RESUMO

BACKGROUND: Malignant fibrous histiocytoma is a sarcoma of uncertain origin that can be found both in soft tissues and in bones. It is currently called undifferentiated pleomorphic sarcoma not otherwise specified and it represents a final common pathway in several tumors that are subject to the progress of dedifferentiation. Local recurrence of the tumor in the same location where it was originated occurs in 20-30% of the total number of soft tissue sarcomas. It is less frequent in limbs and more likely to recur in retroperitoneal sarcomas and in head and neck. Most tumors grow during the first two years after treatment. Most sarcoma metastases, especially those in the head and neck (including malignant fibrous histiocytoma) present as a lung disease (90%). Extrapulmonary diseases are not frequent and can occur in lymph nodes (10%), bones (8%) and in the liver (1%). CASE REPORT: 61 years old woman with history of malignant fibrous histiocytoma in the right portion of the mandible, resected four years ago. Subsequent checkups did not show any disease. The patient reports a constant abdominal pain not diagnosed, related to a pancreatic nodule. CONCLUSION: Malignant fibrous histiocytoma is a potentially curable disease. The most important part of the treatment is complete surgical removal, usually followed by adjuvant radiation therapy. Chemotherapy is a treatment usually used only on those patients with higher recurrence risk or on those showingrecurrence or who have already showed recurrence.