Metastatic parathyroid carcinoma in the MEN2A syndrome

Jenkins, Paul J.; Satta, M. A.; Simmgen, Marcus; Drake, William M; Williamson, Catherine; Lowe, Donna; Britton, K. E.; Chew, Shern L.; Thakker, Rajesh; Besser, G. M.

doi:10.1046/j.1365-2265.1997.3421147.x

Cited by 76 publications

(42 citation statements)

References 10 publications

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“…40 PT-CA occurs rarely in MEN1 41 and a single case has been reported in a patient with type 2A MEN (MEN2A). 42 In contrast to the 3-4:1 predominance of parathyroid adenomas in women, PT-CA has a roughly equal male to female sex ratio. 36 Moreover, PT-CA tends to occur B10 years earlier than parathyroid adenoma.…”

Section: Parathyroid Carcinomamentioning

confidence: 98%

Parathyroid tumors and related disorders

2011

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Primary hyperparathyroidism (P-HPT) is a common endocrine disorder that occurs as a result of adenomas (80-85%), hyperplasias (10-15%) or carcinomas (o1%) of the parathyroid glands. Molecular genetic analyses of heritable P-HPT syndromes have provided considerable insight into the understanding of sporadic parathyroid tumors and hyperplasias. This review will focus on the criteria for classification of parathyroid proliferative disorders and will highlight our understanding of these lesions at the molecular level. Advances in radiological imaging techniques together with the rapid intraoperative parathyroid hormone assay will be reviewed with respect to current treatment approaches for P-HPT.

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Section: Parathyroid Carcinomamentioning

confidence: 98%

Parathyroid tumors and related disorders

2011

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“…Se presenta como una entidad aislada o puede asociarse a un síndrome como NEM tipo I, IIA o hipercalcemia hipocalciúrica familiar [9][10][11] . La clínica y el laboratorio pueden sugerir el diagnóstico, pero los hallazgos son inespecífi cos. Calcemias sobre 14 mg/dL son muy sugerentes.…”

Section: Discussionunclassified

Hiperparatiroidismo primario y cáncer de paratiroides: Caso clínico

et al. 2010

Rev Chil Cir

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Primary hyperparathyroidism and parathyroid cancer. Report of one caseWe report a 51 years old female operated for a primary hyperparathyroidism that relapsed in two occasions. After the fi rst and second operation, the pathological study of the excised glands disclosed an adenoma. After the second relapse, a parathyroid gland in the right lateral and upper cervical region, located with technetium (Tc)-99m Sestamibi scintigraphy, was excised. The pathological study of the surgical piece disclosed a parathyroid carcinoma without local lymph node involvement. A postoperative 18F-fl uorodexyglucose positron emission tomography did not show tumor dissemination.Key words: Hyperparathyroidism, parathyroid cancer, parathyroid adenoma. ResumenPresentamos un caso de sexo femenino, operada por hiperparatiroidismo primario, con 2 recidivas posteriores. En las 2 primeras oportunidades, el diagnóstico histológico fue compatible con adenoma. La última intervención se debió realizar por una glándula ubicada, de acuerdo al Sestamibi Tc-99m, en la región cervical lateral alta derecha. Se realizó exploración supraomohiodea, efectuando la extirpación de la glándula y adenopatías peritumorales. La biopsia defi nitiva informó carcinoma de paratiroides y ganglios linfáticos negativos para neoplasia. Se realizó estudio de diseminación y PET-FDG de control en el postoperatorio alejado, sin nuevos hallazgos. Entre la primera y la segunda intervención, se realizó una tiroidectomía total por un bocio uninodular, cuya biopsia informó 3 paratiroides normotípicas, intratiroideas. En suma, se lograron identifi car histológicamente 6 glándulas paratiroides.

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“…The ectopic production of corticotropin-releasing hormone (CRH) and adrenocorticotropic hormone (ACTH) and the secretion of VIP, insulin, and glucagon were described in individuals with MTC (52,53,189,190) …”

Section: What Should Comprise the Treatment Of Individuals With Hormomentioning

confidence: 99%

Diagnóstico, tratamento e seguimento do carcinoma medular de tireoide: recomendações do Departamento de Tireoide da Sociedade Brasileira de Endocrinologia e Metabologia

Maia

Siqueira

Kulcsar

et al. 2014

Arq Bras Endocrinol Metab

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Introduction: Medullary thyroid carcinoma (MTC) originates in the thyroid parafollicular cells and represents 3-4% of the malignant neoplasms that affect this gland. Approximately 25% of these cases are hereditary due to activating mutations in the REarranged during Transfection (RET) proto-oncogene. The course of MTC is indolent, and survival rates depend on the tumor stage at diagnosis. The present article describes clinical evidence-based guidelines for the diagnosis, treatment, and follow-up of MTC. Objective: The aim of the consensus described herein, which was elaborated by Brazilian experts and sponsored by the Thyroid Department of the Brazilian Society of Endocrinology and Metabolism, was to discuss the diagnosis, treatment, and follow-up of individuals with MTC in accordance with the latest evidence reported in the literature. Materials and methods: After clinical questions were elaborated, the available literature was initially surveyed for evidence in the MedLine-PubMed database, followed by the Embase and Scientific Electronic Library Online/Latin American and Caribbean Health Science Literature (SciELO/Lilacs) databases. The strength of evidence was assessed according to the Oxford classification of evidence levels, which is based on study design, and the best evidence available for each question was selected. Results: Eleven questions corresponded to MTC diagnosis, 8 corresponded to its surgical treatment, and 13 corresponded to follow-up, for a total of 32 recommendations. The present article discusses the clinical and molecular diagnosis, initial surgical treatment, and postoperative management of MTC, as well as the therapeutic options for metastatic disease. Conclusions: MTC should be suspected in individuals who present with thyroid nodules and family histories of MTC, associations with pheochromocytoma and hyperparathyroidism, and/or typical phenotypic characteristics such as ganglioneuromatosis and Marfanoid habitus. Fine-needle nodule aspiration, serum calcitonin measurements, and anatomical-pathological examinations are useful for diagnostic confirmation. Surgery represents the only curative therapeutic strategy. The therapeutic options for metastatic disease remain limited and are restricted to disease control. Judicious postoperative assessments that focus on the identification of residual or recurrent disease are of paramount importance when defining the follow-up and later therapeutic management strategies. Arq Bras Endocrinol Metab. 2014;58(7):667-700

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Metastatic parathyroid carcinoma in the MEN2A syndrome

Cited by 76 publications

References 10 publications

Parathyroid tumors and related disorders

Parathyroid tumors and related disorders

Hiperparatiroidismo primario y cáncer de paratiroides: Caso clínico

Diagnóstico, tratamento e seguimento do carcinoma medular de tireoide: recomendações do Departamento de Tireoide da Sociedade Brasileira de Endocrinologia e Metabologia

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