Les tumeurs mésenchymateuses du nouveau-né

V. Minard-Colin, D. Orbach, H. Martelli, C. Bodemer, O. Oberlin

    Résultats de recherche: Contribution à un journalBrève enquêteRevue par des pairs

    26 Citations (Scopus)

    Résumé

    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.

    Titre traduit de la contributionSoft tissue tumors in neonates
    langue originaleFrançais
    Pages (de - à)1039-1048
    Nombre de pages10
    journalArchives de Pediatrie
    Volume16
    Numéro de publication7
    Les DOIs
    étatPublié - 1 juil. 2009

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