Childhood adrenocortical carcinoma: Case report and review

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Abstract

Adrenocortical cancers in childhood are very rare tumors. The tumors have varied presentation — either virilizing forms or presentation with Cushing's syndrome, or both. In children, due to the rapid development of symptoms they come to attention early, however, if not diagnosed and treated early can have a downhill course. The last decade has seen the emergence of new diagnostic imaging modalities. There is also intense ongoing research in newer treatment modalities as these tumors can be unresectable or have a high recurrence rate.

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Most cited references 17

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Clinical practice. The incidentally discovered adrenal mass.

Jock W. Young (2007)

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Mutations of beta-catenin in adrenocortical tumors: activation of the Wnt signaling pathway is a frequent event in both benign and malignant adrenocortical tumors.

Eric Jullian, Marie-Cécile Vacher-Lavenu, Xavier Bertagna … (2005)

Adrenocortical cancer is a rare cancer with a very poor prognosis. The genetic alterations identified to date in adrenocortical tumors are limited. Activating mutations of the Wnt signaling pathway have been observed in more frequent cancers, particularly digestive tract tumors. We investigated whether Wnt pathway activation is involved in adrenocortical tumorigenesis. In a series of 39 adrenocortical tumors, immunohistochemistry revealed abnormal cytoplasmic and/or nuclear accumulation of beta-catenin in 10 of 26 adrenocortical adenomas and in 11 of 13 adrenocortical carcinomas. An activating somatic mutation of the beta-catenin gene was shown in 7 of 26 adrenocortical adenomas and in 4 of 13 adrenocortical carcinomas; these mutations were observed only in adrenocortical tumors with abnormal beta-catenin accumulation and most were point mutations altering the Ser45 of exon 3 (in the consensus GSK3-beta/CK1 phosphorylation site). Functional studies showed that the activating Ser45 beta-catenin mutation found in the adrenocortical cancer H295R cell line leads to constitutive activation of T-cell factor-dependent transcription. This is the first molecular defect to be reported with the same prevalence in both benign (27%) and malignant (31%) adrenocortical tumors. beta-Catenin mutations are also the most frequent genetic defect currently known in adrenocortical adenomas. In adrenocortical adenomas, beta-catenin alterations are more frequent in nonfunctioning tumors, suggesting that beta-catenin pathway activation might be mostly involved in the development of nonsecreting adrenocortical adenomas and adrenocortical carcinomas. The very frequent and substantial accumulation of beta-catenin in adrenocortical carcinomas suggests that other alterations might also be involved. This finding may contribute to new therapeutic approaches targeting the Wnt pathway in malignant adrenocortical tumors, for which limited medical therapy is available.

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Clinical and outcome characteristics of children with adrenocortical tumors: a report from the International Pediatric Adrenocortical Tumor Registry.

R. F. Ribeiro, Ruth Galindo, Manoel Filho … (2004)

We created a registry for pediatric adrenocortical tumors (ACTs), which are rare and are not well characterized. We provide a descriptive analysis of 254 patients registered on the International Pediatric Adrenocortical Tumor Registry. Between January 1990 and December 2001, 254 patients younger than 20 years of age with newly diagnosed or previously treated ACTs were registered. A histologic diagnosis of ACT was required, although central review was not mandatory. Follow-up information was periodically requested from the referring physician. Treatment was chosen by the primary physician. The overall female-male ratio was 1.6:1, but it varied widely among age groups. The most common presenting sign (84.2%) was virilization. Cushing's syndrome without virilization was uncommon (5.5%). Tumors were completely resected in 83% of patients. Patients with disseminated or residual disease received mitotane, cisplatin, etoposide, and/or doxorubicin, and rarely, radiation therapy. At a median follow-up of 2 years and 5 months, 157 patients (61.8%) survived without evidence of disease and 97 patients (38.2%) had died. The 5-year event-free survival estimate was 54.2% (95% CI, 48.2% to 60.2%). In a multivariate analysis, disease stage, presenting signs of endocrine dysfunction, and age were independently associated with prognosis. Childhood ACTs occur predominantly in females and almost always causes clinical signs. Complete resection is required for cure. Residual or metastatic disease carries a poor prognosis. Our results demonstrate the feasibility of a disease-specific database for obtaining meaningful clinical and outcome information.

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Author and article information

Journal

Journal ID (nlm-ta): Indian J Endocrinol Metab

Journal ID (iso-abbrev): Indian J Endocrinol Metab

Journal ID (publisher-id): IJEM

Title: Indian Journal of Endocrinology and Metabolism

Publisher: Medknow Publications & Media Pvt Ltd (India )

ISSN (Print): 2230-8210

ISSN (Electronic): 2230-9500

Publication date (Print): May-Jun 2012

Volume: 16

Issue: 3

Pages: 431-435

Affiliations

[1] Department of Endocrinology, Army Hospital (Research and Referral), Delhi Cantonment, India

[1 ] Department of Nuclear Medicine, Army Hospital (Research and Referral), Delhi Cantonment, India

[2 ] Department of Pathology, Army Hospital (Research and Referral), Delhi Cantonment, India

Author notes

Corresponding Author: Col M. K. Garg, Department of Endocrinology, Army Hospital (Research and Referral), Delhi Cantonment, India. E-mail: mkgargs@ 123456gmail.com

Article

Publisher ID: IJEM-16-431

DOI: 10.4103/2230-8210.95699

PMC ID: 3354855

PubMed ID: 22629514

SO-VID: 1140e347-9c8c-4da9-8041-c012651c37d9

License:

This is an open-access article distributed under the terms of the Creative Commons Attribution-Noncommercial-Share Alike 3.0 Unported, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Childhood adrenocortical carcinoma: Case report and review

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Bioscientifica Open Endocrinology

Most cited references 17

Clinical practice. The incidentally discovered adrenal mass.

Mutations of beta-catenin in adrenocortical tumors: activation of the Wnt signaling pathway is a frequent event in both benign and malignant adrenocortical tumors.

Clinical and outcome characteristics of children with adrenocortical tumors: a report from the International Pediatric Adrenocortical Tumor Registry.

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