How Should We Treat Infantile Fibrosarcoma?


Tuna R. , Tuğcu D. , Bilici M. , Karaman S. , Aydın A., Aydemir L. , ...More

SIOP 2020 | Virtual Paediatric Oncology Congress, 14 - 17 October 2020, vol.67, pp.403-404

  • Publication Type: Conference Paper / Summary Text
  • Volume: 67
  • Page Numbers: pp.403-404

Abstract

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