Pancreatic Endocrine Tumors

doi:10.1016/S0344-0338(88)80043-8

Pathology - Research and Practice

Volume 183, Issue 2, April 1988, Pages 155-168

https://doi.org/10.1016/S0344-0338(88)80043-8 Get rights and content

Summary

In this review the current state of our understanding of endocrine tumors of the pancreas is considered. It is based on the experience with a series of 365 tumors. The first part of the article focuses on origin and classification, markers, frequency, criteria of malignancy as well as general structural features of the pancreatic endocrine tumors. In the second half of the article the functioning tumors, i.e. tumors that cause hormonal syndromes, and the nonfunctioning tumors as well as the endocrine tumors associated with multiple endocrine neoplasia type 1 are dealt with in detail. Special emphasis is put on the immunocytochemical profile and the biological features of the respective tumors.

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Citation Excerpt :
PanNETs have since been recognized as an endocrine malignancy and are the 2nd most common neoplasm of the pancreas. Historically, the incidence of PanNETs has been low and patients often present with clinical signs and symptoms of peptide hormone hypersecretion (functional PanNETs), such as insulin, glucagon, and gastrin [2]. Furthermore, these historical reports have shown most patients present with locally advanced or distant metastatic disease at the time of diagnosis.
Pancreatic neuroendocrine tumors (PanNETs) are a heterogeneous group of neoplasms with increasing incidence and an ill-defined pathobiology. Although many PanNETs are indolent and remain stable for years, a subset may behave aggressively and metastasize widely. Thus, the increasing and frequent detection of PanNETs presents a treatment dilemma. Current prognostic systems are susceptible to interpretation errors, sampling issues, and do not accurately reflect the clinical behavior of these neoplasms. Hence, additional biomarkers are needed to improve the prognostic stratification of patients diagnosed with a PanNET. Recent studies have identified alterations in death domain-associated protein 6 (DAXX) and alpha-thalassemia/mental retardation X-linked (ATRX), as well as alternative lengthening of telomeres (ALT), as promising prognostic biomarkers. This review summarizes the identification, clinical utility, and specific nuances in testing for DAXX/ATRX by immunohistochemistry and ALT by telomere-specific fluorescence in situ hybridization in PanNETs. Furthermore, a discussion on diagnostic indications for DAXX, ATRX, and ALT status is provided to include the distinction between PanNETs and pancreatic neuroendocrine carcinomas (PanNECs), and determining pancreatic origin for metastatic neuroendocrine tumors in the setting of an unknown primary.
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^a: Prof. Dr. Günter Klöppel, Department of Pathology, Free University of Brussels, Laarbeeklaan 101, B 1090 Brussels

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Pancreatic Endocrine Tumors

Summary

Hum Pathol

Lancet

Am J Med

Hum Pathol

Metabolism

Gastroenterol

Hum Pathol

Am J Surg

Lancet

Lancet

Gastroenterology

Gastroenterology

Hum Pathol

Lancet

Malignant argentaffinoma with carcinoid syndrome and hypoglycemia

Br Med J

Immunohistochemical evidence of peptide hormones in endocrine tumors of the rectum

Cancer

Neurohormonal peptides in endocrine tumors of the pancreas, stomach and upper small intestine: An immunohistochemical study of 27 cases

Ultrstruct Pathol

Pancreatic tumor with carcinoid syndrome and hypoglycemia

Postgrad Med J

Pancreatic endocrine carcinoma with ectopic PTH-production and paraneoplastic hypercalcaemia

Virchows Arch (Pathol Anat)

Pancreatic islet cell carcinoma producing gastrin, ACTH, alpha-endorphin, somatostatin and calcitonin

Am J Gastroenterol

Nesidioblastosis and islet cell changes related to endogenous hypergastrinemia

Virchows Arch (Cell Pathol)

A multihormonal tumor of the pancreas producing neurotensin associated with the WDHA syndrome. Histology, histochemistry and origin

Histol Histopath

Bone metastases in malignant gastrinoma

Gastroenterol

Functional and morphologic characterization of human insulinomas

Diabetes

Pancreatic PP-cell distribution and hyperplasia

Virchows Arch A Path Anat and Histol

A study of glucagonomas by light and electron microscopy and immunofluorescence

Diabetes

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Am J Pathol

Gastrin releasing peptide in human neuroendocrine tumors

Journal of Pathology

Pancreatic islet cell carcinoma. I. Clinical features of 52 patients

Ann Intern Med

Pancreatic islet cell tumors

Surgery

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Virchows Arch A Path Anat and Histol

Morphologic patterns and diagnostic criteria of VIP-producing endocrine tumors. A histologic, histochemical, ultrastructural and biochemical study of 32 cases

Cancer

Pancreatic islet cell tumor associated with Cushing's syndrome

Am J Surg Pathol

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Diabetologia

Endocrine tumors of the pancreas

Pancreatic islet cell carcinoma with hypercalcemia and hypergastrinemia

Cancer

Islet cell carcinoma of the pancreas

Arch Pathol

Psammomatous somatostatinomas of the duodenum

Am J Surg Pathol

Immunocytochemical demonstration of growth hormone-releasing factor in gastrointestinal and pancreatic endocrine tumors

Am J Clin Pathol

Resection of gastrinomas

Ann Surg

Nonfunctioning malignant neuroendocrine tumors of the pancreas

Surgery

Endocrine-paracrine cells in pancreatic exocrine car-cinomas

Histopathology

A multihormonal tumor of the pancreas producing neurotensin

Dig Dis Sci

The development of endocrinopathies in the prospective screening of two families with multiple endocrine adenopathy, type I

World J Surg

Tumors of the endocrine pancreas

New Engl J Med

Pseudo-Zollinger-Ellison syndrome. Hypergastrinemia, hyperchlorhydria without tumor

Ann Sure

Pancreatic polypeptide update: Its roles in detection of the trait for multiple endocrine adenopathy syndrome, type I and pancreatic polypeptide-secreting tumors