Malignant fibrous histiocytoma in the craniocervical junction presenting with severe occipitalgia

We report a patient who complained of severe occipitalgia caused by destruction of the atlantooccipital joint by tumor invasion. Her symptoms were relieved by tumor resection and occipitocervical fixation. Histological examination of the resected tumor revealed that the tumor cells had an irregular arrangement, remarkable atypia, and pleomorphism with multinucleated bizarre giant cells. The tumor demonstrated no definitive sarcoma differentiation and was identified as malignant fibrous histiocytoma. After tumor resection, the patient received adjuvant radiation and chemotherapy. The tumor regrew outside the radiation field. Chemotherapy with ifosfamide, cisplatin, and etoposide caused remarkable tumor reduction, but suspension of chemotherapy resulted in tumor recurrence. The results of our drug protocol suggest that this regimen is feasible as postoperative adjuvant chemotherapy for malignant fibrous histiocytoma. The role of adjuvant chemotherapy and radiation therapy for this highly malignant rare tumor should be evaluated in a prospective study with precise histological diagnosis.