Metastatic primitive neuroectodermal tumour of the ovary: successful treatment with mega-dose chemotherapy followed by peripheral blood progenitor cell rescue.

Primitive neuroectodermal tumours (PNET) of the ovary are rare, aggressive tumours which are associated with high morbidity and mortality. Previously reported cases have shown limited response to therapy in patients presenting with metastatic disease and survival rates have been discouragingly low. We report the case of a 13-year-old girl who presented with a primary ovarian PNET and extensive metastatic disease. Pathologic studies confirmed the neural origin of the tumour and its morphologic appearance of neuroblastoma. Incomplete surgical resection was followed by treatment with aggressive multi-agent chemotherapy including cis-platinum, etoposide, cyclophosphamide, anddoxorubicin as per a neuroblastoma treatment protocol. Complete clinical remission ensued and she received consolidative therapy with myeloablative doses of thiotepa, melphalan, and carboplatin followed by autologous peripheral blood progenitor cell rescue. All therapy was well tolerated and the patient remains in complete remission with no evidence of disease 18 months from presentation. Mega-dose chemotherapy followed by progenitor cell rescue may provide optimal therapy for patients presenting with metastatic ovarian PNET.