Malignant fibrous histiocytoma in childhood.
BACKGROUND
Malignant fibrous histiocytoma (MFH) is a neoplasm of late adult life and often is reported in the pediatric population. It is thought to behave more benignly in children than in adults.


METHODS
Clinical and pathologic features, treatment, and outcome of nine pediatric patients seen at British Columbia's Children's Hospital between 1983 and 1990 were examined. The literature regarding pediatric malignant fibrous histiocytoma was reviewed.


RESULTS
The tumors included one primary renal tumor and two occurring in the orbit after radiation therapy for retinoblastoma during the neonatal period. Histologic examination showed that six tumors had a storiform-pleomorphic pattern, one was myxoid, and two were angiomatoid in type. Six children are alive with a disease-free survival of 20 months to 8 years after surgical resection. Two of these received adjuvant chemotherapy, none had radiation therapy. Three patients have died of disease, two with pulmonary metastases that developed despite multiagent chemotherapy and radiation therapy. Poor outcome was associated with large tumors, deep and proximal location, and the storiform-pleomorphic histologic type with atypical mitoses.


CONCLUSIONS
Malignant fibrous histiocytoma is similar in children and adults. Surgery is the mainstay of therapy, but the risk of local recurrence and pulmonary metastases indicates the need for adjuvant therapy in selected patients.
Department of Hematology/Oncology, Princess Margaret Hospital, Subiaco, Australia.
C H
CH
Cole
J F
JF
Magee
M
M
Gianoulis
P C
PC
Rogers
D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D003094:Collagen; D005134:Eye Neoplasms / Q000532:radiotherapy; D005260:Female; D015726:Giant Cells / Q000473:pathology; D006644:Histiocytes / Q000473:pathology; D018219:Histiocytoma, Benign Fibrous / Q000188:drug therapy / Q000473:pathology* / Q000601:surgery; D006801:Humans; D007680:Kidney Neoplasms / Q000473:pathology; D008264:Macrophages / Q000473:pathology; D008297:Male; D009381:Neoplasms, Radiation-Induced / Q000473:pathology; D012175:Retinoblastoma / Q000532:radiotherapy; D009418:S100 Proteins / Q000032:analysis; D012983:Soft Tissue Neoplasms / Q000473:pathology; D015996:Survival Rate; D016896:Treatment Outcome; D000515:alpha 1-Antitrypsin / Q000032:analysis
D009418:S100 Proteins; D000515:alpha 1-Antitrypsin; D003094:Collagen