PMID: 8940736Nov 1, 1996Paper

Primary malignant fibrous histiocytoma of the lung in a child: a case report and review of literature

Pediatric Hematology and Oncology
S J ShahL C Yu

Abstract

Malignant fibrous histiocytoma (MFH), an aggressive high-grade soft tissue sarcoma, usually occurs in the elderly during the fifth to seventh decade of life. It commonly arises in the retroperitoneum, extremities, 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 completing radiation therapy, he was started on vincristine, actinomycin D, and cyclophosphamide alternating with vincristine, doxorubicin, and cyclophosphamide every 3 weeks. After five such cycles, he had a histologically proven local recurrence. He then received chemotherapy consisting of ifosfamide (2 g/m2) and etoposide (VP-16) (100 mg/m2) given daily for 3 days every 3 weeks. The patient attained complete remission (CR) after five such cycles and completed treatment without any major complications. He received a total of 16 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...Continue Reading

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Citations

Dec 31, 2002·Clinical Imaging·Daphne J TheodorouDavid J Sartoris
Jan 9, 2010·Pediatric and Developmental Pathology : the Official Journal of the Society for Pediatric Pathology and the Paediatric Pathology Society·Rita AlaggioCheryl M Coffin
Mar 4, 1998·The Australian and New Zealand Journal of Psychiatry·J K LiC S Cockram

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