Ovarian germ cell tumors with rhabdomyosarcomatous components and later development of growing teratoma syndrome: a case report

Abstract

Introduction: Development of a sarcomatous component in a germ cell tumor is an uncommon phenomenon. Most cases reported have a grim prognosis. Growing teratoma syndrome is also an uncommon phenomenon and occurs in approximately 2% to 7% of non seminomatous germ cell tumors and should be treated surgically.

Case presentation: We report the case of a 12-year-old Asian girl with an ovarian mixed germ cell tumor containing a rhabdomyosarcomatous component. She was treated with a germ cell tumor chemotherapy regimen and rhabdomyosarcoma-specific chemotherapy. Towards the end of her treatment, she developed a retroperitoneal mass that was increasing in size. It was completely resected, revealing a mature teratoma, consistent with growing teratoma syndrome. She is still in complete remission approximately three years after presentation.

Conclusion: The presence of rhabdomyosarcoma in a germ cell tumor should be treated by a combined chemotherapy regimen (for germ cell tumor and rhabdomyosarcoma). In addition, development of a mass during or after therapy with normal serum markers should raise the possibility of growing teratoma syndrome that should be treated surgically.

Figure 1

Computerized Tomography findings before and during the course of treatment. (A1) showing huge heterogeneous mass filling the upper pelviabdominal cavity (arrows), (A2) showing unremarkable pelvis after chemotherapy, (B) showing the appearance of a new mass situated between right liver lobe, right kidney, and adrenal gland (arrows).

Figure 2

Histological features of the primary ovarian mass and the recurrent new mass. (A) low power magnification of the mature teratoma component, in which mature glands and stroma are seen (H&E X4), (B): higher power magnification of rhabdomyoblasts seen with abundant acidophilic cytoplasm and eccentric nuclei (H&E X 40) (inset show desmin positive staining of the rhabdomyoblasts), (C): mature glandular tissue is seen in the recurrent new mass, no immature tissue was identified (H&E X 40).