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Case 14
Published in Andrew Solomon, Julia Anstey, Liora Wittner, Priti Dutta, Clinical Cases, 2021
Andrew Solomon, Julia Anstey, Liora Wittner, Priti Dutta
SomatostatinomaDiabetes mellitusAnaemiaCholelithiasisDiarrhoea, steatorrhoea and weight loss
Mahvash Disease
Published in Dongyou Liu, Handbook of Tumor Syndromes, 2020
While pancreatic neuroendocrine tumor (PNET) arising from glucagon-producing α cells is called glucagonoma, that arising from insulin-producing β cells is called insulinoma, that arising from somatostatin-producing δ cells is called somatostatinoma, that arising from gastrin-producing D cells is called gastrinoma, that arising from pancreatic polypeptide-producing F (PP/gamma) cells is associated with multiple hormonal syndromes, and that arising from serotonin-producing cells is called carcinoid tumor. Further, nonfunctioning PNET produces no or insufficient hormones but is associated with nonspecific clinical symptoms (e.g., vague abdominal pain).
Pancreatic disorders in children
Published in Brice Antao, S Irish Michael, Anthony Lander, S Rothenberg MD Steven, Succeeding in Paediatric Surgery Examinations, 2017
Christian J Streck Jr, Andre Hebra
Somatostatin receptor scintigraphy is both sensitive and specific for local-ising gastrinomas. Somatostatinoma, VIPoma and glucagonoma are all very rare in children. Somatostatinoma is a tumour of the pancreatic delta cells of the pancreas that produce somatostatin, which suppresses the release of GI hormones. Symptoms include diabetes mellitus (suppressed insulin release), steatorrhoea (suppressed CCK and secretin release), cholelithiasis (suppressed CCK release), and hypochlorhydria (suppressed gastrin release). VIPoma (also known as Verner–Morrison’s syndrome) causes chronic watery diarrhoea with resultant hypokalaemia and achlorhydria (WDHA syndrome). Necrolytic migratory erythema, commonly seen along the lower abdomen and perineum, frequently results from a glucagonoma, potentially from abnormal amino acid and zinc levels, and typically resolves following tumour resection.
Somatostatin-derived amyloidosis: a novel type of amyloidosis associated with well-differentiated somatostatin-producing neuroendocrine tumours
Published in Amyloid, 2022
Benjamin J. Van Treeck, Surendra Dasari, Paul J. Kurtin, Jason D. Theis, Samih H. Nasr, Lizhi Zhang, Saba Yasir, Rondell P. Graham, Ellen D. McPhail, Samar Said
LC MS/MS confirmed that the amyloid deposits in all four cases contained abundant spectra for somatostatin without significant levels of other amyloid precursor proteins, consistent with somatostatin-related amyloid deposition. Out of 19,298 amyloid specimens from numerous anatomic sites typed by LC-MS/MS, we found only four cases of somatostatin-derived amyloidosis, all of which were associated with duodenal or pancreatic somatostatinomas. Conversely, all 408 small bowel and pancreas amyloidosis cases of other amyloid types were devoid of somatostatin by proteomic analysis. We also did not find somatostatin-related amyloid in any other anatomic site, suggesting that this is a localised form of amyloidosis that arises secondary to production of an amyloidogenic form of somatostatin by the somatostatinoma tumour cells. Therefore, we speculate that treating the neuroendocrine neoplasm is probably an adequate management for the amyloid.
Surgical management of pancreatic neuroendocrine tumors: an introduction
Published in Expert Review of Anticancer Therapy, 2019
Elisabeth Hain, Rémy Sindayigaya, Jade Fawaz, Joseph Gharios, Gaspard Bouteloup, Philippe Soyer, Jérôme Bertherat, Frédéric Prat, Benoit Terris, Romain Coriat, Sébastien Gaujoux
For other functional pNETs such as VIPoma or somatostatinoma, whenever possible, R0 resection is indicated. Nevertheless, these lesions often present with synchronous metastasis and surgical management depends on the resectability of the metastasis. For those highly aggressive functional tumors, standard pancreatic surgery with formal lymphadenectomy remains the treatment of choice. For MEN 1 other functional tumor, surgery is also indicated whenever possible.
Prognostic value and impact of cerebral metastases in pancreatic cancer
Published in Acta Chirurgica Belgica, 2020
Andreas Minh Luu, Beat Künzli, Philipp Hoehn, Johanna Munding, Carsten Lukas, Waldemar Uhl, Chris Braumann
Brain metastases are not restricted to pancreatic ductal adenocarcinoma (PDAC) since reports about CNS involvement in malignant somatostatinoma or IPMN carcinoma (intraductal papillary mucinous neoplasia) have been published [13,14]. As shown in our data, only two patients were diagnosed with PDAC. The other two patients were diagnosed with mucinous adenocarcinoma and cystadenocarcinoma of the pancreas.