C. Canpolat et al., FIBROMYXOID SARCOMA IN A 4-YEAR-OLD CHILD - CASE-REPORT AND REVIEW OFTHE LITERATURE, Medical and pediatric oncology, 27(6), 1996, pp. 561-564
We present a child with a rare and chemotherapy-resistant form ef soft
-tissue cancer, low-grade fibromyxoid sarcoma, first noted when he was
4 years old. He is the youngest patient reported to date. An 11-year-
old white male presented to The University of Texas M. D. Anderson Can
cer Center's Department of Pediatrics with a 7-year history of right t
high mass and pulmonary nodules, confirmed on examination. He had unde
rgone extensive prior chemotherapy and surgery. He received chemothera
py with high-dose cyclophosphamide (7 g/m(2)) and later etoposide (150
mg/m(2)/day x 5), with only slight shrinkage of the thigh mass and no
ne in the lungs. Subsequently the tumor in his proximal thigh and his
lung metastases were resected, and radiation therapy was administered
to the thigh. His disease remained stable for 12 months, but he then d
eveloped a pleural-based metastasis on the left side and new, bilatera
l lung metastases also. The tumors on the left side were removed; resi
dual disease is stable after treatment for 6 months with subcutaneous
alpha-interferon-2b. Low-grade fibromyxoid sarcoma is very uncommon in
children. It grows slowly and metastasizes to distant organs, chiefly
to the lungs. It is resistant to conventional chemotherapy, and thus
far only surgery seems to have a life-prolonging effect. Newer chemoth
erapeutic and possibly biologic agents should be tried in future patie
nts, in order to find an effective way to control the disease. (C) 199
6 Wiley-Liss, Inc.