Peripheral neuroepithelioma in childhood

摘要: Peripheral neuroepithelioma is a rare and controversial neoplasm that may occur at any age. Fifteen of the 38 previously reported cases have involved children from birth to 17 years The authors observed course 3-month-old girl who presented with an enlarging mass in left arm manifested hepatic metastases time diagnosis. urinary level vanillylmandelic acid (VMA) was moderately elevated. primary lesion excised metastatic foci showed response regimen vincristine, cyclophosphamide, Adriamycin (doxorubicin), cisplatin. However, tumor recurred brain liver child died 14 months after At autopsy, there no involvement adrenal glands or sympathetic ganglia contained numerous involuted lesions as well active metastases. It suggested this unique neoplasm, derived neural crest but distinct neuroblastoma, which can be characterized by peripheral origin, histologic pattern confluent pseudorosettes, aggressive clinical behavior.