The Glucagonoma Syndrome

Shupack, Jerome L.; Berczeller, Peter H.; Stevens, Dwight M.

doi:10.1111/j.1524-4725.1978.tb00419.x

Cited by 15 publications

(23 citation statements)

References 40 publications

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“…Although localized vascular invasion by tumor cells (73,88,105) is not uncommon, mitoses (6,24,50,79,84,88) are usually absent or noted infrequently. Extreme nuclear atypia or frank anaplasia (8,73,76) is rare.…”

Section: Malignancymentioning

confidence: 97%

“…Forty-two of 84 cases (50%) of proven or probable glucagonoma syndromes showed evidence of metastatic spread. Liver (4-7, 9, 10, 13, 15, 16, 18, 20, 24, 33, 34, 36, 37, 51, 53, 65, 67, 72, 76-78, 79, 83-85, 105, 107, 108) and adjacent nodes (3,7,15,16,24,33,35,79,85,86,88,105) accounted for the vast majority of secondary deposits ( Table 3). The histology of metastases usually parallels that of the primary neoplasm.…”

Section: Malignancymentioning

confidence: 97%

“…Definitive islet structure or even less precise lobular or glandular elements (33,73,82,105) seldom exist. More commonly, tumor cells are pleomorphic and appear in the form of regular sheets or trabeculae separated by fibrous strands (6,8,10,24,33,51,79,84,94), which may occasionally suggest some degree of orga- …”

Section: Histologymentioning

confidence: 99%

“…Of 60 cases in which tumor site was recorded, 30 (50%) appeared confined to the region of the tail (5,7,13,15,18,20,24,35,37,50,52,65,66,(71)(72)(73)(74)(75)(76)(77)(78)(79)(80), whereas tumors of the head or neck (4,13,21,37,73,81,82), body (13,18,(83)(84)(85)(86)(87), and body-tail region (3,28,33,68,89,90) were less frequently described. In seven cases multiple tumors (91) or diffuse involvement by a single mass (6,10,32,51,88,92) precluded specific localization of th...…”

Section: Location and Sizementioning

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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Section: Malignancymentioning

confidence: 97%

Section: Malignancymentioning

confidence: 97%

Section: Histologymentioning

confidence: 99%

Section: Location and Sizementioning

confidence: 99%

Section: Hyperglycemiamentioning

confidence: 99%

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The Glucagonoma Syndrome: Clinical Features, Diagnosis, and Treatment

1981

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The Acrodermatitis Enteropathica-like Syndrome

Brazin¹

1979

Arch Dermatol

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Glucagonomas: Ultrastructure and immunocytochemistry

Warner

Block

Hafez

et al. 1983

Cancer

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Pancreatic tumors harboring glucagon immunoreactive cells were found in four patients with diabetes mellitus. Alpha‐cell (glucagon) granules were present in three tumors; pancreatic polypeptide (PP) immunoreactive cells were detected in two. In two patients the tumors were malignant and one of these had the glucagonoma syndrome; the other was a member of a family with MEN‐type I syndrome. These cases illustrate three clinical subtypes of glucagonoma.

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The Glucagonoma Syndrome

Cited by 15 publications

References 40 publications

The Glucagonoma Syndrome: Clinical Features, Diagnosis, and Treatment

The Glucagonoma Syndrome: Clinical Features, Diagnosis, and Treatment

The Acrodermatitis Enteropathica-like Syndrome

Glucagonomas: Ultrastructure and immunocytochemistry

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