Статті в журналах: "Incidental nonfunctioning endocrine pancreatic tumor"

1

Crippa, Stefano, Stefano Partelli, Giuseppe Zamboni, Aldo Scarpa, Domenico Tamburrino, Claudio Bassi, Paolo Pederzoli, and Massimo Falconi. "Incidental diagnosis as prognostic factor in different tumor stages of nonfunctioning pancreatic endocrine tumors." Surgery 155, no. 1 (January 2014): 145–53. http://dx.doi.org/10.1016/j.surg.2013.08.002.

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2

Kim, Daejin, Hanjun Ryu, Hyunsoo Kim, Changkeun Park, Jaekwon Jung, Yunjin Chung, Youngdai Choi, and Woo Jae Lee. "Visible Pancreatic Neuroendocrine Tumor after Water Intake and Position Change." Clinical Ultrasound 6, no. 2 (November 30, 2021): 71–74. http://dx.doi.org/10.18525/cu.2021.6.2.71.

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Most pancreatic neuroendocrine tumors (NETs) are detected incidentally and arise in the endocrine tissues. NETs can secrete hormones and result in clinical syndromes. However, between 50 and 75 percent of pancreatic NETs are nonfunctioning. Ultrasonography shows a well-circumscribed mass with a smooth margin and round or oval hypoechoic shape. A 38-year-old woman visited our hospital with mild upper abdominal discomfort. We visualized an oval hypoechoic mass with inner hyperechogenicity after water intake in the stomach and position change. The patient underwent surgery, and the mass was diagnosed as pancreatic NET.

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3

Haynes, Alex B. "Implications of Incidentally Discovered, Nonfunctioning Pancreatic Endocrine Tumors." Archives of Surgery 146, no. 5 (May 1, 2011): 534. http://dx.doi.org/10.1001/archsurg.2011.102.

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4

Yang, Daohui, Dansong Wang, Yijie Qiu, Xiaofan Tian, Dan Zuo, Yi Dong, Wenhui Lou, and Wenping Wang. "Incidental nonfunctioning pancreatic neuroendocrine tumors: Contrast enhanced ultrasound features in diagnosis1." Clinical Hemorheology and Microcirculation 80, no. 4 (April 6, 2022): 343–52. http://dx.doi.org/10.3233/ch-211269.

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AIM: Preoperative suspicion of malignancy in nonfunctioning pancreatic neuroendocrine tumors (pNETs) is mostly based on tumor size. We retrospectively analyzed the contrast enhanced ultrasound (CEUS) features of a series of histopathologically proved nonfunctioning pNETs. METHODS: In this retrospective study, 37 surgery and histologically proved nonfunctioning pNETs were included. All pNETs lesions were incidentally detected by transabdominal ultrasound. B mode ultrasound (BMUS) and CEUS features were reviewed and analyzed. 52 histopathologically proved pancreatic ductal adenocarcinoma (PDACs) lesions were included as a control group. RESULTS: All nonfunctioning pNETs patients showed no typical clinical symptoms. No significant differences were observed in size, echogenicity or internal color flow imaging signal between pNETs and PDAC patients (P > 0.05). Most of nonfunctioning pNETs showed a well-defined tumor margin. The presence of pancreatic duct dilatation was less frequently observed in nonfunctioning pNETs patients (P < 0.05). After injection of ultrasound contrast agents, homogeneous enhancement was more commonly observed in nonfunctioning pNETs group (P < 0.05). During arterial phase of CEUS, most of nonfunctioning pNETs were hyper- or isoenhanced (32/37, 86.5%), whereas most of PDACs were hypoenhanced (34/52, 65.4%) (P < 0.05). Nonenhanced necrosis area was more commonly detected in PDACs (P = 0.012). CONCLUSIONS: CEUS features are helpful for preoperative non-invasive differential diagnosis of nonfunctioning pNETs, assisting further clinical decision-making process.

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Bettini, Rossella, Stefano Partelli, Letizia Boninsegna, Paola Capelli, Stefano Crippa, Paolo Pederzoli, Aldo Scarpa, and Massimo Falconi. "Tumor size correlates with malignancy in nonfunctioning pancreatic endocrine tumor." Surgery 150, no. 1 (July 2011): 75–82. http://dx.doi.org/10.1016/j.surg.2011.02.022.

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6

Powell, Anathea C., Cristina H. Hajdu, Alec J. Megibow, and Peter Shamamian. "Nonfunctioning Pancreatic Endocrine Neoplasm Presenting as Asymptomatic, Isolated Pancreatic Duct Stricture: A Case Report and Review of the Literature." American Surgeon 74, no. 2 (February 2008): 168–71. http://dx.doi.org/10.1177/000313480807400217.

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Morphologic irregularities of the pancreatic duct are often noted on abdominal imaging studies obtained for unrelated symptoms or conditions. We report the case of a patient who was found to have an incidental, isolated pancreatic duct dilatation on multiple imaging studies and who was found to have a nonfunctioning pancreatic endocrine neoplasm at resection. His prognosis is excellent based on the histology of the lesion and a curative resection. This case highlights the importance of fully investigating incidental pancreatic duct abnormalities regardless of the setting in which they are found.

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Yamaguchi, Tetsuya, Hiroshi Takahashi, Ryuzaburo Kagawa, Ryoji Takeda, Shingo Sakata, Michihiro Yamamoto, and Yoko Iwasa. "Nonfunctioning Pancreatic Endocrine Tumor Presenting with Hemorrhage from Isolated Gastric Varices." American Surgeon 71, no. 12 (December 2005): 1027–30. http://dx.doi.org/10.1177/000313480507101208.

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Hemorrhage from gastric varices due to left-sided portal hypertension is an unusual presentation for pancreatic endocrine tumor. A case of pancreatic endocrine tumor presenting with gastric variceal hemorrhage secondary to left-sided portal hypertension associated with splenic vein occlusion is presented. A 53-year-old man with hemorrhage from isolated gastric varices was referred to our hospital. Laboratory studies revealed normal liver function. Surveys to identify the cause of gastric varices by an abdominal CT, MRCP, and abdominal angiography revealed splenic vein occlusion secondarily attributed to the pancreatic tail tumor and splenomegaly. The pancreatic tumor was suspected to be a resectable endocrine tumor. A distal pancreatectomy, splenectomy, partial resection of the gastric fundus, and limited lymph node dissection were performed. By the histological examination, the diagnosis of nonfunctioning pancreatic endocrine tumor with malignant potential was determined. Three years after the surgery, the patient is doing well and reveals no sign of recurrence. In this case, the unusual presentation for pancreatic endocrine tumors such as a gastric variceal hemorrhage had an advantage that led to early presentation prior to the development of metastases with possible curative surgery.

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8

Toshikuni, Nobuyuki, Kyohei Kai, and Masayoshi Fujisawa. "Nonfunctioning endocrine pancreatic tumor examined with 18F-FDG PET/CT." Annals of Nuclear Medicine 22, no. 2 (February 2008): 133–37. http://dx.doi.org/10.1007/s12149-007-0085-0.

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FURUTANI, Yuichiro, Takuro TERADA, Syusei SANO, Yoshinori MUNEMOTO, Yosiro IIDA, and Takeshi MITSUI. "A Case of Nonfunctioning Pancreatic Endocrine Tumor Presented with Acute Pancreatitis." Nihon Rinsho Geka Gakkai Zasshi (Journal of Japan Surgical Association) 77, no. 7 (2016): 1784–90. http://dx.doi.org/10.3919/jjsa.77.1784.

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10

Yazawa, Naoki, Toshihide Imaizumi, Ken-Ichi Okada, Masahiro Matsuyama, Shoichi Dowaki, Kosuke Tobita, Yasuo Ohtani, Kyoji Ogoshi, Kenichi Hirabayashi, and Hiroyasu Makuuchi. "Nonfunctioning pancreatic endocrine tumor with extension into the main pancreatic duct: Report of a case." Surgery Today 41, no. 5 (May 2011): 737–40. http://dx.doi.org/10.1007/s00595-009-4321-1.

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11

Inagaki, Mitsuhiro, Kazunori Watanabe, Daitaro Yoshikawa, Shigeki Suzuki, Akira Ishizaki, Kakuya Matsumoto, Masakazu Haneda, et al. "A malignant nonfunctioning pancreatic endocrine tumor with a unique pattern of intraductal growth." Journal of Hepato-Biliary-Pancreatic Surgery 14, no. 3 (May 29, 2007): 318–23. http://dx.doi.org/10.1007/s00534-006-1140-2.

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12

Durleshter, V. M., S. R. Genrykh, E. N. Karanadze, A. V. Andreev, R. V. Gedzun, and N. N. Kuzmenko. "Diagnosis of Cystic Endocrine Tumors of the Pancreas. Differential Possibilities of Application of Radiation Methods (Case Report)." Medical Visualization, no. 3 (June 28, 2017): 105–11. http://dx.doi.org/10.24835/1607-0763-2017-3-105-111.

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Nonfunctioning neuroendocrine tumors (NFET) account for up to 33% of the neuroendocrine tumors of the pancreas, ranging from 1 to20 cmin diameter and showing a higher malignancy rate, up to 90%. The clinical presentation of nonfunctioning neuroendocrine tumors is nonspecific. These tumors, in fact, are predominantly characterized by an expansive growth pattern; therefore, they are clinically silent until adjacent viscera and structures are involved. This makes it difficult to diagnose NFET at an early stage. Correct diagnosis is typically delayed by several years. About 15% of pancreatic NFET are cystic and difficult to differentiation from other cystic pancreatic lesions. In such cases, the important role played by hypervascular rim in the arterial phase image. Literature review and case report оf diagnostics and treatment of Neuroendocrine Tumor with cystic transformation are presented in the article.

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13

Slukvin, Igor I., G. Reza Hafez, John E. Niederhuber, and Thomas F. Warner. "Combined Serous Microcystic Adenoma and Well-Differentiated Endocrine Pancreatic Neoplasm: A Case Report and Review of the Literature." Archives of Pathology & Laboratory Medicine 127, no. 10 (October 1, 2003): 1369–72. http://dx.doi.org/10.5858/2003-127-1369-csmaaw.

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Abstract We report a case of combined microcystic adenoma and pancreatic endocrine neoplasm of the pancreas in a 53-year-old male patient. The pancreatic tumor was an incidental computed tomography scan finding and was not accompanied by gastrointestinal symptoms. The tumor was located in the head of the pancreas and was composed of numerous small cysts lined by uniform clear cells with a centrally located solid endocrine component. Four cases of similar neoplasm have recently been reported, exclusively in women. Literature review and case analysis indicate that combined microcystic adenoma and pancreatic endocrine neoplasm is characterized by the presence of pancreatic endocrine neoplasm within microcystic adenoma in the head of the pancreas, affects women more often than men, and presents at a younger age when compared to microcystic adenoma.

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14

Bassaneze, Thiago, Clovis Augusto Borges do Nascimento, Adam Hiar, Laura Silveira Tanisaka, Beatriz Villas-Boas Weffort, Larissa Mariana Ayde, Maria Laura Kachan Bordignon, and Abner Jorge Jácome Barrozo. "SOLID PSEUDOPAPILLARY NEOPLASM OF THE PANCREAS MIMICKING LOW GRADE NEUROENDOCRINE TUMOR." Relatos de Casos Cirúrgicos do Colégio Brasileiro de Cirurgiões 8, no. 1 (March 15, 2022): 1–5. http://dx.doi.org/10.30928/2527-2039e-20223118.

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Introduction: Solid pseudopapillary neoplasms (SPN) and pancreatic neuroendocrine tumors (NET) are rare diseases that are generally incidental findings in imaging tests. SPN is a low-grade tumor with good prognosis that more commonly affects young female patients. Pancreatic NET has a significant variability in outcomes, with low malignant potential in non-progressing tumors that are welldifferentiated. Both tumors may appear very similar in imaging tests and immunohistochemical (IHC) evaluation, which makes the differential diagnosis challenging, especially in small lesions. Case report: The authors present the case of a 34-year-old male with a medical history of a primary mediastinal germ cell tumor. The patient had no symptoms. Follow-up abdominal CT scan evidenced a single, well-delimited nodular lesion in the pancreatic neck, measuring 17mm in diameter. The patient was submitted to an endoscopic ultrasound-guided fine needle aspiration biopsy (EUS-FNA) and after IHC analysis, there was a diagnostic suspicion of low-grade pancreatic NET. Central pancreatectomy (CP) was performed and complete lesion analysis evidenced a pancreatic SPN. Conclusion: The SPN can mimic low-grade nonfunctioning pancreatic NETs in imaging tests and EUSFNA IHC evaluation.

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15

Roldo, Claudia, Edoardo Missiaglia, John P. Hagan, Massimo Falconi, Paola Capelli, Samantha Bersani, George Adrian Calin, et al. "MicroRNA Expression Abnormalities in Pancreatic Endocrine and Acinar Tumors Are Associated With Distinctive Pathologic Features and Clinical Behavior." Journal of Clinical Oncology 24, no. 29 (October 10, 2006): 4677–84. http://dx.doi.org/10.1200/jco.2005.05.5194.

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Purpose We investigated the global microRNA expression patterns in normal pancreas, pancreatic endocrine tumors and acinar carcinomas to evaluate their involvement in transformation and malignant progression of these tumor types. MicroRNAs are small noncoding RNAs that regulate gene expression by targeting specific mRNAs for degradation or translation inhibition. Recent evidence indicates that microRNAs can contribute to tumor development and progression and may have diagnostic and prognostic value in several human malignancies. Materials and Methods Using a custom microarray, we studied the global microRNA expression in 12 nontumor pancreas and 44 pancreatic primary tumors, including 12 insulinomas, 28 nonfunctioning endocrine tumors, and four acinar carcinomas. Results Our data showed that a common pattern of microRNA expression distinguishes any tumor type from normal pancreas, suggesting that this set of microRNAs might be involved in pancreatic tumorigenesis; the expression of miR-103 and miR-107, associated with lack of expression of miR-155, discriminates tumors from normal; a set of 10 microRNAs distinguishes endocrine from acinar tumors and is possibly associated with either normal endocrine differentiation or endocrine tumorigenesis; miR-204 is primarily expressed in insulinomas and correlates with immunohistochemical expression of insulin; and the overexpression of miR-21 is strongly associated with both a high Ki67 proliferation index and presence of liver metastasis. Conclusion These results suggest that alteration in microRNA expression is related to endocrine and acinar neoplastic transformation and progression of malignancy, and might prove useful in distinguishing tumors with different clinical behavior.

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AOTAKE, Toshiharu, Fumie TANAKA, Hidenori FUJII, Yuki HIROSE, Hiroyuki YAMAMOTO, Toshio MATSUSITA, and Fumio KONISHI. "A COMBINED CASE OF NONFUNCTIONING ENDOCRINE TUMOR OF THE PANCREATIC HEAD AND CANCER OF THE PANCREATIC BODY AND TAIL." Nihon Rinsho Geka Gakkai Zasshi (Journal of Japan Surgical Association) 65, no. 7 (2004): 1937–41. http://dx.doi.org/10.3919/jjsa.65.1937.

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17

Hoshino, Arichika, Takayuki Aimoto, Hideyuki Suzuki, Satoshi Mizutani, Hideaki Ishii, Keisuke Mishima, Yudai Wada, et al. "A Case of Nonfunctioning Pancreatic Endocrine Tumor with Atypical Imaging Findings due to Prominent Fibrosis of the Tumor Stroma." Journal of Nippon Medical School 81, no. 3 (2014): 179–85. http://dx.doi.org/10.1272/jnms.81.179.

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Hoshino, Arichika, Takayuki Aimoto, Hideyuki Suzuki, Satoshi Mizutani, Yoshiharu Nakamura, Hideaki Ishii, Seiichi Satoh, Hidemi Oba, Tetsuo Sibuya, and Eiji Uchida. "A Case of Nonfunctioning Pancreatic Endocrine Tumor with Atypical Imaging Findings due to Prominent Fibrosis of the Tumor Stroma." Journal of Nippon Medical School 81, no. 5 (2014): 346–52. http://dx.doi.org/10.1272/jnms.81.346.

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19

Ikenaga, Naoki, Kazuyoshi Nishihara, Fujio Katsumoto, Hiroaki Matsunaga, Toshihumi Nasu, Yumi Oshiro, Mari Nakamori, Satoshi Toyoshima, Minoru Ono, and Shoshu Mitsuyama. "A Minute Nonfunctioning Pancreatic Endocrine Tumor with Ductal Structures and Prominent Fibrous Stroma: Report of a Case." Japanese Journal of Gastroenterological Surgery 38, no. 6 (2005): 673–78. http://dx.doi.org/10.5833/jjgs.38.673.

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20

Sadowski, S. M., C. R. C. Pieterman, N. D. Perrier, F. Triponez, and G. D. Valk. "Prognostic factors for the outcome of nonfunctioning pancreatic neuroendocrine tumors in MEN1: a systematic review of literature." Endocrine-Related Cancer 27, no. 6 (June 2020): R145—R161. http://dx.doi.org/10.1530/erc-19-0372.

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Metastatic duodenopancreatic neuro-endocrine tumors (dpNETs) are the most important disease-related cause of death in patients with multiple endocrine neoplasia type 1 (MEN1). Nonfunctioning pNETs (NF-pNETs) are highly prevalent in MEN1 and clinically heterogeneous. Therefore, management is controversial. Data on prognostic factors for risk stratification are limited. This systematic review aims to establish the current state of evidence regarding prognostic factors in MEN1-related NF-pNETs. We systematically searched four databases for studies assessing prognostic value of any factor on NF-pNET progression, development of distant metastases, and/or overall survival. In- and exclusion, critical appraisal and data-extraction were performed independently by two authors according to pre-defined criteria. Thirteen studies (370 unique patients) were included. Prognostic factors investigated were tumor size, timing of surgical resection, WHO grade, methylation, p27/p18 expression by immunohistochemistry (IHC), ARX/PDX1 IHC and alternative lengthening of telomeres. Results were complemented with evidence from studies in MEN1-related pNET for which data could not be separately extracted for NF-pNET and data from sporadic NF-pNET. We found that the most important prognostic factors used in clinical decision making in MEN1-related NF-pNETs are tumor size and grade. NF-pNETs <2 cm may be managed with watchful waiting, while surgical resection is advised for NF-pNETs ≥2 cm. Grade 2 NF-pNETs should be considered high risk. The most promising and MEN1-relevant avenues of prognostic research are multi-analyte circulating biomarkers, tissue-based molecular factors and imaging-based prognostication. Multi-institutional collaboration between clinical, translation and basic scientists with uniform data and biospecimen collection in prospective cohorts should advance the field.

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21

Tzontcheva, Anna. "Neuroendocrine Tumors — Laboratory Diagnosis." Journal of Medical Biochemistry 29, no. 4 (October 1, 2010): 254–64. http://dx.doi.org/10.2478/v10011-010-0028-5.

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Neuroendocrine Tumors — Laboratory DiagnosisNeuroendocrine tumors (NETs) are a heterogeneous group of neoplasms originating from endocrine cells, which are characterized by the presence of secretory granules as well as the ability to produce biogenic amines and polypeptide hormones. These tumors originate from endocrine glands such as the adrenal medulla, the pituitary, and the parathyroids, as well as endocrine islets within the thyroid or the pancreas, and dispersed endocrine cells in the respiratory and gastrointestinal tract. The clinical behavior of NETs is extremely variable; they may be functioning or not functioning, ranging from very slow-growing tumors (well-differentiated NETs), which are the majority, to highly aggressive and very malignant tumors (poorly differentiated NETs). Classically, NETs of the gastrointestinal tract are classified into 2 main groups: (1) carcinoids and (2) endocrine pancreatic tumors (EPTs). Most neuroendocrine tumors produce and secrete a multitude of peptide hormones and amines. Some of these substances cause a specific clinical syndrome: carcinoid, Zollinger-Ellison, hyperglycemic, glucagonoma and WDHA syndrome. Specific markers for these syndromes are basal and/or stimulated levels of urinary 5-HIAA, serum or plasma gastrin, insulin, glucagon and vasoactive intestinal polypeptide, respectively. Some carcinoid tumors and about one third of endocrine pancreatic tumors do not present any clinical symptoms and are called ‘nonfunctioning’ tumors. Therefore, general tumor markers such as chromogranin A, pancreatic polypeptide, serum neuron-specific enolase and subunits of glycoprotein hormones have been used for screening purposes in patients without distinct clinical hormone-related symptoms. Among these general tumor markers chromogranin A, although its precise function is not yet established, has been shown to be a very sensitive and specific serum marker for various types of neuroendocrine tumors. This is because it may also be elevated in many cases of less well-differentiated tumors of neuroendocrine origin that do not secrete known hormones. At the moment, chromogranin A is considered the best general neuroendocrine serum or plasma marker available both for diagnosis and therapeutic evaluation, and is increased in 50-100% of patients with various neuroendocrine tumors. Chromogranin A serum or plasma levels reflect tumor load, and it may be an independent marker of prognosis in patients with midgut carcinoids.

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22

Khomiak, I. V., O. V. Duvalko, A. I. Khomiak, I. S. Tereshkevich, and A. V. Malik. "Laparoscopic resections for pancreatic neuroendocrine tumors: case series and discussion." Herald of Pancreatic Club 45, no. 4 (October 30, 2019): 39–43. http://dx.doi.org/10.33149/vkp.2019.04.05.

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Neuroendocrine tumor of the pancreas (pNET) is a wide-spread oncological disease, and its treatment is a widely discussed topic in modern pancreatology. Functioning pNET mainly manifest with hormonal hypersecretion syndrome (accordingly to the type of hormone), whereas nonfunctioning tumors may not produce any symptoms and remain unnoticed, complicating the diagnosis and postponing medical care. Course of the disease and patient’s prognosis largely depend on the stage of the disease and tumor histology. Higher tumor grade, lymph node and liver metastasis, and a larger primary tumor generally portend relatively poor survival. Prognosis of the patients with pNET improves substantially when the disease has been diagnosed and treated on the early stages. Endocrine testing, imaging modalities among which CT, MRI and endoscopic ultrasound are considered most useful; and histological evidence are all required to accurately diagnose pNETs. Recently an “aggressive” approach to pNET treatment has become most popular in academic centers throughout the world. Surgical resection of the tumor is considered the most effective treatment option and a gold treatment standard. Laparoscopic pancreatic resections also play major role in the treatment of pNET. Laparoscopic approach is safe and effective modality, so the number of laparoscopic operations has been growing last years. Among the benefits of minimally invasive surgery are lesser postoperative pain, shorter hospital length of stay, better cosmetic appearance and shorter disease-related inability of work period. In these case series characteristics of the early diagnosis and successful laparoscopic surgical treatment for the patients with pNET of the right anatomical segment of the pancreas are described.

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Orlefors, H., A. Sundin, U. Garske, C. Juhlin, K. Oberg, B. Skogseid, B. Langstrom, M. Bergstrom, and B. Eriksson. "Whole-Body 11C-5-Hydroxytryptophan Positron Emission Tomography as a Universal Imaging Technique for Neuroendocrine Tumors: Comparison with Somatostatin Receptor Scintigraphy and Computed Tomography." Journal of Clinical Endocrinology & Metabolism 90, no. 6 (June 1, 2005): 3392–400. http://dx.doi.org/10.1210/jc.2004-1938.

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Neuroendocrine tumors (NETs) can be small and situated almost anywhere throughout the body. Our objective was to investigate whether whole-body (WB) positron emission tomography (PET) with 11C-5-hydroxytryptophan (5-HTP) can be used as a universal imaging technique for NETs and to compare this technique with established imaging methods. Forty-two consecutive patients with evidence of NET and a detected lesion on any conventional imaging (six bronchial, two foregut, 16 midgut, and two thymic carcinoids; one ectopic Cushing’s syndrome; four gastrinomas; one insulinoma; six nonfunctioning endocrine pancreatic tumors; one gastric carcinoid, one paraganglioma; and two endocrine-differentiated pancreatic carcinomas) were studied. The WB-11C-5-HTP-PET examinations were compared with WB-computed tomography (CT) and somatostatin receptor scintigraphy (SRS). Tumor lesions were imaged with PET in 95% of the patients. In 58% of the patients, PET could detect more lesions than SRS and CT and equal numbers in 34%, whereas in three cases, SRS or CT showed more lesions. In 84% (16 of 19 patients), PET could visualize the primary tumor compared with 47 and 42% for SRS and CT, respectively. The surgically removed PET-positive primary tumor sizes were 6–30 mm. To conclude, this study indicates that WB-11C-5-HTP-PET can be used as a universal imaging method for detection of NETs. This study also shows that WB-11C-HTP-PET is sensitive in imaging small NET lesions, such as primary tumors, and can in a majority of cases image significantly more tumor lesions than SRS and CT.

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Boninsegna, L., S. Crippa, S. Partelli, A. Scarpa, G. Zamboni, and M. Falconi. "OC.01.6 INCIDENTAL DIAGNOSIS AS PROGNOSTIC FACTOR IN DIFFERENT TUMOR STAGES OF NON-FUNCTIONING PANCREATIC ENDOCRINE TUMORS." Digestive and Liver Disease 45 (March 2013): S60. http://dx.doi.org/10.1016/s1590-8658(13)60162-0.

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25

Pieterman, C. R. C., E. B. Conemans, K. M. A. Dreijerink, J. M. de Laat, H. Th M. Timmers, M. R. Vriens, and G. D. Valk. "Thoracic and duodenopancreatic neuroendocrine tumors in multiple endocrine neoplasia type 1: natural history and function of menin in tumorigenesis." Endocrine-Related Cancer 21, no. 3 (January 3, 2014): R121—R142. http://dx.doi.org/10.1530/erc-13-0482.

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Mutations of the multiple endocrine neoplasia type 1 (MEN1) gene lead to loss of function of its protein product menin. In keeping with its tumor suppressor function in endocrine tissues, the majority of the MEN1-related neuroendocrine tumors (NETs) show loss of heterozygosity (LOH) on chromosome 11q13. In sporadic NETs,MEN1mutations and LOH are also reported, indicating common pathways in tumor development. Prevalence of thymic NETs (thNETs) and pulmonary carcinoids in MEN1 patients is 2–8%. Pulmonary carcinoids may be underreported and research on natural history is limited, but disease-related mortality is low. thNETs have a high mortality rate. Duodenopancreatic NETs (dpNETs) are multiple, almost universally found at pathology, and associated with precursor lesions. Gastrinomas are usually located in the duodenal submucosa while other dpNETs are predominantly pancreatic. dpNETs are an important determinant of MEN1-related survival, with an estimated 10-year survival of 75%. Survival differs between subtypes and apart from tumor size there are no known prognostic factors. Natural history of nonfunctioning pancreatic NETs needs to be redefined because of increased detection of small tumors. MEN1-related gastrinomas seem to behave similar to their sporadic counterparts, while insulinomas seem to be more aggressive. Investigations into the molecular functions of menin have led to new insights into MEN1-related tumorigenesis. Menin is involved in gene transcription, both as an activator and repressor. It is part of chromatin-modifying protein complexes, indicating involvement of epigenetic pathways in MEN1-related NET development. Future basic and translational research aimed at NETs in large unbiased cohorts will clarify the role of menin in NET tumorigenesis and might lead to new therapeutic options.

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Kennedy, Randol, and Daniela Ciltea. "PSUN279 Refractory Hypoglycemia in the Setting of Incidental Urothelial Carcinoma." Journal of the Endocrine Society 6, Supplement_1 (November 1, 2022): A403—A404. http://dx.doi.org/10.1210/jendso/bvac150.839.

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Abstract Introduction Hypoglycemia is uncommon in individuals who do not have drug-treated diabetes mellitus. In such patients, differentials include cortisol deficiency, insulinomas or autoantibodies against insulin/ insulin receptor. Malignancy related causes include non-islet cell tumors and extensive liver infiltration. This case highlights the workup of hypoglycemia in a patient with an incidental diagnosis of urothelial cancer. Clinical Case We present a 62 year old male who was assessed by the EMS at home for difficulty in arousal and facial drooling. Initial assessment found him to have a random glucose of 26mg/dL. His glucose was corrected with dextrose and the EMS left. Nine hours later the EMS was called again strange behavior, where he was found again to be hypoglycemic (19mg/dL). Glucagon and dextrose was administered and he was transported to the ED. His finger stick glucose on arrival was 26mg/dL. Endocrine history is primary hypothyroidism only. Other significant past medical history was a retroperitoneal mass seen on routine surveillance imaging one month ago, after being treated for renal cell carcinoma with right partial nephrectomy 10 years ago. On physical exam, vitals were stable, he was alert and oriented. Other physical examination findings were normal. His BMI was 35.23 kg/m2. Relevant initial labs were venous blood glucose 43 mg/dL, TSH 11.4 uU/mL and T4 was 1.1 ng/dL. Blood ethanol level was undetectable. Despite receiving multiple ampules of 50% dextrose and continuous 10-20% dextrose IV infusion he continued to be hypoglycemic and therefore was admitted to the ICU. The initial management course was as follows: He was started on octreotide infusion and given scheduled diazoxide for persistent hypoglycemia with no improvement. For suspected non-islet cell tumor, he was started on glucagon infusion, however there was no significant correction. Calorie intake was monitored and increased by nutrition and was subsequently given enteral/ parenteral nutrition. He was also started on hydrocortisone 50mg every 8 hours. Endocrine investigations were as follows: Insulin, C-peptide, urine sulfonylureas and ß-hydroxybutyrate were undetectable, proinsulin 2.9 pmol/L (ref <= 8.0 pmol/L), Insulin antibody negative, IGF-1 44 ng/mL (ref 49-214 ng/mL), IGF-2 129 ng/dL (IGF-II/IGF-I < 10). Random cortisol was 26 µg/dL. Investigations for the retroperitoneal mass were as follows: a biopsy showed high grade invasive urothelial carcinoma. MRI abdomen showed extensive and diffuse infiltrative metastatic liver disease without evidence of pancreatic endocrine tumor. The final diagnosis was determined to be due to extensive tumor infiltration in the liver. His condition deteriorated during ICU stay and comfort measures were initiated; subsequently he deceased. Conclusion The causes of hypoglycemia in patients with known tumors include extensive tumor infiltration and nonislet cell tumors, two uncommon causes of hypoglycemia. Differentiating between nonislet tumor secretion and direct tumor infiltration is based on IGF-2 level and the IGF-2/IGF-1 ratio. Presentation: Sunday, June 12, 2022 12:30 p.m. - 2:30 p.m.

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FUJIMOTO, Koji, Hiroshi HIGASHIYAMA, Tatsuki ARIMITSU, Takashi KOMATSUBARA, Naoki KOIZUMI, Takatomo KOSHIBA, Hiroyasu NISHIZAWA, Shigeru SAKANO, Rieko ITOH, and Masayuki YAMAMOTO. "A case of multiple nonfunctioning pancreatic endocrine tumors occurring at the same site 10 years after enucleation for the calcitonin-secreting endocrine tumor of the head of the pancreas." Suizo 27, no. 5 (2012): 716–24. http://dx.doi.org/10.2958/suizo.27.716.

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Jeune, F., A. Taibi, and S. Gaujoux. "Update on the Surgical Treatment of Pancreatic Neuroendocrine Tumors." Scandinavian Journal of Surgery 109, no. 1 (January 24, 2020): 42–52. http://dx.doi.org/10.1177/1457496919900417.

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Background and Aims: Pancreatic neuroendocrine tumors (PNET) arise from uncontrolled proliferation of neuroendocrine cell and further genetic alterations that may induce hormone secretion such as glucagon/insulin/gastrin/VIP. Their incidence is rapidelly growing, especially because of the frequent incidental diagnosis of small asymptomatic non-functionnal neuroendocrine tumors with the widespread use of cross-sectional imaging. The vast majority of pancreatic neuroendocrine tumors are sporadic but up to 5%–10% of them arise from genetic syndromes, the main one being Multiple Endocrine Neopalsm type 1 (MEN1). Appropriate management of patients with PNET is a complex challenge for surgeons, and require extensive medical collaboration. This review aims to summarize major and recent updates regarding the medico-surgical management of PNETs. Material and Methods: Review of pertinent English language literature. Results: This article provides a concise summary of the clinical presentation, diagnosis, surgical management, alternative treatments and follow up of PNETs. Conclusion: PNET are a rare, heterogeneous group of neoplasms with a generally favorable prognosis at least compared to pancreatic adenocarcinoma. Surgical resection is the cornerstone of their management, particularly for localized disease, and should always be discussed in multidisciplinary tumor board.

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Marchegiani, Giovanni, Luca Landoni, Stefano Andrianello, Gaia Masini, Sara Cingarlini, Mirko D’Onofrio, Riccardo De Robertis, et al. "Patterns of Recurrence after Resection for Pancreatic Neuroendocrine Tumors: Who, When, and Where?" Neuroendocrinology 108, no. 3 (November 27, 2018): 161–71. http://dx.doi.org/10.1159/000495774.

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Background/Aims: Pancreatic neuroendocrine tumors (pan-NENs) represent an increasingly common indication for pancreatic resection, but there are few data regarding possible recurrence after surgery. The aim of the study was to describe the frequency, timing, and patterns of recurrence after resection for pan-NENs with consequent implications for postoperative follow-up. Methods: We performed a retrospective analysis of pan-NENs resected between 1990 and 2015 at The Pancreas Institute, University of Verona Hospital Trust. Predictors of recurrence were assessed. Survival analysis was conducted using the Kaplan-Meier and conditional survival (CS) methods. Results: The cohort consisted of 487 patients with a median follow-up of 71 months. Recurrence developed in 12.3%: 54 (11.1%) liver metastases, 11 (2.3%) local recurrence, 10 (2.1%) nodal recurrence, and 8 (1.6%) metastases in other organs. Thirty-one (6.4%) died due to disease recurrence. Size > 21 mm, G3 grade, nodal metastasis, and vascular infiltration were independent predictors of overall recurrence. Recurrence occurred either during the first year of follow-up (n = 9), or after 10 years (n = 4). CS analysis revealed that nonfunctioning G1 pan-NEN ≤20 mm without nodal metastasis or vascular invasion had a negligible risk of developing recurrence. In the present series, after 5 years of follow-up without developing recurrence, tumor recurrence occurred only in the form of liver metastases. Conclusions: Recurrence of pan-NENs is rare and is predicted by tumor size, nodal metastasis, grading, and vascular invasion. Patients with G1 pan-NEN without nodal metastasis and vascular invasion may be considered cured by surgery. After 5 years without recurrence, follow-up should focus on excluding the development of liver metastases.

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Santucci, Nicolas, Sébastien Gaujoux, Christine Binquet, Cynthia Reichling, Jean-Christophe Lifante, Bruno Carnaille, François Pattou, et al. "Pancreatoduodenectomy for Neuroendocrine Tumors in Patients with Multiple Endocrine Neoplasia Type 1: An AFCE (Association Francophone de Chirurgie Endocrinienne) and GTE (Groupe d’étude des Tumeurs Endocrines) Study." World Journal of Surgery 45, no. 6 (March 1, 2021): 1794–802. http://dx.doi.org/10.1007/s00268-021-06005-7.

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Abstract Aim To assess postoperative complications and control of hormone secretions following pancreatoduodenectomy (PD) performed on multiple endocrine neoplasia type 1 (MEN1) patients with duodenopancreatic neuroendocrine tumors (DP-NETs). Background The use of PD to treat MEN1 remains controversial, and evaluating the right place of PD in MEN1 disease makes sense. Methods Thirty-one MEN1 patients from the Groupe d’étude des Tumeurs Endocrines MEN1 cohort who underwent PD for DP-NETs between 1971 and 2013 were included. Early and late postoperative complications, secretory control and overall survival were analyzed. Results Indication for surgery was: Zollinger–Ellison syndrome (n = 18; 58%), nonfunctioning tumor (n = 9; 29%), insulinoma (n = 2; 7%), VIPoma (n = 1; 3%) and glucagonoma (n = 1; 3%). Mean follow-up was 141 months (range 0–433). Pancreatic fistulas occurred in 5 patients (16.1%), distant metastases in 6 (mean onset of 43 months; range 13–110 months), postoperative diabetes mellitus in 7 (22%), and pancreatic exocrine insufficiency in 6 (19%). Five-year overall survival was 93.3% [CI 75.8–98.3] and ten-year overall survival was 89.1% [CI 69.6–96.4]. After a mean follow-up of 151 months (range 0–433), the biochemical cure rate for MEN-1 related gastrinomas was 61%. Conclusion In MEN1 patients, pancreatoduodenectomy can be used to control hormone secretions (gastrin, glucagon, VIP) and to remove large NETs. PD was found to control gastrin secretions in about 60% of cases.

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Paiella, Salvatore, Giovanni Marchegiani, Marco Miotto, Anna Malpaga, Harmony Impellizzeri, Greta Montagnini, Tommaso Pollini, et al. "Are Cystic Pancreatic Neuroendocrine Tumors an Indolent Entity Results from a Single-Center Surgical Series." Neuroendocrinology 106, no. 3 (June 7, 2017): 234–41. http://dx.doi.org/10.1159/000477849.

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Introduction: Cystic pancreatic neuroendocrine tumors (CPanNETs) represent an uncommon variant of pancreatic neuroendocrine tumors (PanNETs). Due to their rarity, there is a lack of knowledge with regard to clinical features and postoperative outcome. Methods: The prospectively maintained surgical database of a high-volume institution was queried, and 46 resected CPanNETs were detected from 1988 to 2015. Clinical, demographic, and pathological features and survival outcomes of CPanNETs were described and matched with a population of 92 solid PanNETs (SPanNETs) for comparison. Results: CPanNETs accounted for 7.8% of the overall number of resected PanNETs (46/587). CPanNETs were mostly sporadic (n = 42, 91%) and nonfunctioning (39%). Two functioning CPanNETs were detected (4.3%), and they were 2 gastrinomas. The median tumor diameter was 30 mm (range 10-120). All tumors were well differentiated, with 38 (82.6%) G1 and 8 (17.4%) G2 tumors. Overall, no CPanNET showed a Ki-67 >5%. A correct preoperative diagnosis of a CPanNET was made in half of the cases. After a median follow-up of >70 months, the 5- and 10-year overall survival of resected CPanNETs was 93.8 and 62.5%, respectively, compared to 92.7 and 84.6% for SPanNETs (p > 0.05). The 5- and 10-year disease-free survival rates were 94.5 and 88.2% for CPanNETs and 81.8 and 78.9% for SPanNETs, respectively (p > 0.05). Conclusion: In the setting of a surgical cohort, CPanNETs are rare, nonfunctional, and well-differentiated neoplasms. After surgical resection, they share the excellent outcome of their well-differentiated solid counterparts for both survival and recurrence.

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Pradeep, Pallavi, and Yuval Eisenberg. "A Rare Presentation of Multiple Endocrine Neoplasia Type 1." Journal of the Endocrine Society 5, Supplement_1 (May 1, 2021): A988—A989. http://dx.doi.org/10.1210/jendso/bvab048.2022.

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Abstract Background: Multiple endocrine neoplasia type 1 (MEN1) is a rare, autosomal dominant inherited syndrome caused by mutations in the MEN1 tumor suppressor gene with a reported incidence of 2 in 100,000. Clinical Case: A 26-year-old Caucasian female was seen for surveillance screening given positive familial mutation in the MEN1 gene. She had a significant family history of pathogenic MEN1 in her son, brother, father and paternal aunt. On presentation, she denied any history of headache, nipple discharge, kidney stones, fractures, heart burn, abdominal pain, diarrhea, hypoglycemia, flushing, lightheadedness. She was on hormonal IUD for contraception and had not had menstrual cycles for the last 6 months. Vital signs and physical examination were unremarkable. Her initial evaluation included a normal calcium of 9.8mg/dL (RR:8.6-10.6mg/dL), PTH of 70pg/ml (RR:12-88pg/ml), 25OH-vitamin-D of 11ng/ml (RR:20-80ng/ml). She had normal gastrin, chromogranin A, glucagon, and vasoactive intestinal peptide levels. Prolactin was 17.6ng/ml (RR:3.3-26.7 ng/ml). MRI pituitary showed a 6mm lesion representing a Rathke’s cleft cyst, not compressing surrounding structures. Two months following the initial encounter, she presented with abdominal pain, nausea, vomiting. She was found to have an obstructing renal stone requiring stent placement. Calcium was 10.1mg/dl. She was also noted to have an incidental pancreatic tail mass of 4.2cm on CT abdomen. She underwent laparoscopic robotic distal spleno-pancreatectomy. Surgical pathology showed a well differentiated 3.5cm neuroendocrine tumor with negative margins, which stained positively for insulin. She had not reported symptoms of weight gain or any episodes suspicious for sympathoadrenal activation or neuroglycopenia, and was again confirmed after surgery. Patient was re-admitted to the hospital for right-sided flank pain and was found to have a new 6mm obstructing calculus with moderate hydronephrosis of the right kidney. Corrected calcium level was found to be mildly elevated at 11.1mg/dL. Repeat PTH was stable at 81pg/ml, and urinary calcium was elevated at 447.3mg/24h (RR:50-250mg/24h). Subsequently, she underwent 3.5 gland parathyroidectomy and thymectomy. On POD20, PTH was 29pg/ml and calcium was 8.6mg/dl. Conclusion: This case highlights a rare presentation of MEN1 with an asymptomatic insulinoma and nephrolithiasis, despite having normal calcium and PTH levels initially. Primary hyperparathyroidism is the most common presentation of MEN1, seen in up to 90% of patients. The second most common finding in MEN1 patients, reported in 70% of the cases, are entero-pancreatic tumors, the most common being gastrinoma. Insulinoma, which is less common and is seen in only 10-30% patients, needs documentation of Whipple’s triad. Interestingly, our patient never had any history of hypoglycemia.

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Paniagua, Gabriela Zuniga, Samarth Vimarni, Dominique Broutin, Suzanne Martinez, and Sowmya K. Suryanarayanan. "A Tale of Two Cities: Do They Have the Same Destination: Asymptomatic Pheochromocytomas: Biochemically Functioning vs Non Functioning." Journal of the Endocrine Society 5, Supplement_1 (May 1, 2021): A106. http://dx.doi.org/10.1210/jendso/bvab048.213.

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Abstract Background: Pheochromocytomas (PHEOs) are enterochromaffin tumors arising from the adrenal gland. Their diagnosis and preoperative preparation is crucial due to high morbidity and mortality rates with unrecognized, undiagnosed PHEOs. Case 1: 62-year-old male with a medical history of HCC, noted to have right adrenal adenoma measuring 1.7 x 1.6 cm. Denied any symptoms and normotensive on exam. A PET scan done showed a hypermetabolic right adrenal nodule concerning for malignancy. Serum metanephrines were 45 pg/ml (nl <57 pg/ml) and total plasma metanephrines were 172 pg/ml (nl <205 pg/ml). A CT guided biopsy was consistent with a PHEO. Other labs included: 24 hour urine metanephrines: 155 mcg (nl 90–315), total metanephrines: 520 (nl 224–832) and vanillylmandelic acid was 3.6 (nl <6.0). 24-hour urine epinephrine: 10 mcg (nl 2–24), norepinephrine:57 mcg (nl 15–100) and dopamine: 421 (normal 52–480). Case 2: 55 year old male with UTI and flank discomfort, noted to have incidental 8cm Right adrenal mass noted concerning for malignancy. Also denied any symptoms and normotensive. Plasma fractionated metanephrines 938 (ref <206), metanephrine 279,Normetanephrine 659(ref <148), 24 hr urine metanephrines=1176mcg/24 hr (90–315), Normetanephrines 1487 (122–676), 24 hr urine total metanephrines 2663 (224–832). He is refusing α and β blockade due to normotension in preparation for surgery. Discussion: It is important to suspect, confirm, localize, treat, and PHEOs for several reasons. Most of these tumors hypersecrete catecholamines, and if untreated, cardiovascular morbidity and mortality are high. Another reason to encourage case detection is that, for familial disease, detection of a tumor in the proband may result in earlier diagnosis and treatment in other family members. Alpha-blockade is usually used prior to resection of Pheochromocytomas. However, the data available in the literature regarding alpha blockade for “truly asymptomatic” functioning or non functioning Pheochromocytomas is scarce. However what is unique to our cases is that they both are normotensive and asymptomatic and refusing preoperative preparation. Conclusion: Asymptomatic PHEO are becoming more common presentation given Pheochromocytomas are rare. Are the genetics and biochemical nature any different than classic pheos? Are we supposed to manage them the same way? Would we rethink current guidelines for managing normotensive or nonfunctioning pheochromocytomas pre operatively? We also would like guidelines on how to prepare such truly asymptomatic patients prior to surgery. We refer to Endocrine Society guidelines for management of such tumors.

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Jalil, Fatima, Aleksandra Sliwinska, Aashish G. Samat, and Joseph Rosenblatt. "Mystery Case of Parathyroid Carcinoma Masquerading as a Thyroid Nodule." Journal of the Endocrine Society 5, Supplement_1 (May 1, 2021): A206—A207. http://dx.doi.org/10.1210/jendso/bvab048.419.

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Abstract Introduction: Parathyroid carcinoma is a rare malignancy with a prevalence of 0.005%. Patients typically present with symptoms of hypercalcemia and elevated parathyroid hormone (PTH) levels. Non-functional parathyroid carcinoma presenting with neck pain and normal calcium is a unique and challenging entity. We describe a challenging case of normocalcemic parathyroid carcinoma presenting as neck pain. Case Description: A 59-year old woman with a history of asthma, type 2 diabetes, and tobacco use presented with neck pain and swelling worsening over months. On physical exam, she was noted to have right-sided thyroid mass. CT scan of the neck showed an enlarged thyroid gland with a 1.6 cm nodule with evidence of the trachea’s compression. Subsequent thyroid US revealed a 2.1 cm dominant solid, hypoechoic nodule in the right thyroid lobe. Initial testing revealed normal calcium levels; however, PTH level was not available. FNA of the nodule was non-diagnostic with subsequent repeat FNA interpreted as benign mass. Due to suspicious imaging features and significant neck discomfort, she underwent right hemithyroidectomy. During the surgery, the nodule was noted to be invasive with evidence of extrathyroidal extension. Postoperatively calcium was 8.9 mg/dl (8.6–10.4 mg/dl), ionized calcium 4.7 mg/dl (4.8–5.6mg/dl), PTH 48 pg/mL (14–64 pg/ml), and vitamin D25OH 48 ng/dL (30-100ng/dl). TSH was mildly elevated at 4.59 uIU/ml (0.27–4.2 uIU/ml) with free T4 of 1.09 ng/dl (0.93–1.70ng/dl). The preliminary pathology report described follicular thyroid carcinoma; however, immunostains failed to confirm primary thyroid cancer. The lesion was negative for TTF-1, PAX8, TG, CEA, calcitonin, chromogranin, and synaptophysin. The tumor was strongly positive for GATA3, PTH and positive in nuclear staining for parafibroma. The specimen was evaluated by two pathologists who favored the diagnosis of parathyroid carcinoma. A separate incidental microscopic 4mm papillary thyroid carcinoma (PTC) was found and tested positive for BRAF mutation. Genetic testing for CDC73 was negative. PET scan showed increased uptake in the right thyroid bed. Given microscopic PTC and mildly elevated TSH, she was started on levothyroxine. The patient recovered successfully after the surgery with the resolution of symptoms. She was evaluated by an oncologist, and adjuvant radiation therapy was recommended. Conclusion: Non-functional parathyroid carcinoma is a unique entity, and its diagnosis may be delayed given an atypical presentation and normal endocrine function. Differentiation between nonfunctioning parathyroid carcinoma, parathyroid adenoma, and thyroid disease based on location and proximity to the thyroid may be challenging. Although concomitant thyroid disease is not uncommon, synchronous thyroid cancer is very rare.

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Hashmi, Hiba, and Lisa Fish. "MON-922 Multiple Endocrine Neoplasia Type 1- A Clarion Call for Clarity." Journal of the Endocrine Society 4, Supplement_1 (April 2020). http://dx.doi.org/10.1210/jendso/bvaa046.1123.

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Abstract A 35-year-old gentleman presented with epigastric pain and bilious emesis. He also endorsed urinary frequency, non-bloody diarrhea and diffuse bone pain. On physical examination he had epigastric tenderness and multiple hyperpigmented skin lesions. An abdominal computed tomography (CT) scan revealed multiple diverticula with peri-colonic fat stranding in the descending and sigmoid colon, concerning for diverticulitis. He was started on a course of metronidazole and ciprofloxacin. A 3.1 cm mass was incidentally noted in the uncinate process of the pancreas. Bilateral adrenal nodules were also appreciated. An endoscopic ultrasound (EUS) guided trans-gastric fine needle aspiration biopsy was performed, revealing a well differentiated pancreatic neuroendocrine tumor (pNET - pT3N1Mx, intermediate risk). Chromogranin A was elevated to 108 ng/ml (reference range <93 ng/ml). Serum and urine metanephrine, V-peptide, gastrin, glucagon and parathyroid hormone related peptide were all normal; indicating a nonfunctioning neuroendocrine tumor. He underwent a pancreaticoduodenectomy. Octreotide scan was unrevealing for residual uptake. Adrenal biopsy revealed adrenal adenomas. Three years later, he presented with severe abdominal pain and a new pancreatic mass was noted on CT. Chromogranin A was elevated to 227 ng/mL. EUS revealed a 0.35 cm mass in the bed of the pancreatic head, encasing the superior mesenteric artery. Pathology was positive for recurrence of the neuroendocrine tumor. He was hypercalcemic to 11.4 mg/dL and parathyroid hormone was elevated to 319 pg/mL. CT neck revealed a 0.1 cm nodule concerning for parathyroid adenoma. He underwent a subtotal parathyroidectomy. Genetic testing confirmed Multiple Endocrine Neoplasia Type 1 (MEN1) with a heterozygous mutation of the menin1 gene. MEN1 is a rare genetic syndrome with affected individuals at increased risk of developing pancreatic, pituitary, parathyroid gland and cutaneous tumors. With a kaleidoscope of presentations, clinicians must maintain a high index of suspicion for MEN1, particularly for cases with nonfunctioning pNETs which present insidiously and are the foremost cause of mortality in MEN1 patients.1 Further clarity is needed on MEN1 associated pNET prognostic risk stratification, surveillance and targeted immunochemotherapy.2 Timely and algorithmic screening for MEN 1 syndrome in patients with pancreatic incidentalomas is essential to improving patient outcomes. 1. Kamilaris CDC, Stratakis CA. Multiple Endocrine Neoplasia Type 1 (MEN1): An Update and the Significance of Early Genetic and Clinical Diagnosis. Front Endocrinol. 2019;10:339. doi:10.3389/fendo.2019.00339 2. Yates CJ, Newey PJ, Thakker RV. Challenges and controversies in management of pancreatic neuroendocrine tumours in patients with MEN1. Lancet Diabetes Endocrinol. 2015;3(11):895-905. doi:10.1016/S2213-8587(15)00043-1

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Lesmana, Cosmas Rinaldi Adithya, Mutiara Lirendra, Sri Inggriani, Aru W. Sudoyo, and Laurentius Lesmana. "Treatment Outcome of Endoscopic Ultrasound Radiofrequency Ablation for Incidentaloma Pancreatic Neuroendocrine Tumor in Young Female Patient: A 2-year Follow-Up." Case Reports in Gastroenterology, November 7, 2022, 583–87. http://dx.doi.org/10.1159/000526197.

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Pancreatic neuroendocrine tumors (PNETs) or islet cell tumors are neuroendocrine neoplasms that arise from cells of the endocrine and nervous system within the pancreas. Patients with PNET sometimes do not show any symptoms, known as nonfunctioning (NF) sporadic PNET. It is still debatable regarding the best approach in the NF for small PNET. Currently, the surgical approach is considered the best; however, it is a highly invasive procedure, and it has a potentially high risk of complications as it requires a skilled and experienced operator. Herewith, we reported a 48-year-old female with incidentaloma of nonfunctioning PNET (NF-PNET) whose tumor has been successfully treated with endoscopic ultrasound guided radiofrequency ablation (EUSRA). There was no adverse event observed during and after the EUS procedure, and even 1 week after the procedure. One year later, abdominal magnetic resonance imaging (MRI) examination was carried out and size of the tumor was significantly getting smaller where it could hardly be seen anymore. After 2 years of follow-up, the latest abdominal MRI study showed no solid part of the tumor could be seen anymore. In conclusion, EUSRA can be an alternative option for incidentaloma of NF-PNET management.

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Bettale, Chiara M., Jason W. Allen, Zaid K. Mahdi, and Adriana G. Ioachimescu. "Pancreatic ACTH Hypersecretion and Pituitary Macroadenoma." JCEM Case Reports 1, no. 1 (November 29, 2022). http://dx.doi.org/10.1210/jcemcr/luad007.

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Abstract A 55-year-old woman admitted for hypertensive emergency and myocardial infarction reported weight gain, muscle weakness, easy bruising, and recent-onset diabetes in the past 3 to 12 months. Urinary and salivary cortisol and adrenocorticotropin hormone (ACTH) levels were elevated. Pituitary imaging detected a macroadenoma. ACTH and cortisol did not increase after corticotropin-releasing hormone administration. Imaging revealed a large pancreatic mass. Pathology indicated a well-differentiated World Health Organization (WHO) grade 2 distal pancreatic neuroendocrine neoplasm which stained for ACTH by immunohistochemistry. Postoperatively, Cushing manifestations resolved, ACTH and cortisol levels became low, and patient required hydrocortisone replacement for 7 months. During the 3.5 years of follow-up, the pituitary macroadenoma size remained stable and pituitary hormone axes other than ACTH remained normal. This extremely rare case of ectopic ACTH-secreting pancreatic neuroendocrine tumor coexisting with a nonfunctioning pituitary macroadenoma illustrates the importance of dynamic endocrine testing in Cushing syndrome.

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Shetty, Mahesh S., Sunil Kumar B. B., and Ankit Raj. "INTRAPANCREATIC ACCESSORY SPLEEN MIMICKING A NONSECRETING PANCREATIC NEURO ENDOCRINE TUMOR : A CASE REPORT." INTERNATIONAL JOURNAL OF SCIENTIFIC RESEARCH, September 1, 2020, 1–2. http://dx.doi.org/10.36106/ijsr/0408702.

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The accessory spleen is a congenital anomaly caused by a failure in embryologic development of spleen tissue with occurring in 10% of general population. In 16% of these cases, this tissue is found in tail of the pancreas. Here we present a case of 55 year old female who presented to our OPD with complaints of recurrent pain abdomen. CT showed a mass in the tail of pancreas with thrombosis of superior mesenteric artery. Patient underwent spleen sparing distal pancreatectomy with celiac axis to hepatic artery bypass and mass was sent for analysis. The pathological examination showed an intrapancreatic splenic tissue. As a rare ectopic location of spleen tissue and it should be considered in the differential diagnosis of pancreatic solid tumors. IPAS should be considered as a differential diagnosis in patients with suspected incidental pancreatic neuroendocrine tumour. Preoperative diagnosis is important as unnecessary surgery can be avoided.

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Hamada, Takashi, Shuji Isaji, Shugo Mizuno, Masami Tabata, Kentaro Yamagiwa, Hajime Yokoi, and Shinji Uemoto. "Laparoscopic Spleen-Preserving Pancreatic Tail Resection for an Intrapancreatic Accessory Spleen Mimicking a Nonfunctioning Endocrine Tumor: Report of a Case." Surgery Today 34, no. 10 (October 2004). http://dx.doi.org/10.1007/s00595-004-2839-9.

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Klein Haneveld, Mirthe J., Mark J. C. van Treijen, Carolina R. C. Pieterman, Olaf M. Dekkers, Annenienke van de Ven, Wouter W. de Herder, Wouter T. Zandee, et al. "Initiating Pancreatic Neuroendocrine Tumor (pNET) Screening in Young MEN1 Patients: Results From the DutchMEN Study Group." Journal of Clinical Endocrinology & Metabolism, August 1, 2021. http://dx.doi.org/10.1210/clinem/dgab569.

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Abstract Context Nonfunctioning pancreatic neuroendocrine tumors (NF-pNETs) are highly prevalent and constitute an important cause of mortality in patients with multiple endocrine neoplasia type 1 (MEN1). Still, the optimal age to initiate screening for pNETs is under debate. Objective The aim of this work is to assess the age of occurrence of clinically relevant NF-pNETs in young MEN1 patients. Methods Pancreatic imaging data of MEN1 patients were retrieved from the DutchMEN Study Group database. Interval-censored survival methods were used to describe age-related penetrance, compare survival curves, and develop a parametric model for estimating the risk of having clinically relevant NF-pNET at various ages. The primary objective was to assess age at occurrence of clinically relevant NF-pNET (size ≥ 20 mm or rapid growth); secondary objectives were the age at occurrence of NF-pNET of any size and pNET-associated metastasized disease. Results Five of 350 patients developed clinically relevant NF-pNETs before age 18 years, 2 of whom subsequently developed lymph node metastases. No differences in clinically relevant NF-pNET–free survival were found for sex, time frame, and type of MEN1 diagnosis or genotype. The estimated ages (median, 95% CI) at a 1%, 2.5%, and 5% risk of having developed a clinically relevant tumor are 9.5 (6.5-12.7), 13.5 (10.2-16.9), and 17.8 years (14.3-21.4), respectively. Conclusion Analyses from this population-based cohort indicate that start of surveillance for NF-pNETs with pancreatic imaging at age 13 to 14 years is justified. The psychological and medical burden of screening at a young age should be considered.

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