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Infantile fibrosarcoma

E B Chung, F M Enzinger

    Cancer
    |August 1, 1976
    PubMed
    Summary
    This summary is machine-generated.

    Infantile fibrosarcoma, a rare tumor in young children, often affects extremities but typically has a favorable outcome with wide local excision. Long-term follow-up is crucial due to potential late recurrences.

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    Area of Science:

    • Pediatric Oncology
    • Surgical Pathology
    • Pediatric Surgery

    Background:

    • Infantile fibrosarcoma is a rare soft tissue tumor predominantly affecting infants.
    • Understanding its pathologic features and behavior is crucial for effective management.
    • This study analyzes a cohort of 53 infantile fibrosarcoma cases.

    Purpose of the Study:

    • To present the pathologic features and clinical behavior of infantile fibrosarcoma.
    • To evaluate treatment outcomes and identify optimal management strategies.
    • To emphasize the importance of long-term follow-up for this condition.

    Main Methods:

    • Retrospective review of 53 infantile fibrosarcoma cases.
    • Analysis of tumor characteristics, patient demographics, treatment modalities, and follow-up data.

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  • Pathologic assessment included cellularity and mitotic activity.

    • Main Results:

    • Tumors predominantly occurred in the first two years of life, with a male predominance (60%).
    • Distal extremities were the most common sites (72%); tumors were rapidly growing and infiltrative.
    • Despite aggressive microscopic features, 31 of 48 patients with follow-up were alive and well; 8.3% died of metastatic disease.

    Conclusions:

    • Infantile fibrosarcoma exhibits a more favorable clinical course than adult fibrosarcoma.
    • Wide local excision is the preferred treatment, with amputation reserved for extensive tumors.
    • Long-term surveillance is essential due to the possibility of late recurrences and metastases.