RESUMO
Kaposiform hemangioendothelioma (KHE) is a locally aggressive vascular tumor of childhood although cases occurring in adulthood are also described. The features overlap with juvenile capillary hemangioma and Kaposi sarcoma. We report a rare case of recurrent, multifocal (nose and chin) cutaneous KHE initially occurring in a 3-year-old female child, uncomplicated by Kasabach-Merritt syndrome. Recurrences occurred over the next 6 years and resulted in complete distortion of the nose, requiring plastic repair.
Assuntos
Hemangioendotelioma/diagnóstico , Hemangioendotelioma/patologia , Síndrome de Kasabach-Merritt/diagnóstico , Síndrome de Kasabach-Merritt/patologia , Neoplasias Bucais/diagnóstico , Neoplasias Bucais/patologia , Neoplasias Nasais/diagnóstico , Neoplasias Nasais/patologia , Sarcoma de Kaposi/diagnóstico , Sarcoma de Kaposi/patologia , Biomarcadores Tumorais/análise , Pré-Escolar , Feminino , Histocitoquímica , Humanos , Imuno-Histoquímica , Síndrome de Kasabach-Merritt/complicações , Microscopia , Molécula-1 de Adesão Celular Endotelial a Plaquetas/análise , RecidivaRESUMO
Congenital extraskeletal Ewing's sarcoma or peripheral primitive neuroectodermal tumor is an extremely uncommon and invariably fatal tumor. We report a case of extraskeletal congenital Ewing's sarcoma in a female fetus delivered at 34 weeks of gestation who died immediately after birth. In English literature, majority of cases of Ewing's sarcoma in neonates reported were skeletal. To the best of our knowledge, very few cases of extra-skeletal Ewing's sarcoma in neonates are reported in the literature.
