Poorly differentiated ovarian Sertoli-Leydig cell tumor with heterologous rhabdomyosarcomatous elements associated with elevated serum alpha-fetoprotein level: a case report and review of the literature

Ovarian Sertoli-Leydig cell tumors (SLCTs) are uncommon sex cord–stromal tumors. Moreover, SLCTs with heterologous mesenchymal elements are extremely rare and usually associated with poor differentiation and prognosis. Herein, the authors describe a case of SLCT involving the right ovary in a 16-year-old girl who presented with acute lower abdominal pain and fever. Serum investigation demonstrated abnormally elevated level of alpha-fetoprotein (AFP), slightly elevation of testosterone, and CA125 concentration. Right salpingo-oopherectomy was performed due to ovarian tumor torsion and then histopathological analysis revealed a poorly differentiated Sertoli-Leydig cell tumor with heterologous rhabdomyosarcomatous elements. A brief review of the literature was conducted to explore the management options for patients with SLCTs and the prognosis.

Ovary; Sertoli-Leydig cell tumors; Sex cord-stromal tumors; Rhabdomyosarcoma; Alpha fetoprotein.

[1] Gui T., Cao D., Shen K., Yang J., Zhang Y., Yu Q., et al.: “A clinicopathological analysis of 40 cases of ovarian Sertoli-Leydig cell tumors”. Gynecol. Oncol., 2012, 127, 384.

[2] Young R.H., Scully R.E.: “Ovarian Sertoli-Leydig cell tumors. A clinicopathological analysis of 207 cases”. Am. J. Surg. Pathol.,1985, 9, 543.

[3] Watanabe T., Yamada H., Morimura Y., Abe M., Motoyama T., Sato A.: “Ovarian Sertoli-Leydig cell tumor with heterologous gastrointestinal epithelium as a source of alpha-fetoprotein: a case report”. J. Obstet. Gynaecol. Res., 2008, 34, 418.

[4] Schultz K.A., Schneider D.T., Pashankar F., Ross J., Frazier L.: “Management of ovarian and testicular sex cord-stromal tumors in children and adolescents”. J. Pediatr. Hematol. Oncol., 2012, 34, S55.

[5] Takeda A., Watanabe K., Hayashi S., Imoto S., Nakamura H.: “Gynandroblastoma with a Juvenile Granulosa Cell Component in an Adolescent: Case Report and Literature Review”. J. Pediatr. Adolesc. Gynecol., 2017, 30, 251.

[6] Oliva E, Carcangiu M, Carinelli S, Ip P, Loening T, Longacre T, et al.:”Tumours of the uterine corpus: mesenchymal tumours”. WHO classification of tumours of female reproductive organs. 4 th ed.,2014.

[7] Young R.H.: “Sertoli-Leydig cell tumors of the ovary: review with emphasis on historical aspects and unusual variants”. Int. J. Gynecol. Pathol.,1993, 12, 141.

[8] Goldblum J.R.: “An approach to pleomorphic sarcomas: can we subclassify, and does it matter?”. Mod. Pathol.,2014, 27, S39.

[9] Cessna M.H., Zhou H., Perkins S.L., Tripp S.R., Layfield L., Daines C., et al.: “Are myogenin and myoD1 expression specific for rhabdomyosarcoma? A study of 150 cases, with emphasis on spindle cell mimics”. Am. J. Surg. Pathol., 2001, 25, 1150.

[10] Talerman A., Roth L.M.: “Recent advances in the pathology and classification of gonadal neoplasms composed of germ cells and sex cord derivatives”. Int. J. Gynecol. Pathol., 2007, 26, 313.

[11] Friedman C., Fenster T.: “Laparoscopic Treatment of Mixed Malignant Ovarian Germ Cell Tumor in a 16-Year-Old Female Adolescent”. J. Pediatr. Adolesc. Gynecol., 2016, 29, e91.

[12] de la Motte Rouge T., Pautier P., Genestie C., Rey A., Gouy S., Leary A., et al.:”Prognostic significance of an early decline in serum alphafetoprotein during chemotherapy for ovarian yolk sac tumors”. Gynecol. Oncol., 2016, 142, 452.

[13] Motoyama I., Watanabe H., Gotoh A., Takeuchi S., Tanabe N., Nashimoto I.: “Ovarian Sertoli-Leydig cell tumor with elevated serum alpha-fetoprotein”. Cancer, 1989, 63, 2047.

[14] Horta M., Cunha T.M., Marques R.C., Felix A.: “Ovarian SertoliLeydig cell tumor with heterologous elements of gastrointestinal type associated with elevated serum alpha-fetoprotein level: an unusual case and literature review”. J. Radiol. Case Rep., 2014, 8, 30.

[15] Shu H., Yang X., Gao A.F.: “Ovarian Sertoli-Leydig cell tumor in a 9-month-old infant with special histologic pattern”. Fetal Pediatr. Pathol., 2012, 31, 388.

[16] Plastini T., Staddon A.: “Sertoli-Leydig Cell Tumor with Concurrent Rhabdomyosarcoma: Three Case Reports and a Review of the Literature”. Case Rep. Med., 2017, 2017, 4587296.

[17] Zaloudek C., Norris H.J.: “Sertoli-Leydig tumors of the ovary. A clinicopathologic study of 64 intermediate and poorly differentiated neoplasms”. Am. J. Surg. Pathol.,1984 ,8, 405.

[18] Burnik Papler T., Frkovic Grazio S., Kobal B.: “Sertoli - Leydig cell tumor with retiform areas and overgrowth of rhabdomyosarcomatous elements: case report and literature review”. J. Ovarian Res., 2016, 9, 46.

[19] Guerard M.J., Ferenczy A., Arguelles M.A.:”Ovarian Sertoli-Leydig cell tumor with rhabdomyosarcoma: an ultrastructural study”. Ultrastruct Pathol.,1982, 3, 347.

[20] Grove A., Vestergaard V.: “Ovarian Sertoli-Leydig cell tumor of intermediate grade with heterologous elements of rhabdomyosarcoma. A case report and a review of the literature”. Ann. Diagn. Pathol., 2006, 10, 288.

[21] Kostopoulou E., Talerman A.: “Ovarian Sertoli-Leydig cell tumor of intermediate differentiation with immature skeletal muscle heterologous elements”. Acta Obstet. Gynecol. Scand., 2003, 82, 197.

[22] Prat J., Young R.H., Scully R.E. “Ovarian Sertoli-Leydig cell tumors with heterologous elements. II. Cartilage and skeletal muscle: a clinicopathologic analysis of twelve cases”. Cancer, 1982, 50, 2465.

[23] Sigismondi C., Gadducci A., Lorusso D., Candiani M., Breda E., Raspagliesi F., et al.: “Ovarian Sertoli-Leydig cell tumors. a retrospective MITO study”. Gynecol. Oncol., 2012, 125, 673.

[24] Pautier P., Gutierrez-Bonnaire M., Rey A., Sillet-Bach I., Chevreau C., Kerbrat P., et al.: “Combination of bleomycin, etoposide, and cisplatin for the treatment of advanced ovarian granulosa cell tumors”. Int. J. Gynecol. Cancer, 2008, 18, 446.

[25] Gershenson D.M., Morris M., Burke T.W., Levenback C., Matthews C.M., Wharton J.T.: “Treatment of poor-prognosis sex cord-stromal tumors of the ovary with the combination of bleomycin, etoposide, and cisplatin”. Obstet. Gynecol.,1996, 87, 527.