Artículos de revistas: "Pancreatic neuroendocrine tumor"

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D’Onofrio, M., G. Mansueto, M. Falconi y C. Procacci. "Neuroendocrine pancreatic tumor". Abdominal Imaging 29, n.º 2 (28 de enero de 2004): 246–58. http://dx.doi.org/10.1007/s00261-003-0097-8.

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Abbasi, Arezou, Kristina M. Wakeman y Venu G. Pillarisetty. "Pancreatic paraganglioma mimicking pancreatic neuroendocrine tumor". Rare Tumors 12 (enero de 2020): 203636132098279. http://dx.doi.org/10.1177/2036361320982799.

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Extra-adrenal paragangliomas are rare tumors arising from the chromaffin cells of the autonomic nervous system. Retroperitoneal paragangliomas may present as a pancreatic mass. We present a case of a 61-year-old woman with an incidentally found pancreatic mass (7.2 × 6.5 cm) in the CT scan. EUS- guided FNA result was compatible with pancreatic neuroendocrine tumor. Patient underwent pancreaticoduodenectomy and histopathologic assessment revealed the mass was an extra-adrenal paraganglioma. Preoperative diagnosis of pancreatic paragangliomas can be challenging due to imaging and histopathologic similarities with pancreatic neuroendocrine tumors.

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Manuel Vázquez, A., A. Valle Rubio, R. Latorre Fragua, L. Gijón de la Santa y JM Ramia. "Pancreatic neuroendocrine tumor and chronic pancreatitis: chance or causality?" Cirugía Andaluza 32, n.º 1 (5 de febrero de 2021): 60–62. http://dx.doi.org/10.37351/2021321.11.

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Resumen Los tumores neuroendocrinos pancreáticos representan menos del 3% de neoplasias pancreáticas. Su asociación con pancreatitis puede ser causal por obstrucción ductal tumoral, pero puede ser casual por coexistencia de ambas entidades. Presentamos un caso de asociación casual de pancreatitis crónica y tumor neuroendocrino pancreático y realizamos una revisión sistemática. Se obtuvieron 325 artículos y se seleccionaron 6, todos casos clínicos, donde esta relación no estaba justificada por obstrucción tumoral. En conclusión, la coexistencia pancreatitis crónica-tumor neuroendocrino pancreático en ausencia de causa obstructiva tumoral es un hallazgo excepcional cuya fisiopatología está por esclarecer. Marcadores inmunohistoquímicos y moleculares podrían ayudar a definirla.

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Chetty, Runjan y Ihab El-Shinnawy. "Intraductal Pancreatic Neuroendocrine Tumor". Endocrine Pathology 20, n.º 4 (18 de septiembre de 2009): 262–66. http://dx.doi.org/10.1007/s12022-009-9093-z.

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Diehl, David L., Joseph Blansfield y Jinhong Li. "Cystic pancreatic neuroendocrine tumor". Gastrointestinal Endoscopy 71, n.º 6 (mayo de 2010): 1064–65. http://dx.doi.org/10.1016/j.gie.2009.12.016.

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Yadav, Randhir Sagar, Ashik Pokharel, Shumneva Shrestha, Ashbita Pokharel, Deepshikha Gaire, Sumita Pradhan y Prasan Bir Singh Kansakar. "Pancreatic Neuroendocrine Tumor with Benign Serous Cystadenoma: A Rare Entity". Case Reports in Oncological Medicine 2021 (4 de agosto de 2021): 1–5. http://dx.doi.org/10.1155/2021/9979998.

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Mixed serous-neuroendocrine neoplasm constitutes pancreatic serous cystic neoplasms and pancreatic neuroendocrine tumor, two tumor components with different underlying pathologies. The differentiation of these tumors is important as the management and prognosis depend on the pancreatic neuroendocrine tumor component. We report a case of mixed serous-neuroendocrine neoplasm in a 47-year-old female who presented with epigastric pain abdomen for two years. Imaging studies, tumor markers, thorough systemic evaluation, surgical resection, histopathological examination, and timely follow-up constituted our management approach. A 4 cm × 4 cm mass in the distal pancreas with multiple cysts in the pancreatic parenchyma containing serous fluid on distal pancreatectomy and splenectomy was found. The histopathological examination revealed combined benign serous cystadenoma and neuroendocrine tumor. She did not have any recurrence or metastasis by four years of follow-up.

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Kim, Hanbaro, Ki Byung Song, Dae Wook Hwang, Jae Hoon Lee, Shadi Alshammary y Song Cheol Kim. "Time-trend and recurrence analysis of pancreatic neuroendocrine tumors". Endocrine Connections 8, n.º 7 (julio de 2019): 1052–60. http://dx.doi.org/10.1530/ec-19-0282.

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This study aimed to evaluate the evolving trends in clinicopathological features of pancreatic neuroendocrine tumors and to analyze the predictors of recurrence after curative resection. Data collected retrospectively from a single center between January 1990 and December 2017 were analyzed. Patients were categorized chronologically into three groups for evolving time-trend analysis. Overall, 542 patients (300 female, 55.4%) underwent surgical resection for pancreatic neuroendocrine tumors, including 435 (80.3%) with non-functional tumors. Time-trend analysis revealed that the surgically resected pancreatic neuroendocrine tumor number increased consistently; however, the incidental non-functional pancreatic neuroendocrine tumor number also increased over time (P < 0.001). The 5- and 10-year disease-free survival rates were 86.4 and 81.3%, respectively. The overall recurrence rate was 13.7%, and the most common site of recurrence was the liver. The median time to recurrence after primary surgery was 19.0 (range 0.8–236.3) months, and the median survival time after recurrence was 22.6 (range 0.4–126.9) months. On multivariate analysis, grade G3 pancreatic neuroendocrine tumors (hazard ratio 4.51; P < 0.001), lymph node metastasis (hazard ratio 2.46; P = 0.009), lymphovascular invasion (hazard ratio 3.62; P = 0.004), perineural invasion (hazard ratio 2.61; P = 0.004) and resection margin (hazard ratio 4.20; P = 0.003) were independent prognostic factors of disease-free survival. The surgically resected pancreatic neuroendocrine tumor number increased over time mainly because of an increase in incidentally discovered non-functional pancreatic neuroendocrine tumors. Grade G3 pancreatic neuroendocrine tumors, lymph node metastasis, lymphovascular invasion, perineural invasion and a positive resection margin were significant predictors of worse disease-free survival in patients with surgically resected pancreatic neuroendocrine tumors.

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Kulke, Matthew H., Heinz-Josef Lenz, Neal J. Meropol, James Posey, David P. Ryan, Joel Picus, Emily Bergsland et al. "Activity of Sunitinib in Patients With Advanced Neuroendocrine Tumors". Journal of Clinical Oncology 26, n.º 20 (10 de julio de 2008): 3403–10. http://dx.doi.org/10.1200/jco.2007.15.9020.

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Purpose Standard cytotoxic chemotherapy has limited efficacy in metastatic neuroendocrine tumor patients. Neuroendocrine tumors express vascular endothelial growth factor (VEGF) and its receptor (VEGFR). Sunitinib malate, an oral tyrosine kinase inhibitor, has activity against VEGFRs as well as platelet-derived growth factor receptors, stem-cell factor receptor, glial cell line–derived neurotrophic factor, and FMS-like tyrosine kinase-3. We evaluated the efficacy of sunitinib in a two-cohort, phase II study of advanced carcinoid and pancreatic neuroendocrine tumor patients. Patients and Methods Patients were treated with repeated 6-week cycles of oral sunitinib (50 mg/d for 4 weeks, followed by 2 weeks off treatment). Patients were observed for response, survival, and adverse events. Patient-reported outcomes were assessed. Results Among 109 enrolled patients, 107 received sunitinib (carcinoid, n = 41; pancreatic endocrine tumor, n = 66). Overall objective response rate (ORR) in pancreatic endocrine tumor patients was 16.7% (11 of 66 patients), and 68% (45 of 66 patients) had stable disease (SD). Among carcinoid patients, ORR was 2.4% (one of 41 patients), and 83% (34 of 41 patients) had SD. Median time to tumor progression was 7.7 months in pancreatic neuroendocrine tumor patients and 10.2 months in carcinoid patients. One-year survival rate was 81.1% in pancreatic neuroendocrine tumor patients and 83.4% in carcinoid patients. No significant differences from baseline in patient-reported quality of life or fatigue were observed during treatment. Conclusion Sunitinib has antitumor activity in pancreatic neuroendocrine tumors; its activity against carcinoid tumors could not be definitively determined in this nonrandomized study. Randomized trials of sunitinib in patients with neuroendocrine tumors are warranted.

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Ogbonna, Onyekachi Henry, Marie Carmel Garcon, Kostas N. Syrigos y Muhammad Wasif Saif. "Mixed Acinar-Neuroendocrine Carcinoma of the Pancreas with Neuroendocrine Predominance". Case Reports in Medicine 2013 (2013): 1–3. http://dx.doi.org/10.1155/2013/705092.

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Background. Pancreatic tumors are rare and could arise from either the exocrine (ductal and acinar cells) or the endocrine (neuroendocrine cells) components of the pancreas. In some instances, the occurrence of pancreatic tumors comprising both acinar cells and neuroendocrine cells, with neuroendocrine cells making up more than 30% of the tumor, has been identified. This unique entity has been referred to as mixed acinar-neuroendocrine carcinoma (MANEC). Only about 20 such cases have been reported in the literature.Case Report. We report an interesting case of MANEC with neuroendocrine cell predominance in a woman presenting with epigastric pain secondary to a pancreatic mass with acinar and endocrine differentiation. She underwent surgical resection of the tumor and was offered adjuvant treatment chemotherapy with carboplatin, etoposide, and radiotherapy for positive tumor resection margins.Conclusions. Given the paucity of the cases of MANEC, continuous reporting of these cases when identified should be encouraged to aid oncologists in understanding the disease and help establish standardized management.

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Cheema, A., L. K. Kvols y J. R. Strosberg. "Incidental diagnosis of pancreatic neuroendocrine tumors." Journal of Clinical Oncology 29, n.º 4_suppl (1 de febrero de 2011): 190. http://dx.doi.org/10.1200/jco.2011.29.4_suppl.190.

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190 Background: Pancreatic neuroendocrine tumors are often discovered incidentally during radiologic or endoscopic examinations. The incidence of incidental detection is unknown. It is also unclear whether patients with incidentally discovered, asymptomatic tumors should be treated similarly to patients who present with tumor-related symptoms. Methods: A database of 425 patients with pancreatic neuroendocrine tumors treated at the H. Lee Moffitt Cancer Center was developed. Patient charts were reviewed to assess whether their diagnosis was incidental or prompted by tumor-related symptoms such as pain, jaundice, or neuroendocrine hormone secretion. The frequency of “incidentalomas” was categorized by TNM stage (AJCC, 7th edition). Overall survival was stratified by “incidental” versus “symptomatic” diagnosis. Results: Among 425 patients with histologically proven pancreatic neuroendocrine tumors, 112 patients (26%) had tumors that were discovered incidentally. The majority of stage I tumors (55%) were incidentally discovered. Among patients with stage IV tumors, 20% were detected incidentally (Table). Median survival of patients with incidentally discovered tumors was 103 months versus 84 months in patients who were symptomatic at diagnosis. Conclusions: A sizeable fraction of patients with pancreatic neuroendocrine tumors are diagnosed incidentally during evaluations for other conditions or unrelated symptoms. The majority of patients with stage I tumors are incidentally diagnosed. The increased incidence of pancreatic neuroendocrine “incidentaloms” may be contributing to improving survival rates in this disease. This study highlights the necessity of developing guidelines for management of patients with incidentally discovered, early-stage tumors. [Table: see text] No significant financial relationships to disclose.

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Li, Jingyan, Xinxin Zhang, Qing He, Wenli Feng, Li Ding, Zhuoqun Wang, Haonan Yu et al. "Pancreatic neuroendocrine tumor producing vasopressin". Medicine 100, n.º 40 (8 de octubre de 2021): e27453. http://dx.doi.org/10.1097/md.0000000000027453.

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Piovesan, Deise M., Vinicius Duval da Silva, Carlos Luiz Reichel, Plínio Baú, João Rubião Hoefel Filho y Henrique Luiz Staub. "Neuroendocrine Pancreatic Tumor and Dermatomyositis". Pancreas 39, n.º 5 (julio de 2010): 684. http://dx.doi.org/10.1097/mpa.0b013e3181c7355e.

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Fang, Jiayun M. y Jiaqi Shi. "A Clinicopathologic and Molecular Update of Pancreatic Neuroendocrine Neoplasms With a Focus on the New World Health Organization Classification". Archives of Pathology & Laboratory Medicine 143, n.º 11 (11 de septiembre de 2019): 1317–26. http://dx.doi.org/10.5858/arpa.2019-0338-ra.

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Context.— According to the 2017 World Health Organization classification, pancreatic neuroendocrine neoplasms (PanNENs) include a new category of pancreatic neuroendocrine tumor, grade 3, which is often difficult to differentiate from pancreatic neuroendocrine carcinoma. However, pancreatic neuroendocrine tumor grade 3 and pancreatic neuroendocrine carcinoma are distinct entities with very different clinical presentation, prognosis, and therapeutic strategies. Recent discoveries on the molecular characteristics of pancreatic neuroendocrine tumors also play an essential role in the pathologic differential diagnosis of PanNENs. In addition, the histopathologic varieties of PanNENs bring in many differential diagnoses with other pancreatic neoplasms, especially acinar cell carcinoma, solid pseudopapillary neoplasm, and ductal adenocarcinoma. Objective.— To provide a brief update of the World Health Organization classification; the clinical, histopathologic, immunohistochemical, and molecular characteristics; and the differential diagnoses and biological behavior of PanNENs. Data Sources.— Analysis of the pertinent literature (PubMed) and authors' clinical practice experience based on institutional and consultation materials. Conclusions.— The evolving clinical, histopathologic, immunohistochemical, and molecular features of PanNENs are reviewed. Important differential diagnoses with other neoplasms of the pancreas are discussed.

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Balachandran, Aparna, Eric P. Tamm, Priya R. Bhosale, Matthew H. Katz, Jason B. Fleming, James C. Yao y Chuslip Charnsangavej. "Venous Tumor Thrombus in Nonfunctional Pancreatic Neuroendocrine Tumors". American Journal of Roentgenology 199, n.º 3 (septiembre de 2012): 602–8. http://dx.doi.org/10.2214/ajr.11.7058.

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Han, Xu y Wenhui Lou. "Concomitant pancreatic neuroendocrine tumors in hereditary tumor syndromes". Journal of Pancreatology 2, n.º 2 (junio de 2019): 48–53. http://dx.doi.org/10.1097/jp9.0000000000000016.

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Mitrovic-Jovanovic, Milica, Nikica Grubor, Stefan Milosevic, Aleksandra Jankovic, Katarina Stosic, Slavenko Ostojic, Aleksandar Ninic, Marjan Micev y Jelena Djokic Kovac. "Total Pancreatectomy for Multicentric Cystic Neuroendocrine Tumor of the Pancreas: A Case Report". Diagnostics 12, n.º 4 (15 de abril de 2022): 1003. http://dx.doi.org/10.3390/diagnostics12041003.

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Pancreatic neuroendocrine tumors (PNETs) are uncommon pancreatic neoplasms with malignant potential, heterogeneous clinical behavior, as well as imaging appearance. These tumors represent less than 3% of all pancreatic neoplasms with typical CT presentation as solid, well-circumscribed, hypervascular lesions. Cystic PNET is a rare pancreatic tumor which is nowadays more often detected due to the widespread use of high-resolution cross-sectional imaging. They are mainly solitary lesions most commonly localized in the body and the tail of the pancreas. Due to cystic presentation these lesions often present a diagnostic challenge to both experienced radiologists and pathologists. Herein, we present a rare case of synchronous, multiple cystic and solid pancreatic neuroendocrine tumors, which due to their extensiveness required total dudenopancreatectomy with splenectomy. Histopathological findings confirmed microscopic and macroscopic cystic components as well as typical solid variants of neuroendocrine tumors along the entire pancreas.

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Wang, Zhonglan, Xiao Chen, Jianhua Wang, Wenjing Cui, Shuai Ren y Zhongqiu Wang. "Differentiating hypovascular pancreatic neuroendocrine tumors from pancreatic ductal adenocarcinoma based on CT texture analysis". Acta Radiologica 61, n.º 5 (14 de septiembre de 2019): 595–604. http://dx.doi.org/10.1177/0284185119875023.

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Background Hypovascular pancreatic neuroendocrine tumor is usually misdiagnosed as pancreatic ductal adenocarcinoma. Purpose To investigate the value of texture analysis in differentiating hypovascular pancreatic neuroendocrine tumors from pancreatic ductal adenocarcinoma on contrast-enhanced computed tomography (CT) images. Material and Methods Twenty-one patients with hypovascular pancreatic neuroendocrine tumors and 63 patients with pancreatic ductal adenocarcinomas were included in this study. All patients underwent preoperative unenhanced and dynamic contrast-enhanced CT examinations. Two radiologists independently and manually contoured the region of interest of each lesion using texture analysis software on pancreatic parenchymal and portal phase CT images. Multivariate logistic regression analysis was performed to identify significant features to differentiate hypovascular pancreatic neuroendocrine tumors from pancreatic ductal adenocarcinomas. Receiver operating characteristic curve analysis was performed to ascertain diagnostic ability. Results The following CT texture features were obtained to differentiate hypovascular pancreatic neuroendocrine tumors from pancreatic ductal adenocarcinomas: RMS (root mean square) (odds ratio [OR] = 0.50, P<0.001), Quantile50 (OR = 1.83, P<0.001), and sumAverage (OR = 0.92, P=0.007) in parenchymal images and “contrast” in portal phase images (OR = 6.08, P<0.001). The areas under the curves were 0.76 for RMS (sensitivity = 0.75, specificity = 0.67), 0.73 for Quantile50 (sensitivity = 0.60, specificity = 0.77), 0.70 for sumAverage (sensitivity = 0.65, specificity = 0.82), 0.85 for the combined texture features (sensitivity = 0.77, specificity = 0.85). Conclusion CT texture analysis may be helpful to differentiate hypovascular pancreatic neuroendocrine tumors from pancreatic ductal adenocarcinomas. The three combined texture features showed acceptable diagnostic performance.

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Haq, Iqra, Somashekar G. Krishna, Bhaveshkumar Patel, Thavam Thambi-Pillai, Chencheng Xie, Karah Odegaard, Kimberlee Buohy y Muslim Atiq. "The Impact of Repeating Endosonography with Confocal Endomicroscopy for the Diagnosis of Cystic Neuroendocrine Tumor". Case Reports in Gastrointestinal Medicine 2019 (14 de enero de 2019): 1–4. http://dx.doi.org/10.1155/2019/5187874.

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Cystic pancreatic neuroendocrine tumors represent around 13% of all neuroendocrine tumors (Hurtado-Pardo 2017). There has been an increase in the incidence of cases due to improvement in imaging modalities. This is a case of a 68-year-old male with the incidental finding of a pancreatic cyst on CT. Initial Endoscopic Ultrasound with Fine Needle Aspiration (EUS-FNA) showed sonographic and cytology features suggestive of a pancreatic pseudocyst. However the cyst persisted with no change in size after aspiration leading to a follow-up EUS- FNA, which was combined with needle based confocal laser endomicroscopy (nCLE). The nCLE features were consistent with a cystic pancreatic neuroendocrine tumor, which was later confirmed on histology after surgical resection.

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Akirov, Amit, Vincent Larouche, Sameerah Alshehri, Sylvia L. Asa y Shereen Ezzat. "Treatment Options for Pancreatic Neuroendocrine Tumors". Cancers 11, n.º 6 (14 de junio de 2019): 828. http://dx.doi.org/10.3390/cancers11060828.

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The management of pancreatic neuroendocrine tumors (PanNETs) involves classification into non-functional or functional PanNET, and as localized or metastatic PanNET. In addition, while most PanNETs are sporadic, these endocrine neoplasms can also be manifestations of genetic syndromes. All these factors may assist in forming a risk stratification system permitting a tailored management approach. Most PanNETs are classified as non-functional because they are not associated with clinical sequelae of hormone excess. They are characterized by non-specific symptoms, such as abdominal pain or weight loss, resulting from mass effect related to the pancreatic tumor or secondary to distant metastases. Accurate staging of the disease is essential for determining the appropriate approach to therapy. As cure is only potentially possible with surgical resection of the tumor, it is recommended to remove all localized and limited metastatic disease. However, many patients present with metastatic and/or advanced local disease. In such instances, the goal of therapy is to control tumor growth and/or decrease tumor burden, lengthen survival, and palliate local symptoms and those of hormone excess. This typically requires a multimodal approach, including surgery, liver-directed treatment, and systemic medical therapy.

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Popov, Sergey, Aleksandr Pavlovskiy, Aleksey Polikarpov, Viktor Moiseenko, Andrey Moiseenko, A. Polekhin, A. Statsenko y A. Granov. "NEW POSSIBILITIES OF COMPLEX TREATMENT FOR METASTATIC AND LOCALLY ADVANCED NEUROENDOCRINE TUMORS OF THE PANCREAS". Problems in oncology 64, n.º 4 (1 de abril de 2018): 493–98. http://dx.doi.org/10.37469/0507-3758-2018-64-4-493-498.

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During the period 2006 to 2017 years 86 patients with neuroendocrine pancreatic tumors were observed and treated. 25 (29,1%) patients underwent only chemotherapy due to generalized tumor process or severe concomitant somatic status. 61 (70,9%) patients with neuroendocrine pancreatic tumors underwent surgical treatment. In 34 patients tumors were localized in the body and tail, in 27 - in the head of the pancreas. Women predominated among patients (n = 41), the average age of the patients was 51 ± 3.1 years. Synchronous metastatic liver metastases were detected in 33 (54,1%) of 61 patients, with the size of the primary tumor from 10 to 73 mm. In 47 (77%) neuroendocrine pancreatic tumors were regarded as non-functioning. Radical surgery was performed in 24 patients of 61 (39,3%) with tumor sizes from 11 to 128 mm (average 56 ± 21 mm), cytoreductive surgery was performed in 37 (60.7%) patients. Patients with locally advanced neuroendocrine pancreatic tumors (n = 13) and neuroendocrine pancreatic tumors with synchronous liver metastases (n = 33) undergone combined treatment (n = 46). Combined treatment was performed by means of intra-arterial selective oil chemoembolization and chemoinfusion, supplemented with one or several local methods effects: cytoreductive surgery and radiofrequency ablation (n = 8). 6 (12.2%) of patients with high, moderately and low-grade tumors died after the operation in period from 7 to 63 months from progression of disease. 43 (87.8%) patients survived in period from 4 to 112 months, 32 patients still alive without signs of disease progression. Because of recurrence in five patients (11.9%) repeated operations were performed. One patient (2.4%) underwent conformal radiation therapy. Life expectancy after surgery in patients with low-grade neuroendocrine cancer of the pancreas ranged from 3 to 16 months, and the median survival was 8.3 ± 1.2 months.

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Du, Yeting, Monica Ter-Minassian, Lauren Brais, Nichole Brooks, Amanda Waldron, Jennifer A. Chan, Xihong Lin, Peter Kraft, David C. Christiani y Matthew H. Kulke. "Genetic associations with neuroendocrine tumor risk: results from a genome-wide association study". Endocrine-Related Cancer 23, n.º 8 (agosto de 2016): 587–94. http://dx.doi.org/10.1530/erc-16-0171.

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The etiology of neuroendocrine tumors remains poorly defined. Although neuroendocrine tumors are in some cases associated with inherited genetic syndromes, such syndromes are rare. The majority of neuroendocrine tumors are thought to be sporadic. We performed a genome-wide association study (GWAS) to identify potential genetic risk factors for sporadic neuroendocrine tumors. Using germline DNA from blood specimens, we genotyped 909,622 SNPs using the Affymetrix 6.0 GeneChip, in a cohort comprising 832 neuroendocrine tumor cases from Dana-Farber Cancer Institute and Massachusetts General Hospital and 4542 controls from the Harvard School of Public Health. An additional 241 controls from Dana-Farber Cancer Institute were used for quality control. We assessed risk associations in the overall cohort, and in neuroendocrine tumor subgroups. We identified no potential risk associations in the cohort overall. In the small intestine neuroendocrine tumor subgroup, comprising 293 cases, we identified risk associations with three SNPs on chromosome 12, all in strong LD. The three SNPs are located upstream of ELK3, a transcription factor implicated in angiogenesis. We did not identify clear risk associations in the bronchial or pancreatic neuroendocrine subgroups. This large-scale study provides initial evidence that presumed sporadic small intestine neuroendocrine tumors may have a genetic etiology. Our results provide a basis for further exploring the role of genes implicated in this analysis, and for replication studies to confirm the observed associations. Additional studies to evaluate potential genetic risk factors for sporadic pancreatic and bronchial neuroendocrine tumors are warranted.

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Ungefroren, Hendrik, Björn Konukiewitz, Ulrich F. Wellner, Jörg Schrader y Tobias Keck. "The Use of PDX1 DNA Methylation to Distinguish Two Subtypes of Pancreatic Neuroendocrine Neoplasms with Different Prognoses". Cancers 15, n.º 1 (27 de diciembre de 2022): 160. http://dx.doi.org/10.3390/cancers15010160.

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Amanda Gomes, Rosa Andrea Nogueira Laiso, Luciana Knop, Monique Gonçalves Alves, Laertty Garcia de Sousa Cabral y Durvanei Augusto Maria. "Pancreatic Neuroendocrine Tumors: A Literature Review". JOURNAL OF BIOENGINEERING AND TECHNOLOGY APPLIED TO HEALTH 3, n.º 3 (2 de diciembre de 2020): 288–97. http://dx.doi.org/10.34178/jbth.v3i3.131.

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Pancreatic neuroendocrine tumors (PNETs) affect 1%-3% of patients with pancreatic cancer. This tumor is rare, difficult to diagnose, and clinically laborious. They have an estimated incidence of up to 1 case per 100,000 inhabitants every year. Up on diagnosis, most PNETs are considered malignant, with low healing potential, lesions that are generally unresectable, and a metastasis rate of aproximately 50%. PNETs are classified as functional and non-functional. The tumor functional produces hormones such as gastrin, insulin, somatostatin, glucagon, among others. They are symptomatic due to hormonal hypersecretion and occur in 30% of cases. The other 70% are non-functioning, and despite producing a series of substances and some hormones such as beta HCG and alpha HCG, they are silent tumors, with no significant clinical syndrome. The present study presented scientific evidence about PNETs, the types of pancreas endocrine-tissue tumors, the rate of survival, diagnosis, treatments, and prognosis, to provide solid support to professionals, and contribute to effective decision-making in search of the best clinical outcome.

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SALLER, JAMES, BROOKE HOUGH y DOMENICO COPPOLA. "Pancreatic-type Mixed Acinar-neuroendocrine Carcinoma of the Stomach: A Case Report and Literature Review". Cancer Diagnosis & Prognosis 1, n.º 4 (3 de septiembre de 2021): 285–88. http://dx.doi.org/10.21873/cdp.10037.

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Background: Pancreatic-type mixed acinar-neuroendocrine carcinoma (PMANEC) in the stomach is very rare. We report a case of PMANEC that was initially misdiagnosed as a gastric neuroendocrine tumor. Case Report: A 63-year-old female was found to have a gastric mass by histology and immunohistochemistry. The tumor had a heterogenous histology, with areas resembling pancreatic acinar cell carcinoma and other areas exhibiting neuroendocrine features. Only the neuroendocrine component was present in the initial biopsy, resulting in the erroneous diagnosis of gastric neuroendocrine tumor. Evaluation of the final resected tumor revealed cells expressing pancreatic exocrine markers, including trypsin and chymotrypsin and BCL10 immune signaling adaptor. Large areas of the tumor (>30%) were also positive for chromogranin A and synaptophysin. The final diagnosis was PMANEC. Conclusion: This type of gastric cancer is rare and may cause diagnostic difficulty, especially if only the neuroendocrine component of the tumor is sampled in a biopsy.

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Jeong, Mo Ah, Jun Kyu Lee, Ji Hyung Nam, Dong Kee Jang, Yun Jeong Lim, Jeong-Ju Lee y Eo-Jin Kim. "Pancreatic Hemangioma Suspected of Neuroendocrine Tumor". Korean Journal of Gastroenterology 76, n.º 1 (25 de julio de 2020): 46–48. http://dx.doi.org/10.4166/kjg.2020.76.1.46.

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A A, Ghasura, Patel K H, Prajapati B B, Hitesh Mahida y Manthan R. Merja. "A CASE REPORT: PANCREATIC NEUROENDOCRINE TUMOR". Journal of Evolution of Medical and Dental Sciences 3, n.º 22 (2 de junio de 2014): 6209–16. http://dx.doi.org/10.14260/jemds/2014/2724.

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Hagiya, Hideharu, Takahiro Matsui, Tetsuhiro Kitamura, Takanori Inoue, Minoru Shigekawa, Hisao Yoshida, Eiichi Morii y Kazunori Tomono. "Pancreatic Neuroendocrine Tumor Abnormally Secreting Procalcitonin". Pancreas 46, n.º 1 (enero de 2017): e7-e9. http://dx.doi.org/10.1097/mpa.0000000000000708.

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Melvin, W. S., B. J. Needleman, K. R. Krause y E. C. Ellison. "Robotic Resection of Pancreatic Neuroendocrine Tumor". Journal of Laparoendoscopic & Advanced Surgical Techniques 13, n.º 1 (febrero de 2003): 33–36. http://dx.doi.org/10.1089/109264203321235449.

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Nakazuru, Shoichi, Yuko Sakakibara, Hisashi Ishida, Kiyoshi Mori y Eiji Mita. "Gastric metastasis from pancreatic neuroendocrine tumor". Gastrointestinal Endoscopy 88, n.º 3 (septiembre de 2018): 559–60. http://dx.doi.org/10.1016/j.gie.2018.04.008.

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Ferrarese, Alessia, Alessandro Borello, Valentina Gentile, Marco Bindi, Yuri Ferrara, Mario Solej, Valter Martino y Mario Nano. "Meso-pancreatectomy for pancreatic neuroendocrine tumor". International Journal of Surgery 12 (agosto de 2014): S123—S125. http://dx.doi.org/10.1016/j.ijsu.2014.05.031.

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Sanli, Yasemin, Dilara Denizmen y Rathan M. Subramaniam. "Gastro-Enteric-Pancreatic Neuroendocrine Tumor Treatment". PET Clinics 18, n.º 2 (abril de 2023): 201–14. http://dx.doi.org/10.1016/j.cpet.2022.11.002.

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Satapathy, Swayamjeet, Kunal Ramesh Chandekar y Chandrasekhar Bal. "Gastro-Enteric-Pancreatic Neuroendocrine Tumor Treatment". PET Clinics 18, n.º 2 (abril de 2023): 215–21. http://dx.doi.org/10.1016/j.cpet.2022.11.004.

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Hong, J., K. Wang y Y. Yu. "Hepatobiliary and Pancreatic: Malignant pancreatic perivascular epithelioid cell tumor mimicking pancreatic neuroendocrine tumor". Journal of Gastroenterology and Hepatology 33, n.º 12 (10 de junio de 2018): 1940. http://dx.doi.org/10.1111/jgh.14272.

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Hicks, Bradley S., George Saffouri y Randall Pearson. "Hemosuccus Pancreaticus in a Patient With Pancreatic Neuroendocrine Tumor". American Journal of Gastroenterology 113, Supplement (octubre de 2018): S1137. http://dx.doi.org/10.14309/00000434-201810001-01993.

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Strosberg, J. R., A. Cheema, J. Weber y L. K. Kvols. "Prognostic relevance of a novel AJCC staging classification for neuroendocrine tumors of the pancreas." Journal of Clinical Oncology 29, n.º 4_suppl (1 de febrero de 2011): 177. http://dx.doi.org/10.1200/jco.2011.29.4_suppl.177.

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177 Background: The AJCC Cancer Staging Manual (7th edition, 2010) has introduced a novel TNM staging classification for pancreatic neuroendocrine tumors that is derived from the staging system for exocrine pancreatic adenocarcinomas. This classification has not yet been validated. Methods: Patients with pancreatic neuroendocrine tumors treated at the H. Lee Moffitt Cancer Center between 1999 and 2010 were assigned a stage (I-IV) based on the new AJCC classification. Overall survival from time of initial diagnosis was measured and statistical significance calculated using the log-rank test. The prognostic relevance of the AJCC staging classification was compared to the relevance of a staging classification proposed recently by the European Neuroendocrine Tumor Society (ENETS). Results: 425 patients with histologically proven pancreatic neuroendocrine tumors were identified. Both the novel AJCC classification and the ENETS classification were highly prognostic for survival (p<0.00001; Table). Conclusions: The novel AJCC 7th edition TNM classification for pancreatic neuroendocrine tumors is highly prognostic for overall survival and should be adopted in clinical practice. [Table: see text] No significant financial relationships to disclose.

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Hua, Hao, Xun Ran, Yi-gang Chen, Chao-quan Hu, Fu-tang Li, Shi Zuo, Cheng-yi Sun y Chao Yu. "Synchronous pancreatic neuroendocrine tumor and pancreatic cyst: a case report". World Journal of Clinical Surgery 1, n.º 2 (13 de abril de 2019): 1–4. http://dx.doi.org/10.52338/wjsurgy.2022.1010.

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Synchronous pancreatic neuroendocrine tumor (pNET) and pancreatic cyst are very rare. Herein, we report a case of pNET combined with a pancreatic cyst in a 49-year-old female. The patient first presented with a complaint of persistent pain in the right upper abdomen. As a follow-up, CT scan was performed that showed a solid pNET of 2-cm in diameter and a pancreatic cyst of 5-cm in the tail. Then, the subsequent distal pancreatomy with splenectomy was performed. The tumor histologically diagnosed with a cystic pancreatic neuroendocrine tumor. Therefore, it is critical to get a deep insight into this seldom tumor. Surgery remains the standard option of treatment for pNET.

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Sepp, Krisztián y Zsuzsanna Valkusz. "Various neuroendocrine tumors in a multiple endocrine neoplasia type 1 family". Orvosi Hetilap 154, n.º 51 (diciembre de 2013): 2037–42. http://dx.doi.org/10.1556/oh.2013.29772.

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When multiple endocrine tumors are detected more tests are required to diagnose endocrine tumor syndromes. The authors report the case history of a patient with clinical manifestation of multiplex endocrine neoplasia type 1 (parathyroid adenoma, pancreatic neuroendocrine tumor, pituitary tumor, adrenal gland tumors and thymic neuroendocrine carcinoma). Genetic screening proved a novel stop codon mutation of the MEN1 gene in the patient and in two other members of the family. The son of the index patient showed clinical symptoms of pancreatic neuroendocrine tumor (insulinoma) and parathyroid adenoma. One of the two daughters was also positive for the same mutation, however, she had no clinical symptoms. The authors review current knowledge on the genetic background of multiple endocrine syndrome type 1, the role of menin and the usefulness of gene mutation screening. Orv. Hetil., 2013, 154(51), 2037–2042.

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Koshel, A. P., V. V. Alipov, L. R. Bazilevich, A. I. Khvashchevsky, I. L. Purlik y E. S. Drozdov. "A RARE CLINICAL CASE OF MIXED SEROUS NEUROENDOCRINE CYSTIC NEOPLASM OF THE PANCREAS". Siberian journal of oncology 17, n.º 3 (4 de julio de 2018): 115–21. http://dx.doi.org/10.21294/1814-4861-2018-17-3-115-121.

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Background. Serous cystic pancreatic neoplasm is a rare pancreatic tumor, accounting for 1–2 % of all pancreatic tumors. In 2010, mixed serous neuroendocrine cystic neoplasia was included into the classification of the WHO for pancreatic tumors. Only a few cases of this disease have been reported in the literature. Mixed serous neuroendocrine cystic neoplasia is often associated with von Hippel Lindau disease. Most authors recommend surgery as a main treatment of this disease.Case report. We present a case with mixed serous neuroendocrine cystic neoplasia not associated with von Hippel Lindau disease. The patient underwent spleen – preserving distal pancreas resection. The diagnosis was histologically and immunohistochemically verified. There is no evidence of disease recurrence within 10 months after surgery. Conclusion. Histological examination of the resected serous cystadenoms should take into account the possibility of combining this pathology with pancreatic neuroendocrine tumors. For the purpose of verification, the use of immunohistochemical examination is required. Radical surgical resection allows patients with this disease to be cured.

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Tang, Laura H. "Pancreatic Neuroendocrine Neoplasms: Landscape and Horizon". Archives of Pathology & Laboratory Medicine 144, n.º 7 (16 de abril de 2020): 816–28. http://dx.doi.org/10.5858/arpa.2019-0654-ra.

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Context.— Since the initial description of pancreatic endocrine physiology and the recognition of islet cell tumors in the 1800s, there have been noteworthy advances in the pathobiology of pancreatic neuroendocrine neoplasms (PanNENs), and definition of the important distinction between well-differentiated neuroendocrine tumor (PanNET) and poorly differentiated neuroendocrine carcinoma (PanNEC). The evolving knowledge has resulted in a continuous update in terminology, classification, and grading system for this group of neoplasms. Pancreatic neuroendocrine tumors associated with hereditary conditions have been linked to unique molecular and genetic events, and sporadic PanNETs have specific gene signatures. Based on accumulative experience and knowledge, therapeutic strategies have been defined for this group of neoplasms. Objective.— To review the evolution and description of the pathologic-genomic evolution of PanNENs, and to facilitate accurate pathologic interpretation for the corresponding clinical management. Data Sources.— Literature review of published studies and author's own work. Conclusions.— Evolving experience and knowledge have established subtypes of pancreatic neuroendocrine neoplasms, based on their genotype and phenotype. Accurate pathologic interpretation of the specific neoplasm has significant implications for therapy and prognosis.

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Mathew, Annie, Dagmar Führer y Harald Lahner. "Sunitinib-Induced Hypothyroidism and Survival in Pancreatic Neuroendocrine Tumors". Hormone and Metabolic Research 53, n.º 12 (diciembre de 2021): 794–800. http://dx.doi.org/10.1055/a-1658-3077.

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AbstractSunitinib has been approved for the treatment of pancreatic neuroendocrine tumors, renal-cell carcinoma, and gastrointestinal stromal tumors. The elevation of thyroid-stimulating hormone serum levels is a common side effect. Studies suggest a correlation between sunitinib-induced hypothyroidism and treatment outcome in patients with renal-cell carcinoma and gastrointestinal stromal tumors. This study assessed whether sunitinib-induced hypothyroidism is a predictive marker of the objective response rate, progression-free survival, and overall survival in pancreatic neuroendocrine tumor patients. Twenty-nine patients treated with sunitinib for advanced pancreatic neuroendocrine tumors were included. The incidence of sunitinib-induced hypothyroidism was 33%. The median progression-free survival of patients who developed hypothyroidism was 16 months (95% confidence interval: 6.2–25.8 months) as compared with six months among euthyroid patients (95% confidence interval: 0.1–12.2 months) (p=0.02). The median overall survival was 77 months (95% confidence interval: 31.4–122.6 months) in hypothyroid patients but 12 months (95% confidence interval: 5.9–18.1 months) in subjects with euthyroidism (p=0.001). The median overall survival from the time of initial diagnosis ranged from 247 months in patients with hypothyroidism to 65 months in euthyroid subjects (p=0.015). Elevated thyroid-stimulating hormone levels are a prognostic biomarker of improved outcomes of sunitinib therapy in pancreatic neuroendocrine tumor patients.

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Johannesen, Eric y Van Nguyen. "WHO Grade 2 Neuroendocrine Tumor in a 15-Year-Old Male: A Case Report and Literature Review". Case Reports in Pathology 2014 (2014): 1–4. http://dx.doi.org/10.1155/2014/426161.

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Neuroendocrine tumors, distinguished from adenocarcinomas by their neuroendocrine differentiation, are the most common pediatric epithelial malignancy that most often occurs in the appendix. In 2010, the WHO classified neuroendocrine neoplasms into three grades based on morphology, mitotic count, and Ki67 proliferation index. A 15-year-old male with a history of anemia and failure to thrive was diagnosed with a well-differentiated neuroendocrine tumor in the jejunum that invaded into the subserosal soft tissue and metastasized to four lymph nodes. Pediatric neuroendocrine tumors frequently arise within hereditary tumor syndromes with pancreatic neuroendocrine tumors being the most common. Several studies also indicate an elevated risk of small intestinal neuroendocrine tumors in which children born to a parent with a history of neuroendocrine tumors in the small intestine have a significant increased risk of developing one.

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Chiu, R. y C. G. Yap. "Two Cases of Duodenal Neuroendocrine (Carcinoid) Tumor Concurrent with Invasive Gastric Adenocarcinoma and Pancreatic Intraductal Papillary Mucinous Neoplasm". American Journal of Clinical Pathology 158, Supplement_1 (1 de noviembre de 2022): S68. http://dx.doi.org/10.1093/ajcp/aqac126.137.

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Abstract Introduction/Objective Neuroendocrine (carcinoid) tumors of the duodenum are extremely rare and account for only 2-4% of gastrointestinal tract neuroendocrine tumors. We report two cases of incidentally discovered duodenal neuroendocrine tumor which respectively occurred in a patient with invasive gastric adenocarcinoma and in a patient with pancreatic intraductal papillary mucinous neoplasm (IPMN). Methods/Case Report The first case involved a 60-year old male who presented with melena and symptomatic anemia and was found on EGD to have a large circumferential mass at the gastric antrum/pylorus, which was revealed to be an invasive gastric adenocarcinoma on biopsy. At the time of distal gastrectomy for this malignancy, a concurrent, 0.6 cm-sized, low grade neuroendocrine tumor was incidentally discovered at the duodenal margin. The duodenal tumor consisted of nests and trabeculae of monotonous cells with ovoid/round nuclei, finely speckled “salt and pepper” chromatin, and low mitotic rate (< 2 mitoses/2 mm2), and invaded only into submucosa. The tumor cells were strongly and diffusely positive for neuroendocrine markers chromogranin, synaptophysin, and CD56, positive for CK7 and CDX2, negative for CK20, and with low Ki67 proliferation index (< 3%). The second case involved a 69-year old female who presented with epigastric pain and was found on abdominal CT to have a 4 cm-sized cystic mass in the pancreatic head. While biopsy of the pancreatic mass revealed an IPMN, a separate biopsy of an incidentally discovered, 1 cm-deep ulcer in the duodenum bulb revealed a low-grade neuroendocrine tumor with similar morphologic features, mitotic rate, and immunohistochemical staining pattern as the prior case while also negative for IMP3 stain. The patient had normal serum gastrin and urine HIAA levels, but elevated serum chromogranin-A levels. Results (if a Case Study enter NA) NA. Conclusion Duodenal carcinoids are rare (1-3% of primary duodenal tumors) and are mostly non-functional and unifocal. The duodenum is also the least common site (2-4%) for GI tract neuroendocrine tumors. The pathophysiology of these tumors is still poorly understood, with the majority occurring sporadically and a minority in the setting of hereditary cancer syndromes. This report documents two very rare instances of duodenal neuroendocrine tumor incidentally discovered with invasive gastric adenocarcinoma and pancreatic IPMN.

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Ahmed, Haris, Alan Vu, Junaid Alam, Bonny Ogar y Barbara Lines. "Well-Differentiated Malignant Neuroendocrine Cell Tumor Originating From the Jejunum With Excessive Insulin Secretion". Journal of the Endocrine Society 5, Supplement_1 (1 de mayo de 2021): A1008—A1009. http://dx.doi.org/10.1210/jendso/bvab048.2063.

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Abstract Background: Insulinomas are rare, well-differentiated insulin-secreting neuroendocrine tumors of the pancreas. Insulin-secreting, extra-pancreatic tumors have rarely been reported in the literature. We present a case of a well-differentiated neuroendocrine tumor with clinical, laboratory, and immunohistochemical tumor findings supportive of ectopic insulin production in a non-pancreatic neuroendocrine tumor of the small bowel. Clinical Case: A 70 year-old female with past medical history of end stage renal disease, Type II diabetes mellitus, and hypertension presented to our facility with multiple episodes of dizziness and syncope due to hypoglycemia. She had initially managed her diabetes with metformin but stopped taking the medication due to increasingly frequent episodes of hypoglycemia. Significant labs during the hospitalization include blood glucose <20 mg/dL, insulin 75.9 IU/mL (2.6-21.9 IU/mL) and C-peptide 57.7 ng/mL (1.1-4.4 ng/mL). Her symptoms persisted despite dextrose supplementation. Abdominal and Pelvic CT showed proximal small bowel obstruction and sclerosing mesenteritis with a calcified lymph node but no pancreatic mass. A diagnostic laparoscopy was performed, showing 18 inches of small bowel with malignant appearing masses that were adherent to the mesentery. A 21.0 cm segment of jejunum was resected. There were at least 30, variably sized masses throughout the bowel, involving the mucosa, submucosa, muscularis propria and subserosal fat; along with lymphatic, venous, and perineural space tumor permeation, and metastases involving multiple regional lymph nodes. The tumors ranged in size from 0.5 up to 1.5 cm. They showed well-differentiated nuclear morphology, a mitotic count of 0 per 80 high power fields, and a Ki-67 proliferation index of 1%, indicative of a G1 NET. Immunohistochemical stains confirmed neuroendocrine differentiation with positive staining for chromogranin, synaptophysin, and INSM1 (insulinoma-associated protein 1) transcription factor, while insulin staining was negative. The patient was diagnosed with a pT3N1, G1, ectopic insulin-secreting neuroendocrine tumor of small bowel origin. She was started on diazoxide with significant improvement in her hypoglycemia. Conclusion: Insulin production by non-pancreatic tumors is extremely rare. Clinical, laboratory, and immunohistochemical tumor findings in this case support ectopic insulin production by a well-differentiated neuroendocrine tumor of small bowel origin.

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Gianfredi, Vincenza, Daniele Nucci, Mariateresa Nardi, Omar Enzo Santangelo y Sandro Provenzano. "Using Google Trends and Wikipedia to Investigate the Global Public’s Interest in the Pancreatic Cancer Diagnosis of a Celebrity". International Journal of Environmental Research and Public Health 20, n.º 3 (24 de enero de 2023): 2106. http://dx.doi.org/10.3390/ijerph20032106.

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A cross-sectional study was designed to assess the impact of a celebrity’s announcement of having been diagnosed with pancreatic cancer on the volume of cancer-related research on the Internet. Global searches were carried out on Google Trends (GT) for the period from 1 January 2004 to 20 November 2022 (since data prior to 2004 were not available) using the search words Tumore del Pancreas (pancreatic cancer), Tumore neuroendocrino (neuroendocrine tumor), and Fedez (the name of a popular Italian rapper). The frequency of specific page views for Fedez, Tumore del pancreas, and Tumore neuroendocrino was collected via Wikipedia Trends data. Statistical analyses were carried out using the Pearson correlation coefficient (r). The GT data revealed a strong correlation (r = 0.83) while the Wikipedia Trends data indicated a moderate correlation (r = 0.37) for Tumore neuroendocrino and Tumore del pancreas. The search peaks for the GT and Wikipedia pages occur during the same time period. An association was found between the celebrity’s announcement of his pancreatic cancer diagnosis and the volume of pancreatic-cancer-related online searches. Our findings demonstrate that media events and media coverage of health-related news can raise people’s curiosity and desire for health information.

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Gayathri, K., B. Archana, S. Rajendiran y T. K. Anand. "Pancreatic neuroendocrine tumor trapped in a cyst". Sri Ramachandra Journal of Health Sciences 1 (24 de diciembre de 2021): 28–30. http://dx.doi.org/10.25259/srjhs_8_2021.

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Pancreatic neuroendocrine tumor (Pan NET) accounts for only 1–2% of pancreatic neoplasms and <1% of tumors present as cystic lesions. A diagnosis of cystic tumor in the pancreas can be very challenging and confusing. Endoscopic ultrasound (USG) and fine needle aspiration (FNA) of these cystic lesions can help the clinician to narrow down the differential diagnosis. A multidisciplinary approach is warranted for a definitive diagnosis and optimal treatment. Here, we present to you a very rare case of Pan NET presenting as a cystic mass. A 65-year-old man was admitted with a history of abdominal pain. Laboratory tests showed increased levels of serum amylase, and other biochemical tests were normal. The patient underwent transabdominal USG and computed tomography (CT). CT showed well-defined cystic lesion in the proximal body of the pancreas. Endoscopic guided FNA (EUS-FNA) using 22 gauges was done. Cytological examination demonstrated clusters and sheets of plasmacytoid cells. EUS core needle biopsy was done which showed loose clusters of cells with fine uniformly distributed chromatin that stained positively for insulinoma associated protein-1 which was suggestive of a Pan NET. The differential diagnosis of cystic lesions in the pancreas is very difficult with conventional radiology such as CT and magnetic resonance imaging. Cytology is helpful for a pre-operative diagnosis of cystic Pan NET. A diagnosis of Pan NET in a pancreatic biopsy or FNA with limited and suboptimal material is often challenging. This unusual case highlights the importance of pre-operative workup of EUS followed by FNAC in cystic pancreatic lesions.

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Sung, Min Je y Moon Jae Chung. "Current Paradigm of Treatment for Pancreatic Neuroendocrine Tumor". Korean Journal of Pancreas and Biliary Tract 26, n.º 1 (31 de enero de 2021): 24–32. http://dx.doi.org/10.15279/kpba.2021.26.1.24.

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Pancreatic neuroendocrine tumor (PNET) refer to tumors originating from the islet of Langerhans and shows various prognosis based on the presence or absence of symptoms due to hormone secretion, the Ki-67 cell proliferation index, and the histologic grade, and according to the degree of disease progression defined by the tumor-node-metastasis (TNM) stage classification. The purpose of medical treatment for PNET is to control symptoms or inhibit tumor growth. Somatostatin analogues can be administered for the purpose of controlling symptoms caused by the secretion of specific hormones, and are accepted as effective drugs for inhibiting the progression of G1/G2 tumors based on World Health Organization (WHO) classification with a Ki-67 cell proliferation index less than 20%. Among the molecularly targeted agents, everolimus and sunitinib can be considered in patients with WHO G1/G2 PNET showing progression after somatostatin analog therapy. Cytotoxic chemotherapy is generally administered to patients with large tumor volume and rapidly progressing metastatic NET, and etoposide/cisplatin combination therapy has been considered as a standard treatment. For the patient group of Grade 3 PNET (well differentiated) newly classified by the WHO 2017 classification, guidelines for standard treatment have not yet been established. As it has been reported, studies are needed to evaluate the treatment response rate of somatostatin analogues or molecularly targeted therapies for the patient with Grade 3 PNET. It is important to consider a multidisciplinary approach with all possible treatment options including medical treatment, radical resection of primary or metastatic lesions, liver-directed therapies, and peptide receptor radionuclide therapy for the patients with PNET.

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Viehweger, Florian, Lisa-Marie Tinger, David Dum, Natalia Gorbokon, Anne Menz, Ria Uhlig, Franziska Büscheck et al. "Diagnostic and Prognostic Impact of Progesterone Receptor Immunohistochemistry: A Study Evaluating More Than 16,000 Tumors". Analytical Cellular Pathology 2022 (8 de agosto de 2022): 1–15. http://dx.doi.org/10.1155/2022/6412148.

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Progesterone receptor (PR) is a member of the nuclear/steroid hormone receptor family of ligand-dependent transcription factors. It plays an important role in reproduction and mammary gland development and has various tissue-specific effects in nonreproductive organs. In diagnostic pathology, positive PR immunostaining is used to support a diagnosis of breast or gynecologic origin in a tumor. In this study, the expression of PR was analyzed by immunohistochemistry in 18,176 (interpretable: 16,445) samples from 147 different tumor types and subtypes in a tissue microarray format. PR immunostaining was detected in 57.4% of breast tumors, 28.6% of other gynecological tumors, and 1.8% of nongynecological and nonmammary tumors. Among the group of nongynecological and nonmammary tumors, particularly high rates of PR positivity were seen in neuroendocrine tumors (54.3%) and neuroendocrine carcinomas (35.7%) of the pancreas. A comparison with clinico-pathological parameters showed that reduced PR immunostaining was significantly associated with adverse histopathological and clinical features in breast carcinoma, endometrioid endometrial carcinoma, and pancreatic neuroendocrine tumors. In summary, our analysis of 147 different tumor types for PR immunostaining provides a ranking list of tumor entities according to their prevalence of PR positivity, helps to better understand the diagnostic utility of PR, and highlights the distinct PR positivity among neuroendocrine neoplasms of pancreatic origin.

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Tzontcheva, Anna. "Neuroendocrine Tumors — Laboratory Diagnosis". Journal of Medical Biochemistry 29, n.º 4 (1 de octubre de 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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Bassaneze, Thiago, Clovis Augusto Borges do Nascimento, Adam Hiar, Laura Silveira Tanisaka, Beatriz Villas-Boas Weffort, Larissa Mariana Ayde, Maria Laura Kachan Bordignon y 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, n.º 1 (15 de marzo de 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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Kit, Oleg I., Vladimir S. Trifanov, Natalya N. Timoshkina, Dmitry Yu Gvaldin, Milana Yu Mesheryakova, Evgeniy N. Kolesnikov, Mikhail A. Kozhushko, Mikhail A. Averkin, Stanislav S. Mezentsev y Petr N. Gabrichidze. "Methylation status of eight tumor suppressors in neuroendocrine pancreatic tumors." Journal of Clinical Oncology 39, n.º 15_suppl (20 de mayo de 2021): e16197-e16197. http://dx.doi.org/10.1200/jco.2021.39.15_suppl.e16197.

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e16197 Background: Aberrant DNA methylation is a characteristic feature of cancer, affecting gene expression and tumor phenotype. In this study, we quantified the methylation of promoters of eight tumor suppressor genes in pancreatic neuroendocrine tumors (Pan-NET). Methods: The method of pyrosequencing was used to quantity level (Met,%) of methylation of gene promoters - tumor suppressors AHRR, APC1A, DAPK, MGMT, MLH1, P16, RASSF1A, RUNX3 in tumor samples from 55 patients with pancreatic NET (G1-G3) and in the blood of 10 healthy donors. Met for each sample was calculated as the median methylation of CpG sites in triplicate. Results: Hypermethylation was observed for AHRR (75%), APC1A (25%), RASSF1A (30%). In contrast, DAPK, MGMT, MLH1, P16, RUNX3 had low methylation levels ( < 20%). The median of methylation in the blood of healthy donors for AHRR was 91% (76-98); for all other loci it did not exceed 6%. A high incidence of methylation in excess of blood levels in healthy donors was identified for RASSF1A (0.96); AHRR (0.75); MGMT (0.65); RUNX3 (0.41), APC1A (0.25). For tumor suppressor P16, only one case of increased methylation was recorded (Met = 15%), despite the fact that this phenomenon is not uncommon for NETs of other localizations. In 66% of pancreatic NET cases, hypermethylation of more than two promoters of tumor suppressor genes was noted. An association tendency was found between the presence of MEN1 mutations and the RASSF1A methylation level (p = 0.08). Correlation analysis revealed a significant level of negative association between changes in methylation of MLH1 and AHRR (p < 0.01); for the latter, the prognostic value of a high methylation status and a better prognosis for many malignant neoplasms were described. Conclusions: In the present study, significant methylation of the promoters of the APC1A, DAPK, MGMT, RASSF1A, and RUNX3 genes in well-differentiated pancreatic NETs was identified with a high frequency. At the same time, isolated cases of hypermethylation were noted for the well-known tumor suppressors MLH1 and P16.

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