RESUMO

BACKGROUND: Infantile fibrosarcoma is the most frequent soft tissue sarcoma in newborns or children under one year of age. This tumour often implies high local aggressiveness and surgical morbidity. The large majority of these patients carry the ETV6-NTRK3 oncogenic fusion. Hence, the TRK inhibitor larotrectinib emerged as an efficacious and safe alternative to chemotherapy for NTRK fusion-positive and metastatic or unresectable tumours. However, real-world evidence is still required for updating soft-tissue sarcoma practice guidelines. OBJECTIVE: To report our experience with the use of larotrectinib in pediatric patients. METHODS: Our case series shows the clinical evolution of 8 patients with infantile fibrosarcoma under different treatments. All patients enrolled in this study received informed consent for any treatment. RESULTS: Three patients received larotrectinib in first line. No surgery was needed with larotrectinib, which led to the rapid and safe remission of tumours, even in unusual anatomical locations. No significant adverse effects were observed with larotrectinib. CONCLUSION: Our case series supports that larotrectinib may be a therapeutic option for newborn and infant patients with infantile fibrosarcoma, especially in uncommon locations.

Assuntos

Fibrossarcoma , Sarcoma , Neoplasias de Tecidos Moles , Lactente , Humanos , Criança , Recém-Nascido , Fibrossarcoma/tratamento farmacológico , Fibrossarcoma/genética , Fibrossarcoma/patologia , Sarcoma/tratamento farmacológico , Neoplasias de Tecidos Moles/tratamento farmacológico , Neoplasias de Tecidos Moles/genética , Neoplasias de Tecidos Moles/patologia , Inibidores de Proteínas Quinases/uso terapêutico

RESUMO

Resection and reconstruction of the chest wall for the radical surgical treatment of malignant soft tissue tumors are currently considered a substantial challenge for thoracic surgeons. We present an unusual case of infantile fibrosarcoma with tropomyosin 3-neurotrophic receptor tyrosine kinase 1 fusion in a 13-year-old patient. The surgical treatment consisted of radical resection of the right posterior chest wall and reconstruction with the use of the STRATOS (Strasbourg Thoracic Osteosynthesis System) titanium rib bridge system. The patient had a favorable postoperative course and received respiratory-ventilatory rehabilitation, adjuvant therapy with chemotherapeutic agents, immunotherapy, and radiotherapy.