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Infantile fibrosarcoma of thigh--a case report
Ko Himori1, Masahito Hatori, Mika Watanabe
1Department of Orthopaedic Surgery, School of Medicine, Tohoku University, Japan.
Insights
Infantile fibrosarcoma, a rare childhood tumor, typically affects extremities but can occur in the thigh. This case highlights a favorable clinical course post-surgery and the diagnostic value of the ETV6-NTRK3 gene fusion.
Area of Science:
- Pediatric Oncology
- Surgical Pathology
- Molecular Diagnostics
Background:
- Infantile fibrosarcoma is a rare malignant soft tissue tumor predominantly affecting infants under one year old.
- It generally presents a more favorable clinical course with rare metastasis compared to adult fibrosarcoma.
- While adult fibrosarcoma commonly affects the thigh, infantile forms typically occur in distal extremities.
Observation:
- This case report details an infantile fibrosarcoma located in the thigh, an atypical site for this pediatric tumor.
- The patient experienced a favorable clinical course, with no recurrence observed 36 months post-tumor resection.
- Diagnostic challenges in differentiating infantile fibrosarcoma from other soft tissue tumors were noted.
Findings:
- Wide surgical excision was the primary treatment modality employed.
- The study emphasizes the utility of detecting the ETV6-NTRK3 gene fusion in the differential diagnosis of infantile fibrosarcoma.
- Confirmation of the ETV6-NTRK3 gene fusion aids in accurate diagnosis and management planning.
Implications:
- This case underscores that infantile fibrosarcoma can occur in unusual locations like the thigh.
- Early and accurate diagnosis, potentially aided by molecular markers like ETV6-NTRK3, is crucial for favorable outcomes.
- The findings support the standard treatment of wide surgical excision for infantile fibrosarcoma, even in atypical presentations.
Abstract:
Infantile fibrosarcoma is a rare soft tissue malignant tumor, when it occurs, it is usually seen in the first year of life. The clinical course of infantile fibrosarcoma is more favorable and metastasis is rare compared with that in adulthood. While adult fibrosarcoma are common in the thigh, infantile fibrosarcoma affect chiefly the distal portions of the extremities. Standard treatment is primarily wide surgical excision. In this case report, we present our experience of an infantile fibrosarcoma of thigh with good clinical course 36 months after tumor resection and the usefulness of detecting the ETV6-NTRK3 gene fusion in differential diagnosis.
