The association of pheochromocytoma with carcinoma of the thyroid gland

Sipple, John H.

doi:10.1016/0002-9343(61)90234-0

Cited by 702 publications

(172 citation statements)

References 26 publications

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“…There are biological arguments supporting a relationship between thyroid tumours and neuroblastomas. Firstly, the tumours of the sympathetic system (pheochromocytomas and, in rare instances, neuroblastomas) can be associated with medullary thyroid carcinoma (Sipple, 1961). Secondly.…”

Section: Dscussiomentioning

confidence: 99%

Epidemiological evidence for a common mechanism for neuroblastoma and differentiated thyroid tumour

Vathaire

François²,

Schlumberger³

et al. 1992

Br J Cancer

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Summan-Because genetic predisposition probably plays an important role in the aetiology of most of childhood cancers. studies of second primaries occurring after these cancers may be particularly informative about possible common genetic mechanisms in both of these cancers. We have studied the incidence of thyroid tumours occumng after cancer in childhood in a cohort of 592 children treated before 1970. Among these children. six later developed a thyroid carcinoma, and 18 developed a thyroid adenoma. Radiation doses received to the thyToid by each of the irradiated children have been estimated using individual radiotherapeutic technical records. Thyroid carcinomas and thyroid adenomas were five times more frequent after irradiation for neuroblastoma than after irradiation for anv other first cancer. This ratio did not depend on sex. nor on time elapsed since irradiation. nor on dose of radiation received for the thyToid gland. This result suggests that there is a common mechanism for the occurrence of neuroblastoma and of differentiated thyroid tumour.

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Section: Dscussiomentioning

confidence: 99%

Epidemiological evidence for a common mechanism for neuroblastoma and differentiated thyroid tumour

Vathaire

François²,

Schlumberger³

et al. 1992

Br J Cancer

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“…More than fifty years ago, the medical community witnessed the birth of a new hereditary endocrine syndrome encompassing medullary thyroid cancer (MTC) and pheochromocytoma: 'Sipple syndrome', named after the author of this seminal description (Sipple 1961).…”

Section: Introductionmentioning

confidence: 99%

“…This achievement was brought about by a combination of astute clinical observation (Sipple 1961, Williams 1965) coupled with advances in immunohistochemistry and hormonal assay technology.…”

Section: Introductionmentioning

confidence: 99%

Advances in risk-oriented surgery for multiple endocrine neoplasia type 2

Machens¹,

Dralle²

2018

Endocrine-Related Cancer

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Genetic association studies hinge on definite clinical case definitions of the disease of interest. This is why more penetrant mutations were overrepresented in early multiple endocrine neoplasia type 2 (MEN2) studies, whereas less penetrant mutations went underrepresented. Enrichment of genetic association studies with advanced disease may produce a flawed understanding of disease evolution, precipitating far-reaching surgical strategies like bilateral total adrenalectomy and 4-gland parathyroidectomy in MEN2. The insight into the natural course of the disease gleaned over the past 25 years caused a paradigm shift in MEN2: from the removal of target organs at the expense of greater operative morbidity to close biochemical surveillance and targeted resection of adrenal tumors and hyperplastic parathyroid glands. The lead time provided by early identification of asymptomatic MEN2 carriers under biochemical surveillance delimits a 'window of opportunity', within which (i) pre-emptive total thyroidectomy alone is adequate, circumventing morbidity attendant to central node dissection; (ii) subtotal 'tissue-sparing' adrenalectomy is sufficient, trading the risk of steroid dependency for the risk of a second pheochromocytoma in the adrenal remnant and (iii) parathyroidectomy is limited to enlarged glands, trading the risk of postoperative hypoparathyroidism for the risk of leaving behind hyperactive parathyroid glands. Future research should delineate further the mutation-specific, age-dependent penetrance of pheochromocytoma and primary hyperparathyroidism to refine the risk-oriented approach to MEN2. The sweeping changes in the management of MEN2 since the new millenium hold the hope that death and major morbidity from this uncommon disease can be eliminated in our lifetime.

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“…While the first clinical association between pheochromocytoma and medullary thyroid carcinoma (MTC) was described in 1961 by Sipple (Sipple 1961), it was only 5 years later that the first description of oral mucosal neuromas in 2 patients with MTC and pheochromocytoma was reported in the English literature (Williams & Pollock 1966). The term multiple endocrine neoplasia type 2 was proposed by Steiner a in 1968 (Steiner et al 1968) and the rare association of hereditary MTC with a mucosal neuroma phenotype was eventually named multiple endocrine neoplasia type 2B (MEN2B) by Chong andcoworkers in 1975 (Chong et al 1975).…”

Section: Introductionmentioning

confidence: 99%

A comprehensive review on MEN2B

Castinetti¹,

Moley²,

Mulligan³

et al. 2018

Endocrine-Related Cancer

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MEN2B is a very rare autosomal dominant hereditary tumor syndrome associated with medullary thyroid carcinoma (MTC) in 100% cases, pheochromocytoma in 50% cases and multiple extra-endocrine features, many of which can be quite disabling. Only few data are available in the literature. The aim of this review is to try to give further insights into the natural history of the disease and to point out the missing evidence that would help clinicians optimize the management of such patients. MEN2B is mainly characterized by the early occurrence of MTC, which led the American Thyroid Association to recommend preventive thyroidectomy before the age of 1 year. However, as the majority of mutations are de novo, improved knowledge of the nonendocrine signs would help to lower the age of diagnosis and improve long-term outcomes. Future large-scale studies will be aimed at characterizing more in detail the main characteristics and outcomes of MEN2B.

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The association of pheochromocytoma with carcinoma of the thyroid gland

Cited by 702 publications

References 26 publications

Epidemiological evidence for a common mechanism for neuroblastoma and differentiated thyroid tumour

Epidemiological evidence for a common mechanism for neuroblastoma and differentiated thyroid tumour

Advances in risk-oriented surgery for multiple endocrine neoplasia type 2

A comprehensive review on MEN2B

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