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(Reference retrieved automatically from Web of Science through information on FAPESP grant and its corresponding number as mentioned in the publication by the authors.)

Giant adrenal myelolipoma associated with 21-hydroxylase deficiency: unusual association mimicking an androgen-secreting adrenocortical carcinoma

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Author(s):
Mermejo, Livia Mara [1] ; Elias Junior, Jorge [2] ; Saggioro, Fabiano Pinto [3] ; Tucci Junior, Silvio [4] ; de Castro, Margaret [1] ; Moreira, Ayrton Custodio [1] ; Lamparelli Elias, Paula C. [1]
Total Authors: 7
Affiliation:
[1] FMRP USP, Div Endocrinol, Dept Clin Med, BR-14048900 Ribeirao Preto, SP - Brazil
[2] FMRP USP, Div Radiol, Dept Clin Med, BR-14048900 Ribeirao Preto, SP - Brazil
[3] FMRP USP, Dept Patol, BR-14048900 Ribeirao Preto, SP - Brazil
[4] FMRP USP, Dept Cirurgia & Anat, BR-14048900 Ribeirao Preto, SP - Brazil
Total Affiliations: 4
Document type: Journal article
Source: Arquivos Brasileiros de Endocrinologia e Metabologia; v. 54, n. 4, p. 419-424, JUN 2010.
Web of Science Citations: 16
Abstract

The objective of this study was to describe a case of giant myelolipoma associated with undiagnosed congenital adrenal hyperplasia (CAH) due to 21-hydroxylase (21OH) deficiency. Five seven year-old male patient referred with abdominal ultrasound revealing a left adrenal mass. Biochemical investigation revealed hyperandrogenism and imaging exams characterized a large heterogeneous left adrenal mass with interweaving free fat tissue, compatible with the diagnosis of myelolipoma, and a 1.5 cm nodule in the right adrenal gland. Biochemical correlation has brought concerns about differential diagnosis with adrenocortical carcinoma, and surgical excision of the left adrenal mass was indicated. Anatomopathologic findings revealed a myelolipoma and multinodular hyperplasic adrenocortex. Further investigation resulted in the diagnosis of CAH due to 21OH deficiency. Concluded that CAH has been shown to be associated with adrenocortical tumors. Although rare, myelolipoma associated with CAH should be included in the differential diagnosis of adrenal gland masses. Moreover, CAH should always be ruled out in incidentally detected adrenal masses to avoid unnecessary surgical procedures. (AU)

FAPESP's process: 08/09276-0 - Gene expression in adrenal adenoma and carcinoma
Grantee:Lívia Mara Mermejo
Support Opportunities: Scholarships in Brazil - Doctorate (Direct)