Multiple endocrine neoplasia: How many syndromes?

Schimke, R. Neil

doi:10.1002/ajmg.1320370317

Cited by 45 publications

(13 citation statements)

References 119 publications

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“…Neuroendocrine tnmours (carcinoids) of the lung, thymus and stomach (embryonic foregut tissue) are considered to be an integral part of MEN 1 [20,72] with either low or high penetrance in affected families [20]. They are most frequent in the thymus and/or mediastinum [ [16,37,52,54,80], whereas the jejunum and ileum are only occasionally affected [13,73].…”

Section: Morphological and Clinical Features Of Men 1 Lesionsmentioning

confidence: 99%

Multiple endocrine neoplasia type 1 (MEN 1) revisited

Padberg

Schröder

Heitz

et al. 1995

Vichows Archiv A Pathol Anat

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Multiple endocrine neoplasia type 1 (MEN 1) is an inherited disease of the neuroendocrine cell system affecting primarily the parathyroids, pancreas, duodenum and the anterior pituitary. The pancreatic and duodenal turnouts may metastasize, but generally have a low malignant potential. The diagnosis of MEN 1 is usually made in the second decade of life and based on the involvement of at least two organs and a family history. The recent discovery of the MEN 1 locus on the centromeric region of the long arm of chromosome 11 may become a further diagnostic criterion. The use of flanking DNA markers permits presymptomatic testing for MEN 1 in affected families.

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Section: Morphological and Clinical Features Of Men 1 Lesionsmentioning

confidence: 99%

Multiple endocrine neoplasia type 1 (MEN 1) revisited

Padberg

Schröder

Heitz

et al. 1995

Vichows Archiv A Pathol Anat

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“…Paragangliomas (PGLs) are rare, highly vascularized, and generally benign tumors arising mainly (80% of cases) in the neural-crest cells of the head and neck or the intra-abdominal paraganglia (17%) of the sympathoadrenal neuroendocrine system (Lack, 1997). Most of these tumors and PCCs occur sporadically, although about 25% of PCCs and an uncertain percentage of PGLs may be associated with hereditary syndromes (Schimke, 1990;Anderson and Lynch, 1993;Maher et al, 1996;Baysal et al, 2000;Niemann and Muller, 2000;Astuti et al, 2001;Carney and Stratakis, 2002). Other genes, as yet undiscovered, must be involved in not only familial cases but also the development of sporadic tumors.…”

Section: Introductionmentioning

confidence: 99%

A novel candidate region linked to development of both pheochromocytoma and head/neck paraganglioma

Cascón

Ruiz‐Llorente

Rodríguez‐Perales

et al. 2004

Genes Chromosomes & Cancer

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Although the histologic distinction between pheochromocytomas and head and neck paragangliomas is clear, little is known about the genetic differences between them. To date, various sets of genes have been found to be involved in inherited susceptibility to developing both tumor types, but the genes involved in sporadic pathogenesis are still unknown. To define new candidate regions, we performed CGH analysis on 29 pheochromocytomas and on 24 paragangliomas mainly of head and neck origin (20 of 24), which allowed us to differentiate between the two tumor types. Loss of 3q was significantly more frequent in pheochromocytomas, and loss of 1q appeared only in paragangliomas. We also found gain of 11q13 to be a significantly frequent alteration in malignant cases of both types. In addition, recurrent loss of 8p22-23 was found in 62% of pheochromocytomas (including all malignant cases) versus in 33% of paragangliomas, suggesting that this region contains candidate genes involved in the pathogenesis of this abnormality. Using FISH analysis on tissue microarrays, we confirmed genomic deletion of this region in 55% of pheochromocytomas compared to 12% of paragangliomas. Loss of 8p22-23 appears to be an important event in the sporadic development of these tumors, and additional molecular studies are necessary to identify candidate genes in this chromosomal region.

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“…Ninety percent of insulinomas in humans are solitary lesions; however, multiple endocrine neoplasia (MEN) patients usually have multiple pancreatic endocrine t~m o r s .~~!~~ Pheochromocytomas have been linked with islet cell tumors in one of the MEN syndromes of humans. 37 No clinical signs, other than inability to rise in two cattle, were observed on antemortem inspection of the cows with islet cell tumors. Seizures have been reported in one case of functional bovine islet cell tumor.…”

Section: Discussionmentioning

confidence: 98%

A Retrospective Study of Pancreatic Tumors in Slaughter Cattle

1996

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Abstract. Sixteen primary pancreatic tumors were found in a retrospective study of bovine pancreatic lesions detected in slaughtered cattle. Eleven islet cell tumors and three pancreatic exocrine Carcinomas were identified based on light microscopy and immunohistochemistry. Nine of 1 1 islet cell tumors were classified as malignant. Metastatic sites included iliac, mediastinal, hepatic, and mesenteric lymph nodes, peritoneum, mesentery, and liver. Six cows with multiple islet cell tumors also had pheochromocytomas. All 1 1 islet cell tumors had positive immunoreactivity to insulin and somatostatin. Three tumors also contained cells immunoreactive for glucagon and two tumors contained pancreatic polypeptide immunoreactive cells. Immunoreactivity of tumor cells in metastatic sites was similar to their respective primary tumors. All exocrine pancreatic carcinomas metastasized widely and were immunonegative for insulin, somatostatin, glucagon, and pancreatic polypeptide. No mixed endocrine*xocrine tumors were identified. None of the endocrine or exocrine tumors contained amyloid. Additional primary tumors of the bovine pancreas included one neurofibroma and one neurofibrosarcoma. Additional cases with lesions of the bovine pancreas included nodular hyperplasia in 15 cows, exocrine acinar atrophy and fibrosis in four cows (two of which also had pancreatic lithiasis), pancreatitis in one cow, peripancreatic fibrosis in two cows, pancreatic steatosis in one animal, and pancreatic hemorrhages in one cow.Key words: Cattle; glucagon; immunohistochemistry; insulin; pancreas; pancreatic polypeptide; somatostatin.Few bovine pancreatic tumors have been reported in the veterinary literature. Only two nonmetastatic islet cell tumors36 and 16 metastatic islet cell tumors in cattle have been r e p~r t e d l .~.~.~~.~~.~~.~~.~~ and all but one report pertains to cattle in Italy. In only one report has insulin production been confirmed in a bovine islet cell tumor.39 Pancreatic exocrine adenomas have been described in the l i t e r a t~r e ,~.~~ but are often difficult to separate from nodular hyperplasia of the exocrine pancreas. Ten exocrine carcinomas of the pancreas have been r e p~r t e d .~~,~~ One fibrosarcoma has been observed in the bovine pancreas.I2Other diseases of the bovine pancreas include pancreatic l i t h i a~i s ,~~~~l 1,19,40 pancreatitis,20 pancreatic fluke infestation,16J9 pancreatic atrophy associated with nutritional defi~iencies,'~ and diabetes mellitus due to an infection with the foot-and-mouth disease virus.19 The purpose of this study was to characterize a group of pancreatic tumors and other pancreatic lesions taken from cattle examined at slaughter. Materials and MethodsTissues from lesions detected during routine slaughter and inspection procedures were submitted to the United States Department of Agriculture Food Safety and Inspection Service Eastern Laboratory. Tissues fixed in 10% neutral buffered formalin were embedded in paraffin, sectioned at 5 Fm, stained with hematoxylin and eosin and Congo r...

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Multiple endocrine neoplasia: How many syndromes?

Cited by 45 publications

References 119 publications

Multiple endocrine neoplasia type 1 (MEN 1) revisited

Multiple endocrine neoplasia type 1 (MEN 1) revisited

A novel candidate region linked to development of both pheochromocytoma and head/neck paraganglioma

A Retrospective Study of Pancreatic Tumors in Slaughter Cattle

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