Glucagonoma as part of the polyglandular adenoma syndrome

Croughs, R. J. M.; Hulsmans, H. A. M.; Israël, David E.; Hackeng, W. H. L.; Schopman, W.

doi:10.1016/0002-9343(72)90059-9

Cited by 46 publications

(12 citation statements)

References 16 publications

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“…Most proven gastrinomas in which the staining reactions are recorded have argyrophil granules (Cavallero, Solcia & Sampietro 1967, Royston, Brew, Garnham, Stagg & Polak 1972, Creutzfeldt et al 1975), but again it has been suggested that granules in tumour cells do not necessarily stain as, or show the immunological reactions of their normal counterparts (Schulte et al 1969, Vassallo et al 1972) and Greider, Steinberg & McGuigan (rg72) have failed to correlate argyrophilia with positive immunofluorescence for gastrins. Glucagonomas have been generally described as argyrophil and immunologically identifiable (McGavran, Unger, Recant, Polk, Kilo & Levin 1966, Lomsky, Langr & Vortel 1969, Croughs, Huismans, Israel, Hackerg & Schopman 1972. Both of our immunologically proven glucagonomas were argyrophil, but were also well differentiated; we do not know how an anaplastic tumour would behave immunoIogicalIy or tinctorially.…”

Section: Discussionmentioning

confidence: 66%

Morphology and staining patterns of endocrine cell tumours in the gut, pancreas and bronchus and their possible significance

Jones

Dawson

1977

Histopathology

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We have studied 109 endocrine cell tumours of the gastrointestinal tract, pancreas and bronchus in terms of histological pattern and histochemical staining with immunocytochemical studies on seven tumours. As a result we believe that previous histological classifications need modification. 5-Hydroxytryptamine secreting tumours have a carcinoid (A1) pattern with, in some cases, an additional tubuloacinar element (A1A2) and definable histochemical reactions; well differentiated gastrinomas, insulinomas and glucagonomas are associated commonly, but not exclusively, with particular histological patterns and argyrophilia, but no such association exists for less differentiated tumours. This may be related to the synthesis of precursor hormones by less differentiated tumours. Mixed patterns are common, particularly in tumours of foregut derivation. Prospective studies planned to correlate histology, histochemistry, immunocytochemistry and ultrastructure are needed on all endocrine cell tumours.

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Section: Discussionmentioning

confidence: 66%

Morphology and staining patterns of endocrine cell tumours in the gut, pancreas and bronchus and their possible significance

Jones

Dawson

1977

Histopathology

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“…Finally, as the frequency of reported cases has increased, the tendency of some glucagonomas to appear as part of multiple endocrine neoplasia syndromes has been observed (19,20).…”

Section: Historical Backgroundmentioning

confidence: 99%

“…In such cases, affected patients may have asymptomatic elevation of plasma glucagon without evidence of neoplastic disease (31) or else harbor clinically obvious glucagon-secreting pancreatic tumors as part of their multiple neoplasia syndromes (20,(32)(33)(34). Familial hyperglucagonemia per se has also been observed in two families in which one member of each had a proven glucagonoma (19,35). It is noteworthy that, in both reported families, the predominant molecular weights of the species of glucagon in the asymptomatic, ostensibly tumor-free, relatives were 9,000 and 30,000, whereas that of the tumor-bearing propositi was the 3,500 or "true" glucagon fraction of major biological activity.…”

Section: Geneticsmentioning

confidence: 99%

“…Whether manifested as fasting hyperglycemia (4, 5, 9, 10, 13,18, 20, 24, 28, 32, 34-37, 50-53, 65, 68, 72-74, 77, 78, 81, 82, 85, 88, 90, 92, 95, 108) or as "chemical" diabetes after glucose administration (3,7,12,15,16,18,19,53,65,66,73,76,79,80,84,86,89,91,94,100,107,110), glucose intolerance is the predominate clinical sign of the glucagonoma syndrome (Table 5). Fasting hyperglycemia occurs in the majority (67%) of such patients.…”

Section: Hyperglycemiamentioning

confidence: 99%

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The Glucagonoma Syndrome: Clinical Features, Diagnosis, and Treatment

1981

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Historical BackgroundIslet cell tumors of the pancreas have been described for over 50 yr. Although carcinoma arising from islet tissue had been noted since the turn of the century (1), not until 1927 was the first case of a hormonally active pancreatic neoplasm, an insulinoma, recorded (2). For many years thereafter, pancreatic endocrine tumors were characterized as either insulin-producing or "nonfunctional," the latter being derived from cells apparently devoid of clinically significant hormonal activity. With time, however, other islet cell secretory products were discovered and their tumors identified with various clinical syndromes. Whereas the hypoglycemia of insulinomas and hyperacidity of gastrinomas figured prominently in the initial recognition of these neoplasms, the clinical signs of glucagon-producing tumors were less readily apparent and not as easily attributable to specific hormone excess.Becker and co-workers (3) are credited with the first description of a glucagonoma. Their case, reported in 1947 but diagnosable only in retrospect, typifies what is now regarded as a classic clinical presentation of the syndrome. The patient, a 45-yr-old woman, sought attention because of a skin rash that, over a period of 8 months, progressed to involve almost her entire body. Weight loss, weakness, and amenorrhea were additional complaints. The rash was symmetrically distributed and characterized by confluent, pruritic areas of macular, erythematous, and vesicular erruptions showing evidence of exfoliation and superficial necrosis. Stomatitis and onchonolysis were also found. Laboratory examination revealed a normochromic anemia, mild fasting hyperglycemia, and hypoproteinemia. Deep venous thrombosis developed while the patient was in hospital, and she succumbed to cardiorespiratory failure. At autopsy, an islet cell neoplasm was discovered "replacing the body

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“…The serum TSH and T4 were within the normal range but the serum T3 level was low. The serum rT3 was at the lower limit of the fact that she had other functioning adenomas, such as seen in multiple endocrine adenomatosis sometimes associated with glucagonoma (Croisier et al, 1971;Croughs et al, 1972).…”

Section: Endocrinolmentioning

confidence: 92%