Marginal resection as a potential curative treatment option of infantile fibrosarcoma with good response after chemotherapy: A case report of an ETV6-NTRK3 positive infantile fibrosacroma of the distal tibia

• Published in: Medicine (Baltimore) Vol. 102; no. 27; p. e34194
• Main Authors: Weber, Markus, von Kunow, Frederik, Hillmann, Axel
• Format: Journal Article
• Language: English
• Published: United States Lippincott Williams & Wilkins 07.07.2023
• Subjects: Clinical Case Report; Fibrosarcoma - drug therapy; Fibrosarcoma - surgery; Humans; Infant; Oncogene Proteins, Fusion; Receptor Protein-Tyrosine Kinases; Sarcoma; Soft Tissue Neoplasms - pathology; Tibia - pathology; Tibia - surgery
• ISSN: 0025-7974; 1536-5964
• DOI: 10.1097/MD.0000000000034194

The infantile fibrosarcoma (IFS) is a non-rhabdomyosarcoma soft tissue sarcoma with locally aggressive properties. State of the art therapy consists of neoadjuvant chemotherapy followed by wide resection according to the criteria of the musculoskeletal tumor society. An ETV6-NTRK3 positive IFS of the distal tibia in a 21-months old child showed good response to chemotherapy. Due to refusal of amputation marginal resection completing the margins with a high speed drill and filling the space with bone cement was performed. At latest follow-up 10 years after surgery, no recurrence was observed. An individual therapy for surgical treatment of IIFS is recommended. This comprises marginal resection in instead of the golden standard "wide resection" in selected cases.