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Malignant fibrous histiocytoma in children
C A Corpron1, C T Black, R B Raney
1Department of Surgical Oncology, University of Texas M.D. Anderson Cancer Center, Houston, USA.
Insights
Malignant fibrous histiocytoma (MFH) in children is rare. Prognostic factors like clinical group and tumor size significantly impact survival, with surgery remaining key, though adjuvant therapies warrant further study.
Area of Science:
- Pediatric Oncology
- Surgical Pathology
- Cancer Research
Background:
- Malignant fibrous histiocytoma (MFH) is a rare tumor in children, leading to limited understanding of its natural history and prognostic factors.
- Previous studies have not well-described outcomes for pediatric MFH cases.
Purpose of the Study:
- To review pediatric patients with MFH at a major cancer center.
- To identify prognostic factors predictive of survival in pediatric MFH.
Main Methods:
- Retrospective chart review of 44 pediatric patients diagnosed with MFH.
- Analysis of presentation, treatment, and outcomes.
- Univariate analysis to identify significant prognostic factors.
Main Results:
- The extremities were the most common tumor site (31/44).
- Five-year survival rates varied significantly by clinical group (0% for group IV) and tumor size (<5 cm: 95%, >5 cm: 45%).
- Overall 5-year survival was 71%; clinical group, tumor size, and recurrence were significant prognostic factors.
Conclusions:
- Clinical group and tumor size are critical prognostic indicators for pediatric MFH.
- While surgery is the primary treatment, further investigation into adjuvant chemotherapy and radiation is recommended for specific patient groups.
Abstract:
Because malignant fibrous histiocytoma (MFH) rarely occurs in children, the natural history of this tumor and prognostic factors predictive of outcome have not been well described. The charts of all pediatric patients with MFH seen at M.D. Anderson Cancer Center were reviewed with respect presentation, treatment, and outcome, in an attempt to determine prognostic factors that are predictive of survival. Forty-four pediatric patients were identified. Extremities were the most common tumor site (31 of 44 patients). Five patients presented with angiomatoid histology subtype; all subsequently survived. The estimated 5-year survival rate was 85% for clinical group I patients, 87% for clinical group II, 53% for clinical group III, and 0% for clinical group IV. The estimated 5-year survival rate was 95% for patients with tumors of less than 5 cm in diameter and 45% for those with larger tumors. Overall, the estimated 5-year survival rate was 71%. Significant prognostic factors found to affect survival (by univariate analysis) were clinical group, tumor size, and recurrence. Gender and race were not significant predictors. The use of chemotherapy and radiation was not found to improve the chance of survival, but this most likely reflected the more frequent use of adjuvant therapy in patients with unresectable or high-grade tumors. Although adequate surgical resection continues to be the most effective treatment, investigation of adjuvant chemotherapy and radiation therapy on protocol is warranted.