Clinical and Molecular Characteristics, Management and Outcome of Infantile Fibrosarcoma: A Retrospective 18-Year Single-Institution Review

Author Info

Mathey Marina Peruzzo Luisina Belen López Marti Jessica Innocenti Sergio Ramos Pacheco Maria Luz Cacciavillano Walter Felizzia Guido Viso Marianela Zubizarreta Pedro Rose Aadriana

Corresponding Author

Mathey Marina
Department of Hematology and Oncology, Hospital de Pediatría S.A.M.I.C. “Prof. Dr. Juan P. Garrahan”, Buenos Aires, Argentina

A B S T R A C T

Infantile fibrosarcoma (IFS) is the most common non-rhabdomyosarcoma soft-tissue sarcoma in children under 1 year of age, with local aggressiveness, but a high cure rate with conservative treatment. We report our experience with 15 patients with histological diagnosis of IFS treated at our Institution from January-2003 to December-2020. The median age was 6.72 months (range: 0-36); 66% were males. The extremities were the most common tumor site (66.7%). The tumor size was > 5 cm in 10 patients. Only one patient had metastatic disease (lung). Molecular studies were performed in 14 patients and were positive in ten: nine ETV6/NTRK3 and one RAF1. Initial non-mutilating surgery was performed in six patients. A delayed surgery could be performed in 4/8 patients after neoadjuvant chemotherapy with Vincristine - Actinomycin-D, and in one patient a watch-and-wait strategy was adopted. With a median follow-up of 58.67 months, all patients are alive except for the patient with metastatic disease. The 5-year overall and event-free survival were 93.5% and 86.5% respectively. Although IFS is locally aggressive, it has a high rate of overall survival due to its good response to chemotherapy and non-mutilating surgery even with involved margins. Advances in molecular genetics have improved diagnosis and refined classification.

Article Info

Article Type

Research Article

Publication history

Received: Fri 22, Jul 2022
Accepted: Mon 15, Aug 2022
Published: Wed 14, Sep 2022

Copyright

© 2023 Mathey Marina. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. Hosting by Science Repository.
DOI: 10.31487/j.SCR.2022.09.02