Ovarian steroid cell tumor causing isosexual pseudoprecocious puberty in a young girl: an instructive case and literature review

Abstract

Background Steroid cell tumors (SCTs) are very rare sex cord-stromal tumors and account only for less than 0.1% of ovarian neoplasms. SCTs might comprise diverse steroid-secreting cells; hence, the characteristic clinical features were affected by their propensity to secrete a variety of hormones rather than mass effect resulting in compression symptoms and signs. To date, ovarian SCTs have seldom been reported in children, particularly very young children; and pseudoprecocious puberty (PPP) as its unique principal manifestation should be reiterated. Case presentation We reported a 1-year-8-month-old girl presenting with rapid bilateral breast and pubic hair development within a 2-month period. Undetectable levels of LH and FSH along with excessively high estradiol after stimulation with gonadotropin-releasing hormone (GnRH), as well as a heterogeneous mass inside left ovary shown in pelvic sonography indicate isosexual PPP. Her gonadal hormones returned remarkably to the prepubertal range the day after surgery, and histology of the ovary mass demonstrated SCTs containing abundant luteinized stromal cells. Conclusion The case highlighted that SCTs causing isosexual PPP should be taken into consideration in any young children coexistent with rapidly progressive puberty given a remarkable secretion of sex hormones. This article also reviewed thoroughly relevant reported cases to enrich the clinical experience of SCTs in the pediatric group.

Hyperandrogenism due to ovarian steroid cell tumor in a pediatric patient

“…Existe también reporte de tumores que producen renina como el publicado por Lee et al cuya clínica principal fue la hipertensión arterial. Si bien un 25% puede ser no funcionante, sí puede estar asociado a distensión o dolor abdominal (4) . El uso de marcadores tumorales en pacientes pediátricos con tumores de ovario resulta beneficioso según el tipo de tumor, por ejemplo, AFP o el antígeno del cáncer 125 (CA125) en teratomas; sin embargo, no suelen ser de ayuda en los tumores de células esteroideas, donde el diagnóstico puede ser apoyado por la elevación de los esteroides sexuales (2,4,13) .…”

“…Debido a su baja frecuencia, inicialmente no fueron considerados en estudios de pacientes con hiperandrogenismo. Además, algunos casos se asocian a otras manifestaciones endocrinas como síndrome de Cushing o pubertad precoz, lo que dificulta aún más su diagnóstico (3)(4)(5) .…”

Ovarian steroid cell tumors: what do we know so far?

“…In additional to symptoms related to androgen excess, there are age group-specific presentations. For example, in pediatric population, children may show isosexual precocious puberty ( 22 ). In child-bearing age group, women present with irregular menstrual cycles or infertility ( 23 ).…”