Malignant Fibrous Histiocytoma - An Unusual Transformation from Benign to Malignant

Malignant Fibrous Histiocytoma: An Uncommon Sarcoma with Pathological Fracture of Mandible

“…In 2002, the World Health Organization declassified MFH as a formal diagnostic entity and renamed it as an undifferentiated pleomorphic sarcoma not otherwise specified. This new terminology has been supported by a compelling body of evidence over the last decade to suggest that MFH represents a final common pathway in tumors that undergo progression towards undifferentiation [6]. Additionally, MFH has been associated with hematopoietic diseases such as non-Hodgkin's lymphoma, Hodgkin's lymphoma, multiple myeloma and malignant histiocytosis [5].…”

“…1 Extra-oral view of the patient illustrating swelling of right side of the face 10 % of all soft tissue sarcomas and approximately 1 % of all head and neck neoplasms. Nevertheless, they represent an important group of tumors and are associated with significant morbidity and mortality [6]. Malignant fibrous histiocytoma, the most frequent soft tissue sarcoma of adulthood, was first described as a new malignant tumour by O'Brian and Stout in the 1960s and the details of the histopathological features of MFH were first described by Kempson and Kyriakos [1].…”

“…The giant cell variant represents a highly aggressive lesion in regard to local growth, local recurrence, and distant metastasis. The storiform/pleomorphic and inflammatory MFHs fall between these two extremes [6]. It is common for several morphologic patterns to be observed within one lesion.…”

“…Although local control of the primary tumor is critical to successful treatment of both high-and low-grade lesions, the high rate of distant metastases in high-grade tumors supports the role of combined modality therapy. Since regional lymph nodes are involved in 10-18 % of cases consideration should be given to elective neck dissection for patients with advanced-stage disease or evidence of aggressive histopathological features [6].…”

“…Systemic chemotherapy is recommended for those tumors with a significant risk of distant metastasis [4]. Chemotherapy has been used in patients with a predictably high risk of pulmonary metastasis [6]. The administration of anticancer agents such as methotrexate, cisplatin, doxorubicin, cyclophosphamide, actinomycin-D, vincristine, dacarbazine, and ifosfamide has been reported in patients with soft tissue sarcoma.…”