Multiple Endocrine Adenomatosis; Multiple Hormone-Producing Tumours, a Familial Syndrome

Wermer, Paul

doi:10.1016/s0300-5089(21)00082-1

Cited by 49 publications

(5 citation statements)

References 20 publications

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“…In MEA type I, Wermer has postulated that the inherited tendency manifests itself through pleiotropism [1]. Genetically, this inherited syndrome is a phenotypic effect of a pleiotropic autosomal gene, as a result of which, for instance, the islet cell tumor is the direct phenotypic expression of the gene (autophene) and the acid-peptic ulcer, which is secondary to the tumor hypergastrinism, is the allophene.…”

Section: Discussionmentioning

confidence: 99%

“…1). Wermer's syndrome (MEA type I) [1] is a heritable condition involving independent pathologic changes in the anterior lobe of the pituitary, parathyroid glands, pancreatic islets and, occasionally, adrenal cortex and thyroid gland. Sometimes there is lipomatosis.…”

Section: Types Of Multiple Endocrinopathiesmentioning

confidence: 99%

“…Clinical endocrinopathies occur in either a sporadic or a familial form [1]. The differentiation is occasionally difficult because a patient who appears at first to have the sporadic form may actually have the potentiality of developing multiple endocrinopathies and may even be an affected member of a family with occult endocrinopathies.…”

mentioning

confidence: 99%

“…On the other hand, among some of the familial endocrinopathies, the most frequent pathology is hyperplasia [4,5], while in other familial syndromes, the reverse is true [6,7]; but even in cases of multiple endocrine neoplasia (MEN) type II, medullary carcinoma may be preceded by C cell hyperplasia [8]. It is also true that the entire gamut of hyperplasia, microadenoma, adenoma, carcinoma, and carcinoid changes may be present in patients with multiple endocrinopathies [1]. In fact, the key feature of multiple endocrine adenopathies is multiplicity of involvement, not only of several endocrine organs in predictable combinations, but also, perhaps more importantly, multiple cellular distribution and bilaterality of involvement of all glands, such as the 4 parathyroid glands, diffuse islet cell changes, and bilateral adrenal hyperplasias and tumors in various combinations.…”

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confidence: 99%

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

Friesen

1979

World j. surg.

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The majority of the members of 2 families afflicted with genetic manifestations of the multiple endocrine adenopathy (MEA) type I syndrome have been prospectively studied. The slow and insidious onset of endocrinopathies in these high‐risk relatives was preceded by chemical and humoral abnormalities which could be surgically treated early in the course of the endocrinopathy. The predominant pathologic process in those patients diagnosed prospectively was that of hyperplasia with some benign adenomatous developments; malignancy was not observed except in those studied retrospectively. Screening protocols for the detection of multiple endocrinopathies in the symptomatic patient with one endocrinopathy, as well as in asymptomatic family relatives, appear to yield endocrinopathies at an earlier stage of development. Antral G cell hyperplasia was observed on endoscopic mucosal biopsy to be associated with the development of clinical endocrinopathies; it is not known yet whether this is of predictive value. The pathogenesis of multiple APUD cell abnormalities, though not known, apparently involves an embryologic programming of cells from the neuroectoderm, as well as a genetic pleiotropic factor and environmental influences.

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Section: Discussionmentioning

confidence: 99%

Section: Types Of Multiple Endocrinopathiesmentioning

confidence: 99%

mentioning

confidence: 99%

mentioning

confidence: 99%

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

Friesen

1979

World j. surg.

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“…This diffuse involvement of endocrine systems has a genetic pathogenesis involving pleiotropism, as pro-posed by Wermer [1]. In this mechanism, the pleiotropic autosomat gene may be activated in all cells of its various target organs; thus, all of the chief cells (or clear cells) in all 4 parathyroid glands can potentially react identically, which explains the frequent multiplicity and/or bitaterality of inherited tumors.…”

Section: Pathogenesismentioning

confidence: 99%

Cimetidine in the management of synchronous crises of MEA I

Friesen

et al. 1980

World j. surg.

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The clinical problems associated with the simultaneous and critical presentation of acute hyperparathyroidism and perforated jejunal ulceration in multiple endocrine adenopathy (MEA type I) require a multidisciplinary approach to management. In the patient described here, the use of cimetidine, a histamine-2 receptor blocker, provided sufficient time after surgical closure of the ulcer to control the hypercalcemic crisis medically and surgically, until the persistent hypergastrinemia could be corrected by sequential excisions of the stomach and pancreaticoduodenal apudomas and hyperplasia. The serum gastrin levels, basal and stimulated (by secretin), provide necessary indications for surgical management of otherwise undetected islet cell pathology. The simultaneously associated pituitary hyperproiactinemia in this patient required both surgical and radiologic therapy.Certain endocrinopathies occur in combination sufficiently frequently in predictable associations to be designated as multiple endocrine adenopathy (MEA) syndromes. 123[2], with medullary carcinoma of the thyroid, adrenal medullary pheochromocytoma(s), and hyperparathyroidism; and MEA type lib [3], with medullary carcinoma of the thyroid, adrenal medullary pheochromocytoma, and mucosal neuromas of the alimentary tract. The endocrinopathies within each of these syndromes usually occur metachronously but, when they occur synchronously, the management of such patients requires multidisciplinary attention to diagnosis and treatment. An example of such a critical clinical condition in a young patient is presented with a critique of the sequential management through the multiple and synchronous endocrinopathies. IncidenceIn MEA type I, the relative incidence of the endocrine components is reported to be hyperparathyroidism in about 90% of cases; pancreatic hypergastrinism, hyperinsulinism, and hyperglucagonism in about 50%; and pituitary abnormalities in about 30% [4]. However, in all probability, the incidence of each is higher. When the endocrinopathies occur metachronously, the first to elucidate symptoms is usually parathyroid in origin, while the pituitary disease is the latest to present clinically. When pancreatic islet abnormalities pre-0364-2313/80/0004-0123 $01.40

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Pancreatic endocrine tumours: evidence for a tumour suppressor pathogenesis and for a tumour suppressor gene on chromosome 17p

Beghelli

Pelosi²,

Zamboni

et al. 1998

J. Pathol.

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Multiple Endocrine Adenomatosis; Multiple Hormone-Producing Tumours, a Familial Syndrome

Cited by 49 publications

References 20 publications

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

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

Cimetidine in the management of synchronous crises of MEA I

Pancreatic endocrine tumours: evidence for a tumour suppressor pathogenesis and for a tumour suppressor gene on chromosome 17p

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