Background: Osteogenic sarcoma rarely occurs in soft tissues and generally affects individuals beyond the second decade of life.
Methods: The authors report a rare case of an extra osseous osteogenic sarcoma arising in the retroperitoneum of an adolescent, review the literature, and outline the diagnostic and therapeutic dilemmas. The role of adjuvant chemotherapy, using drugs used in managing bony osteosarcomas, is discussed.
Conclusions: Retroperitoneal sarcomas may simulate ovarian teratomas. Careful consideration of the differential diagnosis of large cystic abdominal masses in adolescent females when size precludes adequate assessment of tumor mobility and imaging fails to demonstrate the ovaries is essential if these rare tumors are to be managed effectively.