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Arch. argent. pediatr ; 118(6): e549-e553, dic 2020. ilus
Article in Spanish | LILACS, BINACIS | ID: biblio-1146218

ABSTRACT

El fibrosarcoma infantil es un tumor raro, perteneciente al grupo de sarcomas no rabdomiosarcoma; se presenta, preferentemente, en infantes, con localización más frecuente en extremidades.Se reporta un varón de 5 meses, con historia de sangrado digestivo desde los 3 meses, interpretados, al principio, como alergia a la proteína de leche de vaca, con evolución tórpida y anemia. Por laparoscopía exploradora, se evidenció un tumor en el intestino medio; se realizó resección y anastomosis término-terminal. Luego, se ampliaron los márgenes de resección por ser < 0,1 cm.Microscópicamente, se observó una proliferación neoplásica fusocelular con positividad difusa para vimentina, y reacción en cadena de la polimerasa con transcriptasa reversa positiva para ETV6-NTRK3. Se diagnosticó fibrosarcoma infantil intestinal. Presentó buena evolución a 24 meses del diagnóstico. Si bien es infrecuente en pediatría, se debe considerar como diagnóstico diferencial en lactantes con sangrado digestivo sin causa clara.


Infantile fibrosarcoma is a rare tumor, belonging to the non-rhabdomyosarcoma, soft tissue sarcoma. It is mostly presented in infants, most commonly involving the extremities.We report a 5-month-old boy, presenting with digestive bleeding since the age of 3 months, initially diagnosed as cow's milk allergy, with a torpid evolution and anemia. He underwent laparoscopic exploration, with evidence of a mass in the small bowel. Resection and end-to-end anastomosis were performed. Because of inadequate microscopic margins (< 1 cm), a new surgery was performed to achieve tumor free margins. Histological examination consisted of spindle cells that mainly expressed vimentin, and reverse transcriptase-polymerase chain reaction was positive for the ETV6-NTRK3 transcript, confirming the diagnosis of infantile fibrosarcoma. The patient did well after 24 months of follow-up.Although infantile intestinal fibrosarcoma is extremely uncommon in children, it should be considered as differential diagnosis for digestive bleeding in infants


Subject(s)
Humans , Male , Infant , Fibrosarcoma/diagnosis , Pediatrics , Fibrosarcoma/surgery , Gastrointestinal Hemorrhage , Intestinal Neoplasms
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