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Case Reports
. 2022 Nov 7;36(1):96-100.
doi: 10.1515/jpem-2022-0384. Print 2023 Jan 27.

Human chorionic gonadotrophin secreting adrenocortical neoplasm presenting with peripheral precocious puberty in an infant

Fatma Dursun  1 , Zekeriya İlçe  2 , İlkay Tosun  3 , Pınar Atla  1 , Sevinç Kalın  4 , Fatma Tuğba Güvenç  2 , Ülkü Miray Yıldırım  5 , Heves Kırmızıbekmez  1
Affiliations
Case Reports

Human chorionic gonadotrophin secreting adrenocortical neoplasm presenting with peripheral precocious puberty in an infant

Fatma Dursun et al. J Pediatr Endocrinol Metab. .

Abstract

Adrenocortical tumor (ACT) is a rare malignant tumor which usually present with Cushing syndrome and virilization. Paraneoplastic syndromes (PNS) due to neoplasms can occur with peptides or cytokines secreted by the tumor. Here, we report a 13-month-old-male presented with severe masculinization. He had signs of precocious puberty with enlarged testicles, very high testosterone levels but low levels of gonadotrophins, and elevated β-hCG. He underwent a left nephrectomy. The histopathological evaluation revealed a diagnosis of adrenocortical neoplasm. The levels of androgens and β-hCG normalized after the resection of tumor, and the clinical findings improved within few months. We report the first pediatric patient with peripheral precocious puberty due to an ACT that secretes β-hCG as PNS. A β-hCG secreting ACT can cause severe virilization due to increased gonadal androgens stimulated by β-hCG as well as due to increased adrenal androgens from the tumor.

Keywords: adrenocortical neoplasm; peripheral precocious puberty; β-hCG.

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