How Should We Treat Infantile Fibrosarcoma?

Abstract

Background and Aims: Infantile fibrosarcoma is a rare soft tissuetumor that can rarely be congenital, usually involving the extremi-ties.Historically these tumors have been treated with surgical resec-tions requiring amputation.Chemotherapy and conservative surgicalapproaches have been favored in recent studies.We aimed to discussthe treatment approach of these tumors with3different congenitalinfantile fibrosarcoma casesMethods: Descriptive case reportResults: Case1:A term male baby was born with a mass lesion on theleft forearm.Ultrasound showed a4*3*3cm sized hypervascular, solidlesion surrounding the half of the radius.Biopsy pathology resulted asinfantile fibrosarcoma(IF).Surgical excision was performed.The pathol-ogy report showed the lesion was adjacent to the surgical border.Tumorboard favored follow up without chemotherapy.He is stable with nomacroscopic residue in the follow-up imaging for 24 months.Case2:A21-day-old girl was brought with the complaint of enlarged swellingon the back of her right hand.MRI revealed a large mass surround-ing the right-hand bones.It was operated due to compartment syn-drome.The pathological examination resulted as IF.She was treatedwithVA followed byVAC courses due to residual mass.MRI showed amilimetric residual contrast enhancement.Family favored cessation ofchemotherapy.She is under follow up with stabile millimetric residuefor more than12 months.Case3:In the 22nd-week scanning of the firstpregnancy of a healthy mother, a mass was detected on the baby’sneck.Fetal MRI showed7*8*7cm sized infiltrative mass on the front leftside of the neck that extends to the root of the tongue and causes signif-icant narrowing of the airway.Biopsy pathology showed IF.The patientwas not appropriate for curative surgery,therefore tracheostomy andgastrostomy were performed.He was treated with VA followed by VAC courses but there was no reduction in lesion size.Continuationchemotherapy with vinblastine is ongoing.All cases were positive forETV6-NTRK3fusion.Conclusions: IF is the most common soft tissue sarcoma under1 year, 40% present at birth. Curative surgery may be muti-lating. Current treatment approach is non-mutilating surgery& conservative approach with alkylating and anthracycline-freechemotherapy