Uncommon adrenal rest tumors and massive adrenal enlargement in adult with congenital adrenal hyperplasia mimicking metastasis from pleomorphic sarcoma.

Pierluigi Mazzeo, Irene Tizianel, Francesca Galuppini, Marta Sbaraglia, Mattia Barbot

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Article in BMC endocrine disorders, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

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5 authors.

Pierluigi MazzeoDepartment of Medicine DIMED, University of Padua, Padua, Italy.

Irene TizianelDepartment of Medicine DIMED, University of Padua, Padua, Italy.

Francesca GaluppiniDepartment of Medicine DIMED, University of Padua, Padua, Italy.

Marta SbaragliaDepartment of Medicine DIMED, University of Padua, Padua, Italy.

Mattia BarbotDepartment of Medicine DIMED, University of Padua, Padua, Italy. mattia.barbot@unipd.it.ORCID http://orcid.org/0000-0002-1081-5727

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6 · The paper itself

Abstract

backgroundCongenital adrenal hyperplasia (CAH) encompassed a bunch of autosomal recessive disorders characterized by impaired cortisol levels due to an enzymatic deficiency in steroid synthesis. In adult male patients with CAH, a frequent complication related to poor disease control is the development of ectopic adrenocortical tissue in the testes, named testicular adrenal rest tumors (TART). Conversely, ovarian adrenal rest tumors (OART) in females are extremely rare and adrenal rests in sites other than gonads are so uncommon to have been described only few times in literature. CASE PRESENTATION: We report a case of a male patient with untreated CAH and oncologic history of pleomorphic sarcoma who presented with massive bilateral adrenal enlargement and adrenal rest tumors in peri-lumbar and peri-cecal sites, which mimicked metastasis from sarcoma.

conclusionsThe development of massive adrenal enlargement and ectopic adrenal rest tumors in sites other than gonads, even if very uncommon, should be suspected in patients with CAH and prolonged periods of undertreatment.

Indexed as

Adrenal Hyperplasia, CongenitalAdrenal Rest TumorAdrenal Gland NeoplasmsAdultDiagnosis, DifferentialHumansMalePrognosisSarcoma21-hydroxylase deficiencyCase reportCongenital adrenal hyperplasiaEctopic adrenal rest tumorTesticular adrenal rest tumors

Identifiers

PMID38977992

DOI10.1186/s12902-024-01635-z

PMCPMC11229217

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