Leydig cell tumors (LCTs) are rare cord-stromal tumors that may occur in testis or ovaries and may produce androgens or estrogens. The majority has been found in men between the ages of 20 and 60 years. Adults with androgen-secreting LCTs are usually asymptomatic; feminizing syndromes may result from the production of estradiol or the peripheral aromatization of testosterone. In children, LCTs usually present between 5 and 10 years of age with isosexual precocious pseudopuberty or gynecomastia. We report 2 cases of LCT in prepubertal boys presenting with advanced unilateral pubarche and testicular volume asymmetry. Both subjects had normal penis size for age; no axillary hair or other signs of puberty were present. Height velocity was normal, and bone age was coincident with chronological age. Androgen levels were normal, as well as estrogen, corticotropin, and cortisol concentration. Testicular ultrasound demonstrated a testicular mass. Histology examination revealed a well-differentiated LCT. This is the first report of 2 pediatric patients with LCT presenting with advanced pubarche in absence of systemic hyperandrogenism. We hypothesize that the neoplastic cells may locally produce high levels of androgens or androgen-like bioactivity molecules that are responsible for the clinical manifestation. We suggest that a testicular ultrasound should be obtained in all children presenting with unilateral pubarche, with or without hyperandrogenism.
Atypical leydig cell tumor in children : Report of 2 cases / C. Mameli, G. Selvaggio, C. Cerini, G. Bulfamante, C. Madia, G. Riccipetitoni, G.V. Zuccotti. - In: PEDIATRICS. - ISSN 0031-4005. - 138:5(2016 Nov).
|Titolo:||Atypical leydig cell tumor in children : Report of 2 cases|
MAMELI, CHIARA (Corresponding)
ZUCCOTTI, GIAN VINCENZO (Ultimo)
|Parole Chiave:||pediatrics; perinatology and child health|
|Settore Scientifico Disciplinare:||Settore MED/38 - Pediatria Generale e Specialistica|
|Data di pubblicazione:||nov-2016|
|Digital Object Identifier (DOI):||http://dx.doi.org/10.1542/peds.2016-0151|
|Appare nelle tipologie:||01 - Articolo su periodico|