad ligament. She was treated with total abdominal hysterectomy, bilateral salpingo-oophorectomy, omentectomy, and pelvic radiation, and remains without evidence of disease 15 months after diagnosis. This is, to the best of our knowledge, the first report of a malignant PEComa arising in the broad ligament. To correctly diagnose PEComa, an extensive immunohistochemical panel is essential. As PEComas can behave in an aggressive manner, careful follow-up is warranted.

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Lian, D. W. Q., K. L. Chuah, M. H. W. Cheng, and W. M. Yap. "Malignant perivascular epithelioid cell tumour of the fibula: a report and a short review of bone perivascular epithelioid cell tumour." Journal of Clinical Pathology 61, no. 10 (September 26, 2008): 1127–29. http://dx.doi.org/10.1136/jcp.2008.060137.

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Perivascular epithelioid cell tumour (PEComa) is a term applied to a family of mesenchymal tumours composed of varying proportions of spindle and epithelioid cell components associated with HMB-45 expression. PEComa rarely arises in the soft tissue, visceral organs, skin and bone. This report details an instance when a purely epithelioid PEComa arose from the right fibula of a 52-year-old Chinese woman without features of tuberous sclerosis complex. The excision specimen disclosed an epithelioid tumour with a nested pattern associated with areas of nuclear pleomorphism, mitotic activity, necrosis and vascular invasion in addition to HMB-45 expression on immunohistochemistry. To the best of the authors’ knowledge, this represents the first case of a histologically malignant PEComa of the bone. A short review of primary bone PEComas and potential problems in diagnosis is presented.

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Lanchinsky, V. I., Yu V. Chushkov, A. B. Ponomarev, Yu I. Osmanov, A. I. Ishchenko, and N. V. Vorobev. "The problem of rare tumors in gynecology: a clinical case of benign retroperitoneal sclerosing pelvic pecoma in 49-year-old woman." Research and Practical Medicine Journal 9, no. 2 (May 4, 2022): 113–24. http://dx.doi.org/10.17709/2410-1893-2022-9-2-10.

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The tumor lesions of the reproductive system organs carry the leading position in the structure of gynecological diseases. One of the rare tumors that can affect the organs of the reproductive system is pecoma, which is a tumor of mesenchymal origin. Because of it’s rare occurrence the gynecologists often misinterpret available clinical data and the results of patient`s examination, diagnosing and treating the tumor as a myoma before having received the results of the histological examination. Our article describes a clinical case of a patient who had a preliminary diagnosis of uterine fibroids, and because of this fact the planned surgical treatment was carried out. But a retroperitoneal tumor originating from the uterine vein was detected during the surgery. The diagnosis of sclerosing pecoma was established by histological and immunohistochemical studies. The article analyzes the clinical features of the patient`s disease, as well as the available results of instrumental methods of examination which can lead to final diagnosis of pecoma, also difficult issues of diagnosis and tactics of diagnostic search are noted.The literature data on the frequency of detection of pecomas, the features of their structure are presented. It is marked, that there is also a possibility of pecoma`s localization in various organs, for example, lungs, liver, kidneys, as well as in soft tissues. The possibility of multiple lesions – pecomatosis is emphasized. It is noted that among the pelvic organs the uterus is affected most often, but furthermore, the pelvic lymph nodes, the broad ligament, the omentum, the peritoneum of the pelvis and the rectum wall can be also involved in the pathological process. Risk factor of pecomas malignancy is discussed separately, the presence of three types of pecomas is determined. This fact significantly effects the probability of relapse of the tumor after surgical removal and, respectively, the tactics of postoperative follow‑up of such patients.

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Fassan, Matteo, Mauro Cassaro, Massimo Vecchiato, Roberto Clemente, Gianmaria Pennelli, Stefano Merigliano, and Giuseppe Altavilla. "Malignant Perivascular Epithelioid Cell Tumor of the Esophagus." Case Reports in Pathology 2012 (2012): 1–5. http://dx.doi.org/10.1155/2012/438505.

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Malignant perivascular epithelioid cell tumor (PEComa) is a rare tumor composed of hybrid tumor cells characterized by immunoreactivity for both melanocytic and smooth muscle markers. This paper describes the uncommon esophageal location of an 8 cm PEComa in a 75-year-old Caucasian man who was presented with ingravescent dysphagia. Although PEComas arising within the gastrointestinal tract are exceptional findings, clinicians should not exclude this class of tumors in the diagnostic investigation of a bulky lesion of the esophageal wall.

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Khatib, Rawaan, Jacqueline Nguyen, Jasika Grover, Kat Chin, Nikhita Tandon, Abhigna Reddy, Israa Jahan, et al. "Perivascular epithelioid cell tumors: A study of demographic and socioeconomic factors." Journal of Clinical Oncology 42, no. 16_suppl (June 1, 2024): e17594-e17594. http://dx.doi.org/10.1200/jco.2024.42.16_suppl.e17594.

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e17594 Background: Perivascular epithelioid cell tumors (PEComas) are rare soft tissue tumors composed primarily of epithelioid cells and found most commonly in perivascular spaces. Previously, many different tumors, such as renal angiomyolipoma and leiomyosarcoma, were considered disparate conditions. However, the more recent identification of perivascular epithelioid cells common to these tumors has recategorized them as PEComas. Due to this, there is an increase in PEComa cases but development of curative treatments has not seen the same growth. Further investigation into the epidemiology of patients diagnosed with PEComas may improve treatment options and outcomes. Data obtained from the National Cancer Database (NCDB) were utilized to analyze the demographics of PEComa patients. Methods: A retrospective cohort study analyzed 114 individuals diagnosed with PEComa (ICD-8714-3) utilizing data from the 2018-2020 NCDB. The analysis used descriptive statistics to examine demographic factors including age, sex, race, Hispanic origin, educational attainment, insurance status, facility type, distance from facility, and Charlson/Deyo score. Regression analysis was used to interpret incidence trends. Results: A total of 114 patients were diagnosed with PEComa between the years 2018-2020, with a slight increase in diagnoses made each year (R2= 0.893). The average age of diagnosis was 53.1 years (SD = 15.2, range = 7-86) and women (89%) were more likely to be diagnosed than men (11%). Primary site of diagnosis was the uterus, NOS (22%). Primary treatment options were surgery (81%), chemotherapy (27%), and radiation (9%). Many individuals (83%) had no concurrent comorbidities (Charlson-Deyo comorbidity score = 0) and survival rates following treatment were 81% at 2 years and 63% at 5 years. The majority of patients identified as non-Spanish/non-Hispanic (83%) and White (71%). Most patients were treated in an academic/research program (49%), 33% qualified for the highest quartile of income earners ($74,063 or more), lived in a metropolitan area (61%), and were privately insured (63%). Patients lived on average 67.3 miles (SD=187.5) from the treatment facility. Conclusions: To the best of our knowledge, this is the first NCDB analysis on PEComa, which addresses a key knowledge gap within current literature regarding the topic. In concurrence with previous reports, female diagnoses predominated with the primary site of diagnosis in the uterus. The socioeconomic factors of PEComa patients have not been covered in the literature until now: patients are more likely to have a median income of $74,063 or more, be treated in an academic/research program, and live over 60 miles from the treatment facility. Additional research is essential to understand the impact of socioeconomic and demographic factors on diagnosis, treatments, and survival rates of patients with PEComa.

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Sobiborowicz, Aleksandra, Tomasz Świtaj, Paweł Teterycz, Mateusz J. Spałek, Anna Szumera-Ciećkiewicz, Michał Wągrodzki, Marcin Zdzienicki, Anna M. Czarnecka, and Piotr Rutkowski. "Feasibility and Long-Term Efficacy of PEComa Treatment—20 Years of Experience." Journal of Clinical Medicine 10, no. 10 (May 19, 2021): 2200. http://dx.doi.org/10.3390/jcm10102200.

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Perivascular epithelioid cell tumors (PEComas) represent a family of rare mesenchymal neoplasms, some of which are malignant. There are no specific management guidelines for PEComas, and factors correlating with the disease course are not well defined. This analysis aimed to describe the outcomes of PEComa patients treated radically, including those treated exclusively in the national reference sarcoma center. The secondary aim of the study was to analyze factors associated with PEComa treatment efficacy. We performed an analysis of 27 patients subsequently treated radically for PEComa between 1999 and 2019 who were in follow-up in the national sarcoma reference center. The proportional-hazards model was used to compare the risk of death. The median age at diagnosis was 45 (21–67) years, and 67% of patients were female. The median follow-up period was 68 months (95% CI: 39–101). At the time of analysis, eleven patients (40.7%) experienced progression of the disease and four (14.8%) died. Surgery in the reference sarcoma center was associated with a longer disease control (log-rank p < 0.001). The 5-year-OS rate was 88% (95% CI: 74–100) for the whole analyzed group. We concluded that PEComa treatment should be managed in reference sarcoma centers by a multidisciplinary tumor board with an experienced surgical team. Microscopically radical resection is associated with a longer disease-free survival. Patients requiring long-term follow-ups as late recurrence may be expected.

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Kokabee, M., X. Zhong, S. Sham, P. Unger, and M. Grasso. "Sclerosing Perivascular epithelioid cell neoplasms (PEComa), a rare variant in a unique family of tumors." American Journal of Clinical Pathology 160, Supplement_1 (November 1, 2023): S51. http://dx.doi.org/10.1093/ajcp/aqad150.115.

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Abstract Introduction/Objective Perivascular epithelioid cell neoplasms (PEComas) are a rare group of neoplasms that characteristically show both smooth muscle and melanocytic differentiation. The most common tumor in this family is renal angiomyolipoma but may occur in various sites. Sclerosing PEComa (S-PEComa) is an extremely rare variant. its only reported in females and it is usually an incidental finding in the retroperitoneum, in close proximity to the kidney. Methods/Case Report . We present a 52-year-old female, who had a chest CT scan for worsening cough. An - 4 cm exophytic renal mass in the left renal upper pole was found. Patient underwent left partial nephrectomy. Grossly the specimen consisted of 2.5 cm well-circumscribed, white tan mass, abutting the renal capsule, and extending into perinephric adipose tissue. Histology revealed uniform bland epithelioid cells with palely eosinophilic cytoplasm and round nuclei embedded in abundant densely sclerotic stroma. No fat was identified. Immunohistochemistry showed the tumor to be positive for desmin, HMB-45 and SMA with focal staining for AE1/AE3 and negative staining for EMA, cam 5.2, CD 117, calretinin, CD 34, S-100, PAX 8, and Melan A, which confirming the diagnosis of S-PEComa. Results (if a Case Study enter NA) NA Conclusion PEComas, excluding the epithelioid variant, usually has a benign course. In the 13 cases previously reported S-PEComa, 2 patients had metastasis. Our patient, 3-month post-op, has no evidence of disease, but close follow up still warranted.

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Unluoglu, Saime, Umit Bayol, Nilay Korkmaz, Bekir Ozenen, Fuat Ipekci, and Emel Ebru Pala. "Perivascular Epithelioid Cell Tumor of the Ileum Presenting as Diverticulitis." Case Reports in Pathology 2012 (2012): 1–4. http://dx.doi.org/10.1155/2012/476941.

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Perivascular epithelioid cell tumors (PEComas) are a group of rare mesenchymal neoplasms. Gastrointestinal PEComas are exceptionally rare, there being only a few case reports in the literature involving the colon and small intestine. Nearly all PEComas show immunoreactivity for both melanocytic (HMB45 and/or Melan-A) and smooth muscle (actin and/or desmin) markers. A 36-year-old male was admitted to the hospital with acut- abdomen. At laparatomy, a nodular mass protruding from the ileum which clinically simulated a diverticulitis was noticed. Gross examination of the specimen revealed a2×1,5×1 cm secondarily ulcerated, solid, nodular, gray white tumor mass in the ileal wall. Histologically, tumor cells were composed of nests of round-polygonal epithelioid cells with abundant clear to slightly eosinophilic granular cytoplasm and round vesicular nuclei. The nests were separated by thin fibrovascular septa. Minimal necrosis and low mitotic activity were noticed in the tumor. Immunohistochemically, tumor cells were positive for SMA, HMB45, and Melan-A and negative for CD10, RCC, CD45, CD117, CD34, EMA, and Desmin. Diagnosis was PEComa of the ileum. We report the case of ileal PEComa to remind the unusual presentation (diverticulitis) of these tumors, besides rarity and diagnostic difficulties.

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Randhawa, Samender, Jessica Pinsker, Madhurya Amirapu, Metlapalli Venkata Sravanthi, Prashanth Ashok Kumar, and Komal Akhtar. "A Rare Cause of Chronic Hip Pain From PEComa: An Aggressive Mesenchymal Sarcoma." Journal of Investigative Medicine High Impact Case Reports 10 (January 2022): 232470962211033. http://dx.doi.org/10.1177/23247096221103385.

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Perivascular epithelioid cell tumors (PEComas) are related to the tuberous sclerosis complex (TSC) and are commonly benign. When malignant, they can be aggressive with local invasion and metastatic spread. Conventional PEComas do not have TFE3 gene rearrangement and are associated with TSC with a preference for an occurrence at a younger age. We report a case of a young male who had progressive chronic hip pain and was found to have a TFE3-associated PEComa in his pelvic region.

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Marinho, Bárbara Monteiro, António Gâmboa Canha, Donzília Sousa Silva, and José Davide Pinto Silva. "Primary retroperitoneal PEComa: an incidental finding." BMJ Case Reports 15, no. 11 (November 2022): e250466. http://dx.doi.org/10.1136/bcr-2022-250466.

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Perivascular epithelioid cell neoplasm (PEComa) is a rare type of tumour, and primary retroperitoneal PEComa is rarer still. Although pulmonary lymphangioleiomyomatosis (LAM), angiomyolipomas and clear cell ‘sugar’ tumours of the lung are well described, relatively little is known about other members of the PEComa family. We describe a case of an asymptomatic retroperitoneal PEComa, lymphangioleiomyoma type, which appeared in a previously healthy middle-aged woman as an incidental finding, in a CT scan performed in the context of spontaneous pneumothorax. The patient underwent surgical excision of the tumour and the histopathological and immunohistochemical analysis of the surgical specimen made the definitive diagnosis. Although rare, reports of isolated retroperitoneal lymphangioleiomyoma and primary retroperitoneal PEComas NOS (not otherwise specified) are described in the literature, normally associated with pulmonary LAM. The patient’s pulmonary imaging was normal. Short-term re-examination did not detect any recurrence. We also provide a literature review of this rare group of tumours.

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Yang, Han-Teng, Fu-Rong Wang, Na He, Yuan-Hua She, Yong-Yue Du, Wen-Gui Shi, Jing Yang, et al. "Massive simultaneous hepatic and renal perivascular epithelioid cell tumor benefitted from surgery and everolimus treatment: A case report." World Journal of Gastrointestinal Surgery 16, no. 10 (October 27, 2024): 3334–42. http://dx.doi.org/10.4240/wjgs.v16.i10.3334.

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BACKGROUND Perivascular epithelioid cell tumor (PEComa) is a rare mesenchymal neoplasm that predominantly affects the kidney and uterus. The occurrence of this tumor in the liver, particularly with simultaneous involvement of the liver and kidney, is exceedingly uncommon. Pathological diagnosis is the gold standard. PEComas usually show positive immunohistochemical staining for melanocytic (HMB-45, Melan-A) and myoid (SMA, muscle-specific actin) markers. CASE SUMMARY We presented a noteworthy case of malignant PEComa affecting both the liver and kidney in a 53-year-old man with tuberous sclerosis complex (TSC). FAT2 and TP73 mutations in the kidney were identified and positive expression of diagnostic markers including HMB-45, Melan A, and TFE3 were detected. In addition, we demonstrated that hepatic artery perfusion chemotherapy was ineffective for hepatic PEComa, while surgery remained the most effective approach. Everolimus showed an excellent efficacy in the postoperative treatment of the tumor. CONCLUSION Surgical treatment is preferred for malignant PEComa affecting liver and kidney, especially with TSC; everolimus is effective postoperatively.

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Cabrera Cisneros, Juliana, Cynthia Laura Rossi, María Laura Trotta, and Andrea Vanesa Giuliani. "PEComa cutáneo: desafío diagnóstico de una neoplasia infrecuente." Dermatología Argentina 29, no. 2 (August 8, 2023): 89–91. http://dx.doi.org/10.47196/da.v29i2.2378.

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Los tumores de células epitelioides perivasculares (PEComas) son un grupo raro de neoplasias mesenquimáticas caracterizadas histológicamente por la proliferación de células con citoplasma claro, aspecto epitelioide y disposición perivascular, que tienen la capacidad de coexpresar marcadores inmunohistoquímicos melanocíticos y miogénicos. Los PEComas cutáneos primarios son infrecuentes; representan alrededor del 8%. Se presenta un caso de PEComa cutáneo para tenerlo en cuenta como diagnóstico diferencial de enfermedades que comparten características histopatológicas e inmunohistoquímicas similares como el melanoma amelanótico o las metástasis de carcinoma renal de células claras.

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Hamza, Ameer, and Charles C. Guo. "Perivascular Epithelioid Cell Tumor of the Urinary Bladder: A Systematic Review." International Journal of Surgical Pathology 28, no. 4 (December 22, 2019): 393–400. http://dx.doi.org/10.1177/1066896919895810.

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Perivascular epithelioid cell tumor (PEComa) of the urinary bladder is a rare neoplasm showing distinct melanocytic and smooth muscle differentiation. We aimed to review the clinicopathologic features of bladder PEComa using all the available cases in the literature, along with 2 new cases from our database. The patients included 15 females and 15 males with a mean age of 39.2 ± 15.3 years. Painless hematuria was the most common clinical presentation. The tumors were usually well circumscribed with a mean tumor size of 4.4 ± 2.7 cm. Bladder PEComas demonstrated nests, trabeculae, or sheets of epithelioid cells with intermixed spindled cells and numerous thin-walled vessels. Immunohistochemical studies showed that the tumors were positive for HMB45 (27/27), cathepsin (4/4), SMA (20/22), and caldesmon (3/3) and were negative for pan cytokeratin (0/18) and EMA (0/4). Molecular studies revealed that PEComa was associated with the TFE3 (n = 3) and EWSR1 (n = 1) gene rearrangements. Treatment included partial cystectomy (n = 18), transurethral resection (n = 8), and radical cystectomy (n = 4). Twenty patients had no evidence of disease during a mean follow-up time of 19.4 ± 17.2 months. Two patients had recurrence, and 1 patient died of metastatic disease. In conclusion, bladder PEComas demonstrate distinct morphologic and immunohistochemical features. Although most tumors follow a benign course, a small subset may develop metastasis and cause death.

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Nguyen, Duy, Truong Nguyen, Thu Phan, and Dat Ngo. "A case report of hepatic perivascular epithelioid cell tumours (pecomas) and literature review." MedPharmRes 2, no. 2 (July 8, 2018): 39–42. http://dx.doi.org/10.32895/ump.mpr.2.2.39.

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Perivascular epithelioid cell tumours (PEComas) belongs to the family of mesenchymal neoplasms that can occur in many organs, but rarely found in liver. Preoperative diagnosis could be challenging due to unspecific and variable radiologic patterns, which can be clinically misdiagnosed with hepatocellular carcinoma and other benign tumors. This report aims to announce a case of hepatic PEComa with extramedullary hematopoiesis. A 44- year- old woman accidentally presented a nodular mass in the left hepatic segment on ultrasonography with normal liver function result. Abdominal enhanced CT Scan showed hypervascular lesion. Morphology exibits classical characteristics of PEComa with mature fat tissue predominance, thick-wall vessel; somewhat amount of extramedullary hematopoiesis was also recognized. Tumor cells are diffusely immunoreactive with HMB45. PEComa is a rare disease in liver, pathologists need to recognize three components of this tumor to avoid misdiagnosing with malignant conditions.

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Kapoor, Raghav, Anila Sharma, Sunil Pasricha, Meenakshi Kamboj, and Anurag Mehta. "Beyond the ordinary: A rare glimpse of PEComa TFE3 rearranged in soft tissue." Indian Journal of Pathology and Oncology 11, no. 1 (April 15, 2024): 56–58. http://dx.doi.org/10.18231/j.ijpo.2024.012.

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The term “Perivascular epithelioid cells” was described as early as 1992. WHO has defined PEComas as mesenchymal tumors composed of histologically and immunohistochemically distinctive perivascular epithelioid cells. These tumors have a varied morphology including sheets and nested pattern comprising of epithelioid to spindle tumor cells with increased vasculature often in a sinusoidal pattern. PEComas show immunoreactivity for melanocytic and smooth muscle markers, however small number cases show lack of muscle marker expression along with strong TFE3 nuclear positivity. Here we describe a PEComa of soft tissue of leg, TFE3 rearranged.

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Matkowskyj, Kristina A., M. Sambasiva Rao, and Kirtee Raparia. "Transcription Factor E3 Protein–Positive Perivascular Epithelioid Cell Tumor of the Appendix Presenting as Acute Appendicitis: A Case Report and Review of the Literature." Archives of Pathology & Laboratory Medicine 137, no. 3 (March 1, 2013): 434–37. http://dx.doi.org/10.5858/arpa.2012-0103-cr.

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Perivascular epithelioid cell tumors (PEComas) are a group of mesenchymal tumors that coexpress melanocytic and smooth muscle markers; their exact origin remains unknown. This group includes renal angiomyolipoma, clear cell sugar tumor, and lymphangioleiomyomatosis, although the term perivascular epithelioid cell tumors is currently used for lesions that exhibit a similar morphologic and immunohistochemical profile throughout the human body. Recently, a distinct subset of PEComas has been shown to harbor transcription factor E3 gene (TFE3) fusions. We report, for the first time, a unique case of TFE3-positive PEComa presenting as acute appendicitis in a 24-year-old woman. Microscopically, the tumor was composed of benign-appearing epithelioid cells with clear and eosinophilic cytoplasm, and arranged in nested and alveolar patterns. Immunohistochemical studies showed diffuse strong positivity for neuron-specific enolase, TFE3, and progesterone receptor and focal strong positivity for human melanoma black–45 (HMB-45) and melanocyte differentiation antigen (Melan-A) in the tumor cells. Although rare, PEComa should be included in the differential diagnosis of mesenchymal tumors of the appendix.

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Krawczyk, Marek, Bogna Ziarkiewicz-Wróblewska, Tadeusz Wróblewski, Joanna Podgórska, Jakub Grzybowski, Beata Gierej, Piotr Krawczyk, et al. "PEComa—A Rare Liver Tumor." Journal of Clinical Medicine 10, no. 8 (April 18, 2021): 1756. http://dx.doi.org/10.3390/jcm10081756.

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PEComa (perivascular epithelioid cell tumor) is a rare liver tumor. Decisions regarding patient management are currently based on a few small case series. The aim of this study was to report the clinicopathological features of PEComa in order to provide guidance for management, complemented by our own experience. This retrospective observational study included all patients with PEComa who underwent surgical treatment in two departments between 2002 and 2020. A total of 20 patients were diagnosed with PEComa following histopathological examination. The age of the patients ranged from 21 to 73 years. The majority of patients were women (85%). In most patients, the tumors were incidental. In diagnostic studies, PEComas with high arterial vascularization have been described. Liver resection was the treatment of choice. There was only one postoperative complication. During histopathological evaluation, tumors were composed mostly of epithelioid cells, rarely with spindle cell components, thick-walled vessels, and adipocytes in different proportions. Melanocytic markers (HMB45, MelanA) and at least one smooth muscle marker were expressed in all tumors. Features suggestive of malignancy were found in three cases. In conclusion, PEComa is a rare liver tumor that is usually diagnosed incidentally. In radiological studies, tumors with high arterial vascularization are observed. Liver resection is the treatment of choice.

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Giannella, Luca, Giovanni Delli Carpini, Nina Montik, Valeria Verdecchia, Francesca Puccio, Jacopo Di Giuseppe, Dimitrios Tsiroglou, Gaia Goteri, and Andrea Ciavattini. "Ultrasound Features of a Uterine Perivascular Epithelioid Cell Tumor (PEComa): Case Report and Literature Review." Diagnostics 10, no. 8 (August 3, 2020): 553. http://dx.doi.org/10.3390/diagnostics10080553.

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Background: Perivascular epithelioid cell tumors (PEComas) are rare mesenchymal tumors. One of the most frequent localizations of PEComas is the female genitourinary tract, and the uterus is the most involved site after the kidney. Correct preoperative diagnosis is rarely achieved due to the presence of nonspecific imaging features. We report a case of a uterine PEComa with particular reference to ultrasound’s role in characterizing this rare occurrence. Case presentation: a 45-year-old White woman came to our observation for cyclic abdominopelvic pain and chronic constipation. The pre-surgical ultrasound examination showed a heterogeneous tumor that was 4 cm in size, localized on the right anterolateral uterine wall. The mass had well-delimited borders and a central hypoechoic portion. The use of color Doppler showed a rich, irregular vasculature in the center with low impedance. The preoperative diagnostic hypothesis was of a smooth muscle tumor of uncertain malignant potential. After careful counseling, a surgical approach was decided upon, including a total laparoscopic hysterectomy with bilateral salpingectomy. The histological and phenotypical features were consistent with a uterine PEComa. At the last follow-up, two years after surgery, the patient is alive and well. Conclusions: Uterine PEComa is a rare occurrence that should be included in the differential diagnosis of uterine wall tumors. It can appear as a small uterine mass with heterogeneous echogenicity and a rich vascular pattern during an ultrasound evaluation. This diagnostic suspicion may assist in better surgical planning.

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Lee, Whayoung, Josephine HaDuong, Aaron Sassoon, Tuan Dao, and Ali Nael. "A Liver Transplant for Local Control in a Pediatric Patient with Metastatic TFE3-Associated Perivascular Epithelioid Cell Tumor (PEComa) to the Liver." Case Reports in Pathology 2021 (October 5, 2021): 1–5. http://dx.doi.org/10.1155/2021/3924565.

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Perivascular epithelioid cell tumors (PEComas) are rare mesenchymal tumors with widespread distribution throughout the body and unpredictable clinical behavior. Recently, a subset of these tumors has been reported to harbor Transcription Factor E3 (TFE3) gene rearrangement with distinct morphologic and immunophenotypic features. Although limited, these tumors may represent a separate entity from the conventional PEComas and may require different treatment approaches. Surgery is the main treatment option with no clear consensus on systemic therapy. Here, we present the first case of a malignant pediatric colonic TFE3-associated PEComa with isolated liver metastasis leading to liver transplantation for the local control.

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Lee, Whayoung, Josephine HaDuong, Aaron Sassoon, Tuan Dao, and Ali Nael. "A Liver Transplant for Local Control in a Pediatric Patient with Metastatic TFE3-Associated Perivascular Epithelioid Cell Tumor (PEComa) to the Liver." Case Reports in Pathology 2021 (October 5, 2021): 1–5. http://dx.doi.org/10.1155/2021/3924565.

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Perivascular epithelioid cell tumors (PEComas) are rare mesenchymal tumors with widespread distribution throughout the body and unpredictable clinical behavior. Recently, a subset of these tumors has been reported to harbor Transcription Factor E3 (TFE3) gene rearrangement with distinct morphologic and immunophenotypic features. Although limited, these tumors may represent a separate entity from the conventional PEComas and may require different treatment approaches. Surgery is the main treatment option with no clear consensus on systemic therapy. Here, we present the first case of a malignant pediatric colonic TFE3-associated PEComa with isolated liver metastasis leading to liver transplantation for the local control.

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Niu, Huilin, F. W. Wang, Paul J. Zhang, and Zhanyong Bing. "Cardiac Epithelioid PEComa: Report of Two Cases and Review of the Literature." Case Reports in Medicine 2012 (2012): 1–6. http://dx.doi.org/10.1155/2012/521678.

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Cardiac PEComa is very rare. We reported two cases of epithelioid PEComas, one in an adult and one in a 2-year-old child. Both tumors were composed of sheets of epithelioid cells with coagulation necrosis. In addition, the adult case showed marked nuclear atypia and high mitotic activity with atypical mitosis and the pediatric case showed unusual clear cell features. Immunohistochemically, both tumors were positive for HMB-45 and SMA and negative for S100 and cytokeratin. Electron microscopy was performed in the pediatric case and showed premelanosomes. The adult patient developed extensive metastasis indicating malignant behavior. Prior to the two cases, only 5 other cases of cardiac PEComa were reported and the literatures are reviewed.

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Iyengar, Pratibha, Dan D. DeAngelis, Mark Greenberg, and Glenn Taylor. "Perivascular Epithelioid Cell Tumor of the Orbit: A Case Report and Review of the Literature." Pediatric and Developmental Pathology 8, no. 1 (January 2005): 98–104. http://dx.doi.org/10.1007/s10024-004-5055-0.

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We present a unique case of a perivascular epithelioid cell tumor (PEComa) in the orbit of a 9-year-old female patient. The entity of PEComas has been described only recently. Characteristic histologic features and an immunohistochemical profile of negativity for epithelial markers and positivity for melanogenesis-related markers define the tumors. In children and young adults, this tumor has a predilection for the falciform ligament and ligamentum teres of the liver. It is associated with, but not exclusive to, tuberous sclerosis. To the best of our knowledge, this is the first reported case of a PEComa of the orbit in a child or adult. The main differential diagnoses for this melanin pigment-producing lesion include melanoma and pigmented paraganglioma. The histologic features, immunohistochemical profile, ultra-structural studies, and molecular studies led us to favor a diagnosis of PEComa. The prognosis of this entity is undetermined due largely to the small number of reported cases.

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Lombardi, Welington, Luciana Borges Lombardi, Marina Abud Ferreira, Flávia Vicentin Silva, Silvia Paino Ruas, Fernando Pereira Vanni, Caroline Barbieri Rodrigues, and Luana Albaricci Carreon Fernandes. "PECOMA DE COLO UTERINO: UM RARO RELATO DE CASO." Relatos de Casos Cirúrgicos do Colégio Brasileiro de Cirurgiões 7, no. 3 (September 30, 2021): 1–5. http://dx.doi.org/10.30928/2527-2039e-20213036.

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Introdução: Os Pecomas são tumores de células epitelióides, de origem mesenquimal, normalmente benignos e extremamente raros. São encontrados mais frequentemente no retroperitônio, em região abdominopélvica, no trato gastrointestinal e no trato ginecológico, podendo ser encontrada também em tecidos moles e ossos. Recentemente foram publicados aproximadamente 100 casos de Pecoma, e cerca de 30% desses são de origem uterina, sendo o corpo do útero a região mais acometida. Em sua grande maioria possuem comportamento benigno, sendo raro o acometimento de estruturas ou órgãos vizinhos. Relato do caso: Trata-se de uma paciente de 38 anos, que apresentava sangramento uterino persistente, volume uterino aumentado e anemia associada. O ultrassom transvaginal mostrava a presença de vários nódulos sugestivos de leiomiomas. Após tratamento cirúrgico com histerectomia abdominal total, o anatomopatológico confirmou a presença de miomatose uterina, neoplasia intraepitelial cervical grau III e Pecoma de colo uterino. O exame de imunohistoquímica confirmou o diagnóstico de Pecoma, através da positividade para HMB-45, no achado cirúrgico. Conclusão: Portanto, por haver um número pequeno de relatos e estudos sobre o diagnóstico e tratamento do Pecoma, é importante e relevante estudarmos mais sobre essa afecção para melhorar os níveis de evidências e conhecimento sobre tal doença.

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Sadeghi, Negin, Sarah Smyth, Stephen Damato, and Hooman Soleymani majd. "Perivascular epithelioid cell tumour and investigation of genetic susceptibility." BMJ Case Reports 15, no. 11 (November 2022): e250460. http://dx.doi.org/10.1136/bcr-2022-250460.

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A patient in her 60s was referred to be investigated for an incidental large uterus with a history of renal cell carcinoma and melanoma. Uterine biopsy revealed features of perivascular epithelioid cell tumours (PEComas) and she underwent total abdominal hysterectomy and bilateral salpingo-oophorectomy. Final histology confirmed PEComa with malignant features. Genomic studies did not reveal any deleterious germline variants; however, in view of her history, she is now under a 6-month follow-up with gynaecology-oncology. PEComas are rare tumours associated with tuberous sclerosis and melanoma, sharing genetic abnormalities. Gynaecological PEComas usually present with no or non-specific symptoms. Preoperative investigations are often misleading. Final histology and immunohistochemistry have overlapping features with smooth muscle tumours. Although rare, PEComas need to be treated aggressively to minimise the potential risk of spread. There is currently little evidence about further adjuvant treatment and no clear follow-up protocol. However, the literature suggests that the prognosis is generally good.

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Petrides, Christos, Kyriakos Neofytou, and Aamir Z. Khan. "Pancreatic Perivascular Epithelioid Cell Tumour Presenting with Upper Gastrointestinal Bleeding." Case Reports in Oncological Medicine 2015 (2015): 1–4. http://dx.doi.org/10.1155/2015/431215.

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PEComa is a family of rare mesenchymal tumours which can occur in any part of the human body. Primary PEComas of the pancreas are extremely rare tumours with uncertain malignant potential. A 17-year-old female was admitted to the hospital due to melena. She required several transfusions. CT scan demonstrated a mass at the head of the pancreas measuring 4.2 cm in maximum diameter. An endoscopic ultrasound showed an ulcerating malignant looking mass infiltrating 50% of the wall of the second part of the duodenum in the region of the ampulla. Multiple biopsies taken showed extensive ulceration with granulation tissue formation and underlying large macrophages without being able to establish a definite diagnosis. We proceeded with pylorus-preserving pancreaticoduodenectomy. The postoperative course of the patient was unremarkable, and she was discharged on the 8th postoperative day. Histology examination of the specimen showed a PEComa of pancreas. Eighteen months after resection the patient is disease free. To the best of our knowledge this is the first time we describe a case of a pancreatic PEComa presenting with massive gastrointestinal bleeding.

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Gan, David Eng Yeow, Rebecca Xin Yi Choy, Harivinthan Sellappan, Firdaus Hayati, and Nornazirah Azizan. "Malignant Subcutaneous Perivascular Epithelioid Cell Tumor of Anterior Abdominal Wall." Oman Medical Journal 36, no. 2 (March 15, 2021): e239-e239. http://dx.doi.org/10.5001/omj.2021.21.

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Perivascular epithelioid cell tumors (PEComas) are a family of rare mesenchymal tumors with discrete histological and immunohistochemical characteristics. Even rarer among them are cutaneous and subcutaneous PEComas. We describe a 34-year-old woman who presented with a large anterior abdominal subcutaneous lesion showing intact overlying skin and no obvious invasion of the abdominal musculature. A wide local excision was performed. Histopathology revealed a solitary tumor measuring 75 × 55 × 90 mm with epithelioid cells in nests with thin fibrovascular septa and spindle cells. Resection margins were clear with no invasion to the skin or rectus sheath. Tumor cells were positive for HMB-45 but negative for other markers. This is the largest subcutaneous PEComa reported to date.

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Paula, Diogo, Maria João Amaral, Joana Madeira, João Simões, André Lázaro, Nuno Silva, and José Guilherme Tralhão. "Rare Encounter: A Case Report of Hepatic Perivascular Epithelioid Cell Tumor – An Uncommon Mesenchymal Tumor in the Liver." Case Reports in Gastroenterology 19, no. 1 (January 24, 2025): 43–51. https://doi.org/10.1159/000543018.

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Introduction: Perivascular epithelioid cell tumor (PEComa) is a rare neoplastic mesenchymal tumor, more frequently found in the uterus, although it can occur in different organs. Hepatic PEComa is extremely rare, with only a few cases described in the literature. Case Presentation: We present a case report of a 33-year-old female patient with a history of macroprolactinoma. She was initially referred to our Department due to a 9-mm hepatic nodule incidentally diagnosed in an abdominal ultrasound in 2018. She was asymptomatic. Follow-up ultrasound showed a growth from 9 mm to 16 mm in 2019 and 30 mm in a liver magnetic resonance imaging (MRI) scan in 2022. The case was discussed in a multidisciplinary team meeting, and since malignant transformation or hepatocellular carcinoma could not be ruled out, the decision was to undergo hepatic resection. An open hepatic subsegmentectomy of segment 5 was performed, with uneventful postoperative period. The definitive diagnosis was hepatic PEComa. Conclusion: Hepatic PEComas are rare liver tumors, and their preoperative diagnosis is challenging due to the lack of specific radiological features. In most cases, the diagnosis is only confirmed through histopathological and immunohistochemical studies. Resection of the lesion appears to be the curative treatment; however, due to the rarity of the condition, there are no studies comparing surgical treatment with other options. In our case, the hypervascular lesion was initially misdiagnosed as an adenoma. PEComas should be considered as a differential diagnosis in liver nodules with well-defined margins and increased uptake in the arterial phase in computed tomography or MRI scan. Surgical resection was curative, and no recurrence was detected during the patient’s follow-up.

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Journal articles on the topic 'PECome'

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