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1.
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
2.
Arch Argent Pediatr ; 118(6): e549-e553, 2020 12.
Article in Spanish | MEDLINE | ID: mdl-33231059

ABSTRACT

Infantile fibrosarcoma is a rare tumor, belonging to the nonrhabdomyosarcoma, 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 transcriptasepolymerase 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.


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 términoterminal. 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.


Subject(s)
Fibrosarcoma , Milk Hypersensitivity , Sarcoma , Soft Tissue Neoplasms , Animals , Cattle , Diagnosis, Differential , Female , Fibrosarcoma/diagnosis , Fibrosarcoma/surgery , Humans , Infant , Male
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