Poorly differentiated, ovarian Sertoli-Leydig cell tumor with heterologous rhabdomyosarcoma and glandular elements: Diagnosis and management of a rare neoplasm

Abstract

Ovarian Sertoli-Leydig cell tumors (SLCT) represent <2% of primary ovarian tumors, which range from benign to malignant; majority of the latter are low-grade. We present the case of a 12-year-old pre-pubertal female with poorly differentiated SLCT and heterologous rhabdomyosarcoma (FIGO stage-IA). She presented with worsening abdominal pain, pelvic mass, and elevated pre-operative serum Alpha-fetoprotein (AFP, 77.1 ng/mL). She underwent right salpingo-oophorectomy, omentectomy, and lymphadenectomy. The high-risk histology warranted 4-cycles of adjuvant BEP chemotherapy. There was no clinical evidence of recurrence at the 20-month follow-up. High-grade sex cord-stromal tumors are rare, present with low-stage disease, and have good progression-free survival following chemotherapy. Rarity of these tumors poses challenges in their diagnosis and treatment. Review of literature suggests that the presented case is youngest patient with dedifferentiated SLCT.

Keywords: AFP; Literature review; Ovarian cyst; Rhabdomyosarcoma; Sertoli-Leydig cell tumor.

Fig. 1

Computed tomography (CT) scan abdominopelvic region showing heterogeneous hemorrhagic cystic and solid mass.

Fig. 2

(A) Tubules of well-differentiated Sertoli cell tumor (weak Inhibin) and single cells of Leydig tumor (dark Inhibin). (B) Spindled areas of poorly differentiated Sertoli cell tumor and heterologous glandular elements. (C) High-grade spindled rhabdomyoblastic cells, positive for nuclear myogenin expression.

Fig. 3

(A) Follow-up of Alpha-fetoprotein (AFP) levels from day-zero (post-operative) to the most recent serologic follow-up. Not shown is pre-operative (at diagnosis) AFP level of 77.1 ng/ml; (B) AFP is secreted by Leydig cells (brown) in this tumor. (For interpretation of the references to colour in this figure legend, the reader is referred to the web version of this article.)