Primary Adrenocortical Nodular Dysplasia, a Distinct Subtype of Cushing’s Syndrome

Larsen, Jennifer L.; Cathey, William J.; Odell, William D.

doi:10.1016/s0022-5347(17)45072-5

Cited by 7 publications

(7 citation statements)

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“…It is well known that primary adrenocortical micronodular dysplasia is one of the causes of Gushing's syndrome [24][25][26]. Its onset in young patients is characteristic with a slight female predominance.…”

Section: Discussionmentioning

confidence: 99%

Acromegaly with Hyperprolactinemia Developed after Bilateral Adrenalectomy in a Patient with Cushing's Syndrome due to Adrenocortical Nodular Hyperplasia.

et al. 1993

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Abstract. A 27-yr-old woman was referred for evaluation of acromegaly and hyperprolactinemia. She had undergone left adrenalectomy at 12 and right adrenalectomy at 17 for Cushing's syndrome due to adrenocortical nodular hyperplasia. At this time a pituitary tumor was found by brain computerized tomography, but plasma levels of growth hormone (GH), prolactin (PRL) and adrenocorticotropin (ACTH) were normal. When she was 23, symptoms and signs of acromegaly and subsequently galactorrhea-amenorrhea had developed. Plasma GH and PRL were increased and she was followed up by the administration of bromocriptine (2.5 mg-12.5 mg/day, p.o.). However the plasma GH level had been increasing gradually. On admission, plasma GH and PRL were high (19.5 µg/L, 61.0 µg/L, respectively) and increased in response to thyrotropin releasing hormone (TRH, 500 µg iv). An intrasella mass, which had been detected when she was 17, had become enlarged and was removed by Hardy's operation. Microscopically, the resected tumor was an eosinophilic adenoma. Immunohistochemical studies showed GH, PRL and ACTH positive cells localized in the tumor. Immunoultrastructural analysis of the tumor confirmed that GH, PRL and ACTH were present in secretory granules and Golgi apparatus in the tumor cells. The patient was a rare case of acromegaly with hyperprolactinemia developed after bilateral adrenalectomy of fishing's syndrome due to adrenocortical nodular hyperplasia, all of which manifestations may be caused by a GH, PRL and ACTH secreting pituitary adenoma.

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Section: Discussionmentioning

confidence: 99%

Acromegaly with Hyperprolactinemia Developed after Bilateral Adrenalectomy in a Patient with Cushing's Syndrome due to Adrenocortical Nodular Hyperplasia.

et al. 1993

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“…Em 1984, o termo PPNAD (primary pigmented nodular adrenocortical disease) foi sugerido para identificar esta nova doença e, novamente, foi ressaltada a associação com outros tumores e hiperpigmentação de pele (5). Revisão detalhada, macro e microscópica, das adrenais de 8 pacientes portadores dessa doença, realizada por pesquisadores do NIH, também permitiu concluir que a denominação mais adequada fosse PPNAD, e sugere que as denominações anteriores, como displasia adrenal bilateral, hiperplasia micronodular adrenal, hiperplasia adenomatosa adrenal etc, fossem abandonadas, já que a doença não era uma patologia pré-maligna como o nome displasia poderia sugerir, não existia, em geral, hiperplasia e, sim, atrofia do córtex adrenal internodular, podiam existir macronódulos e não havia evidências de que os nódu-los fossem adenomatosos ou neoplásicos.…”

Section: Posteriormente Pesquisadores Do N a T I O N A Lunclassified

Complexo de Carney: relato de um caso e revisão da literatura

Almeida

Villares

Mendonça

2004

Arq Bras Endocrinol Metab

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RESUMOComplexo de Carney (CNC) pode ser definido como uma forma de neoplasia endócrina múltipla familial associada a alteração de pigmentação cutânea e de mucosa, doença nodular pigmentosa primária das adrenais, mixomas cardíacos e cutâneos, adenomas hipofisários produtores de GH e PRL, neoplasia testicular, adenoma ou carcinoma de tireóide, além de cistos ovarianos. CNC tem herança autossômica dominante e possui manifestações clínicas que são, em alguns aspectos, similares às da síndrome de McCune-Albright. Recentemente, genes envolvidos na via de sinalização dependente de AMPc foram implicados na etiologia do CNC. Vamos apresentar, inicialmente, um caso de um paciente masculino de 17 anos com doença adrenal nodular pigmentosa, lentiginose facial e osteoporose severa. A seguir, procuramos analisar os aspectos clínicos e a genética molecular do CNC, assim como descrever os critérios diagnósticos e recomendações para o seguimento. Carney complex (CNC) is a familial multiple neoplasia syndrome associated with abnormal skin and mucosal pigmentation, primary pigmented nodular adrenocortical disease (PPNAD), cardiac and cutaneous myxomas, GH and PRL pituitary adenoma, testicular tumors, thyroid adenoma or carcinoma and ovarian cysts. CNC is inherited as an autosomal dominant trait and has some clinical similarities to McCuneAlbright syndrome. Recently, genes related to stimulation of the cAMP signaling pathway have been considered candidates for causing CNC. We report a 17-yr-old man with PPNAD, lentigines on the face and severe osteoporosis. In this article we aim at describing the clinical aspects and molecular genetics of CNC and also summarizing diagnostic criteria for CNC and recommendations for follow-up.

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“…Some have suggested that the adrenal hyperplasia is secondary to circulating stimulatory antibodies analogous to Graves' disease [Teding van Berkhout et al, 1986;Wulffraat et al, 1988;Young et al, 19891 although it is difficult to account for the other components of the syndrome on this basis. Conceivably, all familial cases of micronodular pigmented adrenal hyperplasia may be examples of this syndrome, since it is possible that the cutaneous findings are so mild as to be overlooked in some patients, particularly those who are not overly cushingoid in appearance [Arce et al, 1978;Bohm et al, 1983;Donaldson et al, 1981;Larsen et al, 1986;Hodge and Froesch, 19881.…”

Section: Schweitzer-cagianut-carney Syndromementioning

confidence: 99%

Multiple endocrine neoplasia: How many syndromes?

Schimke¹

1990

Am. J. Med. Genet.

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The phrase multiple endocrine neoplasia (MEN) generally denotes an association of tumors so specific as to constitute a syndrome. Three well-recognized such syndromes exist. All are autosomal dominant traits and all have been tentatively mapped to specific chromosomes. Other purported endocrine tumor syndromes have been suggested either as new entities or as subtypes of the existing MEN syndromes. The evidence in favor of these contentions is examined critically. Only one "new" association, that of pheochromocytomas and islet cell tumors, seems reasonable, and even in this setting, some relatives have had manifestations of von Hippel-Lindau syndrome. There is no compelling reason why such conditions as von Hippel-Lindau syndrome, peripheral neurofibromatosis, McCune-Albright syndrome, and others should be reclassified as MEN syndromes, although awareness of their collective endocrine abnormalities is clinically important.

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Primary Adrenocortical Nodular Dysplasia, a Distinct Subtype of Cushing’s Syndrome

Cited by 7 publications

References 0 publications

Acromegaly with Hyperprolactinemia Developed after Bilateral Adrenalectomy in a Patient with Cushing's Syndrome due to Adrenocortical Nodular Hyperplasia.

Acromegaly with Hyperprolactinemia Developed after Bilateral Adrenalectomy in a Patient with Cushing's Syndrome due to Adrenocortical Nodular Hyperplasia.

Complexo de Carney: relato de um caso e revisão da literatura

Multiple endocrine neoplasia: How many syndromes?

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