Clinical, Hormonal and Pathological Findings in a Comparative Study of Adrenocortical Neoplasms in Childhood and Adulthood

Mendonca, Berenice B.; Lucon, Antônio Marmo; Menezes, C A V; Saldanha, Luís Balthazar; Latrônico, Ana Claudia; Zerbini, Maria Cláudia Nogueira; Madureira, Guiomar; Domenice, Sorahia; Albergaria, Maria Adelaide P.; Camargo, Marcia H. A.; Halpern, Alfredo; Liberman, Bernardo; Arnhold, Ivo Jorge Prado; Bloise, W; Andriolo, Adagmar; Nicolau, Wilian; Silva, Frederico A.Q.; Wroclaski, Eric; Arap, Sami; Wajchenberg, B. L.

doi:10.1016/s0022-5347(01)66673-4

Cited by 101 publications

(69 citation statements)

References 14 publications

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“…Adrenal adenomas showing a pattern of enzymatic block related to impairment of 21-hydroxylase or 11b-hydroxylase have been suggested to be malignant and it could be used to follow up recurrence [9]. We thought that the elevation of 17aOHP levels suggesting evidence of impairment of 21-hydroxylase enzyme activity was related to ACC in our case.…”

Section: Discussionmentioning

confidence: 71%

“…It was reported as 13.9 yr (6-30 yr) by Sert et al and 6.1 yr by Goswami et al in their series [2,3]. Adrenocortical carcinomas may be non-functional and may be discovered incidentally or it may be diagnosed while investigating the symptoms and signs of different endocrine syndromes or suspicious adrenal masses [9,10]. Tumors have a tendency to multiple hormone secretion.…”

Section: Discussionmentioning

confidence: 97%

“…Our case was determined at stage 4, because tumor diameter was >5 cm and had distant organ metastases [5,10]. The average survival in the cases of ACC is generally 18 months [5,9]. The essential treatment of ACC is excision of mass even in the presence of distant organ metastases, after which treatment with mitotane is recommended [5].…”

Section: Discussionmentioning

confidence: 99%

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A Case of Postpartum Hypopituitarism Accompanied by Cushing's Syndrome as a Result of an Adrenocortical Carcinoma

Şencan

Dökmetaş

2005

Endocr J

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Abstract. Sheehan's syndrome frequently causes hypopituitarism either immediately or after a delay of several years, depending on the degrees of postpartum ischemic pituitary necrosis. A 55 year-old woman whose last child was born 27 yr ago with massive hemorrhage was diagnosed as postpartum hypopituitarism. She had deficiency of growth hormone, prolactin, gonadotropins and thyrotropin. However, she interestingly had apparent hypercortisolism without suppression response to the dexamethasone tests. We found an adrenal mass with distant metastases to the liver and lung while investigating the origin of the hypercortisolism. Hyperandrogenism and very high levels of 17a hydroxyprogesterone were present. Accordingly, the patient was diagnosed as hypopituitarism due to Sheehan's syndrome accompanied by Cushing's syndrome as a result of an adrenocortical carcinoma.

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Section: Discussionmentioning

confidence: 71%

Section: Discussionmentioning

confidence: 97%

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A Case of Postpartum Hypopituitarism Accompanied by Cushing's Syndrome as a Result of an Adrenocortical Carcinoma

Şencan

Dökmetaş

2005

Endocr J

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“…No entanto, essa classificação tem sido mais eficaz para a predição de pior prognóstico somente em TAC em adultos. Em crianças, o estadiamento tumoral é mais confiável para a predição de prognóstico (16).…”

Section: Descritoresunclassified

Tumores adrenocorticais na criança: da abordagem clínica à avaliação molecular

Antonini

Colli²,

Ferro

et al. 2011

Arq Bras Endocrinol Metab

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sumárioTumores do córtex adrenal (TCA) são mais frequentes em crianças, mas podem ocorrer em qualquer faixa etária. São classificados como funcionantes, não funcionantes (predominam no adulto), e mistos. O diagnóstico é baseado na avaliação clínica, hormonal e exames de imagem. Em crianças, o método de escolha para diferenciar entre benigno ou maligno é a classificação baseada no estadiamento do tumor. Alguns marcadores moleculares merecem destaque: além de mutações inativadoras no gene supressor tumoral TP53, há evidências de envolvimento do IGF2 em 90% de TAC malignos, e mutações no éxon 3 do gene CTNNB1 foram encontradas em 6% dos TAC pediátricos. Além disso, microRNAs podem atuar como reguladores negativos da expressão gênica e participar da tumorigênese adrenocortical. Métodos para análise da expressão gênica permitem identificar TCA com prognóstico bom ou ruim, e espera-se que esses estudos possam facilitar o desenvolvimento de drogas para tratar pacientes de acordo com as vias de sinalização específicas que estiverem alteradas. Arq Bras Endocrinol Metab. 2011;55(8):599-606 Descritores Tumor do córtex adrenal; doenças da adrenal; fatores de crescimento; proteína supressora de tumor P53; biologia molecular summArY Adrenocortical tumors (ACT) are more frequent during childhood, but they can appear at any age. ACTs can be classified in functioning, nonfunctioning (mainly observed in adults) and mixed. The diagnosis is based on clinical, biochemical findings and imaging. In children, in order to classify ACT as benign or malignant, tumor staging classification is recommended. Regarding molecular markers some studies should be taken into account: besides TP53 mutations, previous studies have also provided evidences of IGF2 involvement in 90% of the malignant ACT. Mutations altering exon 3 of CTNNB1 gene have been found in 6% of childhood ACTs. In addition, microRNAs can act as negative regulators of gene expression by targeting mRNA controlling cell growth, differentiation and apoptosis and have been implicated in adrenal tumorigenesis. High-throughput methods to analyze genome-wide expression have been developed over the last decade and identified a subset of tumors with good or poor prognosis. In the future, these studies can provide the basis of specific drug development, which can treat patients according to specific altered signaling pathway. Arq Bras Endocrinol Metab. 2011;55(8):599-606 Keywords Adrenal cortex tumor; adrenal gland diseases; molecular biology growth factors; tumor suppressor protein P53 iNTroDuÇÃo O s tumores adrenocorticais (TAC) apresentam incidência bimodal, sendo mais comuns nos primeiros cinco anos de vida e entre a 4ª e 5ª décadas de vida. Em todas as faixas etárias esses tumores são mais frequentes no sexo feminino (1,2). Sua incidência varia de acordo com a região geográfica, sendo dez a quinze vezes mais elevada no Sul e Sudeste do Brasil, onde ocorrem apro-

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“…A valiosa experiência nacional neste campo tem gerado diversos estudos clínicos e bási-cos na última década. Revisões extensas e dados originais foram publicados (1)(2)(3)(4)(5). Destacamos a recente descrição de uma mutação germinativa no gene supressor tumoral p53 em 87% das crianças e 13% dos adultos brasileiros com tumores adrenais, indicando maior suceptibilidade genética para o desenvolvimento tumoral do córtex adrenal na população brasileira (6,7).…”

Section: Perspectivas Do Manuseio E Tratamento Dos Tumores Adrenais Nunclassified

Tumores adrenocorticais: novas perspectivas

Latrônico

Mendonça

2004

Arq Bras Endocrinol Metab

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RESUMOUma freqüência elevada de tumores adrenocorticais tem sido observada em crianças e adultos brasileiros das regiões sul e sudeste. A valiosa experiência nacional no manuseio destes tumores tem gerado diversas e relevantes publicações de conteúdo básico e clínico. Entretanto, a criação de um registro nacional dos tumores adrenocorticais, a uniformização de condutas e o desenvolvimento de estudos colaborativos são objetivos ainda a serem alcançados. Nesta revisão apresentamos de forma resumida os pontos principais discutidos em dois eventos científicos sobre tumores adrenais: o consenso internacional "International Consensus Conference ABSTRACT Adrenocortical Tumors -New Perspectives.A high frequency of adrenocortical tumors has been observed in Brazilian children and adults from South and Southwestern regions. The valuable national experience in the management of these tumors have resulted in several and relevant basic and clinical reports. However, the creation of an adrenocortical tumor national registry, the uniformity of approaches and collaborative studies are target to pursue. In this review article, we briefly described the fundamental points which were discussed in two scientific events on adrenocortical tumors: "International Consensus Conference on Treatment of Adrenal Cancer" and "I Simpó -sio de Diagnóstico e Tratamento dos Tumores Adrenocorticais". The task force involving several Brazilian centers will increase the progress in the diagnosis, prognosis and treatment of this devastating disorder.

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Clinical, Hormonal and Pathological Findings in a Comparative Study of Adrenocortical Neoplasms in Childhood and Adulthood

Cited by 101 publications

References 14 publications

A Case of Postpartum Hypopituitarism Accompanied by Cushing's Syndrome as a Result of an Adrenocortical Carcinoma

A Case of Postpartum Hypopituitarism Accompanied by Cushing's Syndrome as a Result of an Adrenocortical Carcinoma

Tumores adrenocorticais na criança: da abordagem clínica à avaliação molecular

Tumores adrenocorticais: novas perspectivas

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