Multihormonality and entrapment of islets in pancreatic endocrine tumors

The gastroenteropancreatic neuroendocrine tumors (GEP-NETs) are composed of cells with a neuroendocrine phenotype. Well-differentiated tumors, well-differentiated carcinomas, poorly differentiated carcinomas, functioning tumors (with a hormonal syndrome), and nonfunctioning tumors are identified. To predict their clinical behavior, these neuroendocrine tumors are classified on the basis of their clinicopathological features, including size, local invasion, angioinvasion, proliferative activity, histological differentiation, and metastases, into neoplasms with benign, uncertain, low-grade malignant and high-grade malignant behavior. In addition, a tumor/nodes/metastases classification and a grading system are presented. In the light of these criteria, the various GEP-NET entities are reviewed.

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“…Immunohistochemically, they stain for insulin and proinsulin. In addition there may be cells [72,79].…”

Section: Pancreasmentioning

confidence: 99%

Site-specific biology and pathology of gastroenteropancreatic neuroendocrine tumors

et al. 2007

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“…In addition, five patients with MEN1 were included. Some of the patients were included in earlier studies investigating the histopathology and genetics of NETs (Pipeleers et al 1983, Ohike et al 2004, Sipos et al 2004, Anlauf et al 2005, Kapran et al 2006.…”

Section: Patients and Tissuesmentioning

confidence: 99%

Somatostatin-producing neuroendocrine tumors of the duodenum and pancreas: incidence, types, biological behavior, association with inherited syndromes, and functional activity

Garbrecht¹,

Anlauf²,

Schmitt³

et al. 2008

Endocrine Related Cancer

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166

128

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Somatostatin-producing neuroendocrine tumors (SOM-NETs) of the duodenum and pancreas appear to be heterogeneous. To determine their clinicopathological profiles, respective data were analyzed on a series of 82 duodenal and 541 pancreatic NETs. In addition, the clinical records of 821 patients with duodenal or pancreatic NETs were reviewed for evidence of a somatostatinoma syndrome. Predominant or exclusive expression of somatostatin was found in 21 (26%) duodenal and 21 (4%) pancreatic NETs. They were classified as sporadic (nZ31) or neurofibromatosis type 1 (NF1)-associated duodenal NETs (nZ3), gangliocytic paragangliomas (GCPGs; nZ6), or poorly differentiated neuroendocrine carcinomas (pdNECs; nZ2). In addition, five duodenal and four pancreatic SOM-NETs were found in five patients with multiple endocrine neoplasia type 1 (MEN1). Metastases occurred in 13 (43%) patients with sporadic or NF1-associated SOM-NETs, but in none of the duodenal or pancreatic MEN1-associated SOM-NETs or GCPGs. Sporadic advanced (stage IV) SOM-NETs were more commonly detected in the pancreas than in the duodenum. None of the patients (including the 821 patients for whom only the clinical records were reviewed) fulfilled the criteria of a somatostatinoma syndrome. Our data show that somatostatin expression is not only seen in sporadic NETs but may also occur in GCPGs, pdNECs, and hereditary NETs. Surgical treatment is effective in most duodenal and many pancreatic SOM-NETs. MEN1-associated SOM-NETs and GCPGs follow a benign course, while somatostatin-producing pdNECs are aggressive neoplasms. The occurrence of the so-called somatostatinoma syndrome appears to be extremely uncommon.

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“…Large non-functioning PanNETs are reported to occur most frequently in the head of the pancreas, possibly because they are most likely to produce cholestasis in this location. Immunohistochemically, they often express various hormones (Kapran et al 2006) and some of them are associated with elevated hormone levels in the blood, reflecting the hormonal immunoreactivity in the tumour. A special histologic feature of glucagon-producing NETs is grossly cystic changes (Yagihashi et al 1992, Ligneau et al 2001.…”

Section: Non-functioning Pannetsmentioning

confidence: 99%

Classification and pathology of gastroenteropancreatic neuroendocrine neoplasms

Klöppel¹

2011

Endocrine-Related Cancer

287

219

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Gastroenteropancreatic neuroendocrine neoplasms (GEP-NENs) are composed of cells with a neuroendocrine phenotype. The old and the new WHO classifications distinguish between well-differentiated and poorly differentiated neoplasms. All well-differentiated neoplasms, regardless of whether they behave benignly or develop metastases, will be called neuroendocrine tumours (NETs), and graded G1 (Ki67 !2%) or G2 (Ki67 2-20%). All poorly differentiated neoplasms will be termed neuroendocrine carcinomas (NECs) and graded G3 (Ki67 O20%). To stratify the GEP-NETs and GEP-NECs regarding their prognosis, they are now further classified according to TNM-stage systems that were recently proposed by the European Neuroendocrine Tumour Society (ENETS) and the AJCC/UICC. In the light of these criteria the pathology and biology of the various NETs and NECs of the gastrointestinal tract (including the oesophagus) and the pancreas are reviewed.

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Multihormonality and entrapment of islets in pancreatic endocrine tumors

Cited by 29 publications

References 16 publications

Site-specific biology and pathology of gastroenteropancreatic neuroendocrine tumors

Site-specific biology and pathology of gastroenteropancreatic neuroendocrine tumors

Somatostatin-producing neuroendocrine tumors of the duodenum and pancreas: incidence, types, biological behavior, association with inherited syndromes, and functional activity

Classification and pathology of gastroenteropancreatic neuroendocrine neoplasms

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