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April 18, 2026JBJS Case Connector0 citations

Sclerosing Rhabdomyosarcoma Arising in Desmoid Fibromatosis 17 Years After Therapeutic Radiation

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JLJean G. LoukaMJMaryam JawidTATaha Aksoy

Key Points

  • To analyze a case of sclerosing rhabdomyosarcoma occurring after radiation therapy for desmoid fibromatosis.
  • Case report of a 54-year-old man with prior radiation treatment for desmoid fibromatosis.
  • Follow-up after 17 years revealed an enlarging mass leading to biopsies for diagnosis.
  • Treatment included chemotherapy and transfemoral amputation.
  • Initial biopsy indicated recurrent desmoid fibromatosis; repeat biopsy confirmed sclerosing rhabdomyosarcoma.
  • The patient developed pulmonary and chest wall metastases following intervention.
  • The tumor's location in the radiation field suggests potential malignant transformation due to radiation.

Abstract

Case: A 54-year-old man received radiation therapy (50 Gy) for popliteal desmoid fibromatosis. After 17 years, he developed an enlarging mass in the same location. Initial biopsy suggested recurrent desmoid, but a repeat biopsy revealed sclerosing rhabdomyosarcoma. Despite chemotherapy and transfemoral amputation, he developed pulmonary and chest wall metastases. Conclusion: The 17-year latency period and tumor location within the prior radiation field are consistent with radiation-associated malignant transformation; however, definitive causality cannot be established. Orthopaedic surgeons should maintain a low threshold for rebiopsy of any new or changing mass in previously irradiated fields.

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Cite This Study

Louka et al. (2026) studied this question.

synapsesocial.com/papers/69e3216540886becb6540b36https://doi.org/10.2106/jbjs.cc.25.00707
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