[Soft tissue tumors in neonates].
Soft tissue tumors account for approximately 25% of neonatal tumors and are most often benign (more than 2/3 of cases). Vascular tumors are the most frequent benign tumors and infantile hemangioma accounts for 32% of these tumors, affecting 1 out of 200 children at birth. Kaposiform hemangioendothelioma (KH) is a rare vascular tumor with locally aggressive behavior. More than 50% of KH are associated with the Kasabach-Merritt phenomenon, a condition characterized by thrombocytopenia and consumptive coagulopathy. Malignant soft tissue tumors are, after neuroblastoma, the second cause of cancer in neonates. Infantile fibrosarcoma (IF) is a rare tumor that most often affects the extremities of children aged 4 years or younger. A recurrent t(12;15) (p13;q25) rearrangement fusing the ETV6 gene with the NTRK3 neurotrophin-3 receptor gene has been identified in IF. Complete conservative surgical resection is usually curative. Chemotherapy is indicated when initial surgical removal cannot be accomplished without unacceptable morbidity. Prognosis of IF is excellent, with reported overall survival rates ranging from 80 to 100%. Neonatal rhabdomyosarcoma (RMS) is a rare tumor (0.5-1% of RMS). The primary tumor predominantly involves the limbs and the genitourinary tract. Treatment is based on age-adapted chemotherapy and surgery. Prognosis of RMS in children less than 1 year old appears to be comparable with that of older children.
Minard-Colin
V
V
Département de pédiatrie, institut Gustave-Roussy, 39, rue Camille-Desmoulins, 94805 Villejuif cedex, France.
Orbach
D
D
Martelli
H
H
Bodemer
C
C
Oberlin
O
O
D005354:Fibrosarcoma / Q000151:congenital / Q000175:diagnosis / Q000235:genetics / Q000628:therapy; D050939:Gene Fusion / Q000235:genetics; D015321:Gene Rearrangement / Q000235:genetics; D006390:Hemangioendothelioma / Q000151:congenital / Q000175:diagnosis / Q000628:therapy; D006391:Hemangioma / Q000151:congenital / Q000175:diagnosis / Q000628:therapy; D006801:Humans; D007231:Infant, Newborn; D011379:Prognosis; D050783:Proto-Oncogene Proteins c-ets / Q000235:genetics; D020812:Receptor, trkC / Q000235:genetics; D012097:Repressor Proteins / Q000235:genetics; D012208:Rhabdomyosarcoma / Q000151:congenital / Q000175:diagnosis / Q000628:therapy; D012983:Soft Tissue Neoplasms / Q000151:congenital* / Q000175:diagnosis / Q000628:therapy; D000097764:ETS Translocation Variant 6 Protein
D050783:Proto-Oncogene Proteins c-ets; D020812:Receptor, trkC; D012097:Repressor Proteins; D000097764:ETS Translocation Variant 6 Protein