Giant cell rich Osteosarcoma of the skull – Case report

Abstract

Giant cell rich osteosarcoma account for 3% of all osteosarcomas and often arise in the appendicular skeleton Giant cell rich Osteosarcoma of the skull is an extremely rare manifestation.
Case Presentation: Here we present a 15 year old girl with Giant cell rich osteosarcoma of the skull. Our patient presented with holocranial headache, diplopia and bilateral fundus atrophy. MRI brain revealed a heterogenous soft tissue density in sphenoid sinus with destruction of roof of sphenoid sinus and extending to posterior ethmoid sinus. Initial transnasal biopsy was suggestive of Giant cell tumor. Hence she underwent decompression of the sphenoclival mass. Subsequent histopathology confirmed Giant cell rich variant of Osteosarcoma. She received External beam Radiation followed by six cycles of chemotherapy with Cisplatin, Adriamycin and Ifosfamide. Currently she is asymptomatic and in clinical remission at 1 year of follow up.
A close differential diagnosis of this rare entity is Giant cell tumor of bone. Likewise in our patient the preoperative cytology resembled Giant cell tumor of bone. Hence she underwent upfront surgery. Post operative histopathology was in favour of Giant cell rich osteosarcoma. The standard approach to the treatment of Giant cell rich osteosarcoma is not yet well established. However the available literature suggests a multidisciplinary approach as that for high grade osteosarcoma. Nevertheless further studies are required to determine the appropriate management of this rare bone tumor.