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Malignant fibrous histiocytoma of soft tissue in childhood
Insights
Complete surgical removal is key for treating malignant fibrous histiocytoma in children. Chemotherapy and radiation therapy show promise, especially Adriamycin, but their roles require further definition for this rare soft tissue sarcoma.
Area of Science:
- Pediatric Oncology
- Surgical Oncology
- Medical Oncology
Background:
- Malignant fibrous histiocytoma (MFH) is a rare soft tissue sarcoma affecting children.
- Treatment strategies for pediatric MFH have not been well-defined.
Purpose of the Study:
- To evaluate the outcomes of surgical management, chemotherapy, and radiation therapy for childhood malignant fibrous histiocytoma.
- To compare the response of MFH to treatment with that of childhood rhabdomyosarcoma.
Main Methods:
- Retrospective review of seven pediatric patients with MFH treated between 1975 and 1983.
- Treatment modalities included surgery (complete removal or biopsy), multi-agent chemotherapy (vincristine, dactinomycin, cyclophosphamide, with or without Adriamycin), and radiation therapy (RT).
Main Results:
- Two patients with completely excised localized tumors remain disease-free at 1.4 and 9 years.
- Three of five patients with residual tumor achieved complete response to RT and chemotherapy, with two remaining recurrence-free at 4 and 5 years.
- Three patients experienced regional or distant metastasis.
Conclusions:
- Complete tumor removal is critical for successful treatment of pediatric MFH.
- Multi-agent chemotherapy and RT show potential, with Adriamycin being a promising agent, but their definitive roles need further investigation.
- Treatment protocols for pediatric MFH may mirror those for other childhood soft tissue sarcomas.
Abstract:
Seven children aged 6 months to 11 years with malignant fibrous histiocytoma, a type of sarcoma of soft tissues, have been treated at the Children's Hospital of Philadelphia from January 1975 through July 1983. The primary tumor arose in the head and neck region in three patients, the chest wall in two patients and the pelvis or buttock in one patient each. Operative management consisted of complete tumor removal in the two patients with chest wall tumors, and biopsy only in the remaining five children. Afterward, all seven patients were treated with a multiple-agent chemotherapy program consisting of vincristine, dactinomycin, and cyclophosphamide for two years, with or without Adriamycin (doxorubicin). The five patients with residual tumor also received radiation therapy (RT) in doses of 1500 to 5500 rad. The two children with localized, completely excised sarcoma are continuously free of tumor at 1.4 and 9 years after initiation of treatment. Of the five with residual sarcoma, three had a complete response to radiation and chemotherapy, and two of them are free of recurrence at 4 and 5 years, respectively. In the three remaining children, the tumor spread regionally into the central nervous system or distantly into the lungs, subcutaneous tissues, and liver. Childhood malignant fibrous histiocytoma of soft tissue appears to be similar to childhood rhabdomyosarcoma in its modes of spread and response to management. Operative removal is the key to successful therapy. The roles of multiple-agent chemotherapy and RT remain to be defined. Adriamycin appears to be the most promising single agent. In the absence of concrete data, it seems prudent to follow the same guidelines for irradiation as those used for other soft tissue sarcomas of childhood.