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.

Inhabitual presentation of Sertoli-Leydig cell tumor of the ovary with xeroderma pigmentosum: Case report with review of literature

“…On macroscopic examination, the tumor is solid and cystic, with a size ranging between sixand 30 cm in diameter and usually exceeding 15 cm. Hemorrhage and necrosis are frequently observed [ 2 , [4] , [5] , [6] , [7] , [8] , [9] , [10] ]. Microscopically, rhabdomyomatous differentiation can be isolated or in association with other heterologous elements, most often cartilage and intestinal glands [ 5 ].…”

“…The spindle cell type, pleomorphic and embryonalrhabdomyosarcoma have been reported. The SLTs component can be of intermediate differentiation, poorly differentiated, or retiform [ 2 , [4] , [5] , [6] , [7] , [8] , [9] , [10] ]. In some cases, the primary neoplasm did not contain heterologous elements, which were identified only in recurrent tumors [ 4 , 10 ].…”

“…Most reported cases are at a localized or locally advanced stage in the diagnosis time; however, recurrence with peritoneal involvement is frequent [ 2 , [4] , [5] , [6] , [7] , [8] , [9] , [10] ]. The recurrent tumor can be confined to the pelvis, involving the contralateral, or generalized within the abdomen.…”

“…Heterologous components may be present, most commonly in the intermediate differentiation and poorly differentiated groups [ 1 ]. Because of their scarcity, SLCTs with heterologous differentiation represent a challenge in both the diagnosis and the management, with limited available experience [ 2 ].…”

Pediatric ovarian Sertoli‐Leydig cell tumors with heterologous rhabdomyosarcoma elements: Clinical case series and review of the literature

“…and only 20% of SLCTs have heterologous elements. Several cases have been published in the adult literature describing cases of patients with ovarian SLCT with RMS components 7,8,[12][13][14][15]. Given its dual histologic features, diagnosis and management of these rare tumors is complex and challenging.…”