Retiform pattern of Sertoli–Leydig cell tumor of the ovary in a 4‐year‐old girl

Abstract

Sertoli-Leydig cell tumors are rare sex cord-stromal neoplasms that account for <0.2% of ovarian tumors. These tumors with a retiform pattern pose difficult diagnostic problems, with the majority being misinterpreted as serous papillary cystadenocarcinoma and endodermal sinus tumor. Retiform pattern Sertoli-Leydig cell tumor seen mostly in young patients is clinically malignant and has a worse prognosis, underlining the importance of recognition of this histopathologic entity. In the literature only two such cases have been described in patients under the age of 5 years; reported herein is the third case. Described here is a rare case of ovarian Sertoli-Leydig cell tumor with retiform pattern in a 4-year-old child without any endocrine manifestations.

Sex cord stromal tumors of the ovary in children: A clinicopathological report from the Italian TREP project

“…As previously reported 16, 29, 30, the aggressiveness of SLCTs is higher in tumors with retiform pattern and/or heterologous elements. These features suggest a possible malignant behavior and the necessity of a more aggressive treatment.…”

Recurrent Sertoli–Leydig cell tumor of ovary in 8-year-old girl

“…In cases with advanced stage or metastasis, cytoreductive surgery and postoperative chemotherapy or irradiation should be performed. 1,2,4 To conclude, the retiform variant is a special subtype of SLCTs. Because of their young age, the uncertain malignant potential, rare bilaterality and uncertain response to chemotherapy, patients should be treated conservatively whenever possible.…”

“…4 These tumors with a retiform pattern are mostly seen in younger patients in their first or second decade, the average age being 15 years as compared to 25 years for SLCTs as a group. 1,3,5 Androgenic manifestations are seen in only 25% of the retiform variant patients. 3 Most of the patients present unilateral huge abdominal mass and pain such described in our case.…”

“…In our case, fertility sparing unilateral salpingo-oopherectomy was performed with reference to Lou and Kawatran's case reports which describe 4 and 9 years old similar patients. 1,4 In the paper by Talerman, eight of nine SLCT cases (88.9%) were in stage IA and were treated by unilateral salpingo-oophorectomy. If the tumor confined to the ovary with no involvement of the contralateral ovary and abdominal metastases, unilateral salpingo-oophorectomy is the treatment of choice.…”

Retiform Sertoli-Leydig Cell Tumor in a 38-Year-Old Woman: A Case Report, Retrospective Review, and Review of Current Literature

“…Additionally, poorly differentiated tumors, tumors with heterologous mesenchymal elements, and tumor rupture have been associated with adverse outcomes [1, 3, 19]. The presence of a retiform growth pattern is thought to confer slightly worse prognosis in cases of moderately differentiated SLCT; however, the evidence is inconclusive [1, 7, 19, 20]. For patients with well-differentiated to moderately differentiated SLCT confined to the ovary and without mesenchymal heterologous elements, unilateral salpingo-oophorectomy is the surgical management of choice.…”