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Malignant fibrous histiocytoma in childhood
C H Cole1, J F Magee, M Gianoulis
1Department of Hematology/Oncology, Princess Margaret Hospital, Subiaco, Australia.
Insights
Malignant fibrous histiocytoma (MFH) in children is similar to adults. Surgery is key, but adjuvant therapy may be needed for selected pediatric patients due to recurrence and metastasis risks.
Area of Science:
- Pediatric Oncology
- Surgical Pathology
- Cancer Epidemiology
Background:
- Malignant fibrous histiocytoma (MFH) is a rare tumor typically affecting adults, but also reported in children.
- Pediatric MFH is often considered to have a more benign clinical course compared to adult cases.
Purpose of the Study:
- To analyze the clinical and pathological characteristics of pediatric malignant fibrous histiocytoma.
- To evaluate treatment strategies and outcomes for children diagnosed with MFH.
- To compare pediatric MFH behavior and outcomes with existing adult data.
Main Methods:
- Retrospective review of nine pediatric patients with MFH treated at British Columbia's Children's Hospital (1983-1990).
- Examination of clinical data, pathological features, treatment regimens, and patient outcomes.
- Comprehensive literature review of pediatric malignant fibrous histiocytoma cases.
Main Results:
- Tumor locations varied, including one primary renal tumor and two orbital tumors post-radiation for retinoblastoma.
- Histological subtypes identified were storiform-pleomorphic (6), myxoid (1), and angiomatoid (2).
- Six children achieved disease-free survival (20 months to 8 years) post-surgery; three patients died of disease, with two developing pulmonary metastases despite aggressive therapy.
Conclusions:
- Pediatric malignant fibrous histiocytoma shares similarities with adult MFH in terms of presentation and behavior.
- Surgical resection remains the primary treatment modality for pediatric MFH.
- Adjuvant therapy is recommended for selected pediatric patients due to the significant risk of local recurrence and pulmonary metastasis.
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.
Method:
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.
Conclusion:
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.