PRIMARY MALIGNANT FIBROUS HISTIOCYTOMA OF THE LUNG IN A CHILD - A CASE-REPORT AND REVIEW OF LITERATURE

Malignant fibrous histiocytoma (MFH), an aggressive high-grade soft ti ssue sarcoma, usually occurs in the elderly during the fifth to sevent h decade of life. It commonly arises in the retroperitoneum, extremiti es, and head and neck region. Primary pulmonary MFH is extremely rare and is frequently fatal. We present the youngest known case, a 9-year- old boy with a primary left lung grade II inflammatory MFH, stage II. He underwent a left upper lobectomy for tumor resection. After complet ing radiation therapy, he was started on vincristine, actinomycin. D, and cyclophosphamide alternating with vincristine, doxorubicin, and cy clophosphamide every 3 weeks. After five such cycles, he had a histolo gically proven local recurrence. He then received chemotherapy consist ing of ifosfamide (2 g/m(2)) and etoposide (VP-16) (100 mg/m(2)) given daily for 3 days every 3 weeks. The patient attained complete remissi on (CR) after five such cycles and completed treatment without any maj or complications. He received a total of IG courses and is continuing in CR 36 months off treatment. Ifosfamide and etoposide (VP-16), known for their usefulness in treatment of adult soft tissue sarcomas, can be used as salvage chemotherapy for patients with MFH who fail the fro nt-line conventional chemotherapy.