두개골에 발생한 골육종-섬유형성이상의 악성전환 1예보고-

초록

Osteosarcomas are malignant bone tumors, which commonly affect the long bones of young adults. Primary osteosarcomas of the skull are exceedingly rare. Osteosarcomatous transformation in fibrous dysplasia is unusual, too. In the review of the literature, among the malignant transformation in fibrous dysplasia, osteosarcoma was the most common type of tumor. The next most common were fibrosarcoma and chondrosarcoma. The malignant transformation usually developed in the third or fourth decade of life and the most frequent anatomic site were the craniofacial bones, the femur, and the tibia. About 25% of patients had the previous history of treated local radiation. We experienced and case of osteosarcoma of the skull showing the pathologic findings of malignant tranformation in the fibrous dysplasia. The patient was 28 year old man suffered from a headache. He intermittently complained of headache which aggravated 1 month ago. Neurologic examination was unremarkable except for the mild hypoesthesisa in right V2 and V3. Neither past history of rediation nor polyostotic bony lesion was present. Brain MRI revealed a solid mass with multifratemporal fossa. It showed iso-intensity on T1WI and T2WI with good enhancement. Craniectomy and excision of tumor were performed. The tumor was mostly composed of cellular spindle cell sarcoma with destruction of skull bone. Small amount of extracellular osteoid matrix was directly produced by sarcomatous tumor cells. Some transitional area form this sarcomatous portion to less celular benign fibrous dysplasia area. The fibrous dysplasia portion showed Chinese letter-like woven bone without osteoblastic rimming. After operation, chemotherapy was performed. However, 15 days after operation, the tumor rapidly recurred forming 4cm sized well enhancing mass at the same place. The tumor taken by second operation showed typical histologic findings of conventional oeteosarcoma without previous features of fibrous dysplasia