How Should We Treat Infantile Fibrosarcoma?

SIOP 2020 | Virtual Paediatric Oncology Congress, 14 - 17 Ekim 2020, cilt.67, ss.403-404

Background and Aims: Infantile fibrosarcoma is a rare soft tissue

tumor 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 surgical

approaches have been favored in recent studies.We aimed to discuss

the treatment approach of these tumors with3different congenital

infantile fibrosarcoma cases

Methods: Descriptive case report

Results: Case1:A term male baby was born with a mass lesion on the

left forearm.Ultrasound showed a4*3*3cm sized hypervascular, solid

lesion surrounding the half of the radius.Biopsy pathology resulted as

infantile fibrosarcoma(IF).Surgical excision was performed.The pathol-

ogy report showed the lesion was adjacent to the surgical border.Tumor

board favored follow up without chemotherapy.He is stable with no

macroscopic residue in the follow-up imaging for 24 months.Case2:A

21-day-old girl was brought with the complaint of enlarged swelling

on 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 treated

withVA followed byVAC courses due to residual mass.MRI showed a

milimetric residual contrast enhancement.Family favored cessation of

chemotherapy.She is under follow up with stabile millimetric residue

for more than12 months.Case3:In the 22nd-week scanning of the first

pregnancy of a healthy mother, a mass was detected on the baby’s

neck.Fetal MRI showed7*8*7cm sized infiltrative mass on the front left

side of the neck that extends to the root of the tongue and causes signif-

icant narrowing of the airway.Biopsy pathology showed IF.The patient

was not appropriate for curative surgery,therefore tracheostomy and

gastrostomy were performed.He was treated with VA followed by VAC courses but there was no reduction in lesion size.Continuation

chemotherapy with vinblastine is ongoing.All cases were positive for

ETV6-NTRK3fusion.

Conclusions: IF is the most common soft tissue sarcoma under

1 year, 40% present at birth. Curative surgery may be muti-

lating. Current treatment approach is non-mutilating surgery

& conservative approach with alkylating and anthracycline-free

chemotherapy