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Peripheral neuroepithelioma in childhood
Author(s) -
Voss Barbara L.,
Pysher Theodore J.,
Humphrey G. Bennett
Publication year - 1984
Publication title -
cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.052
H-Index - 304
eISSN - 1097-0142
pISSN - 0008-543X
DOI - 10.1002/1097-0142(19841215)54:12<3059::aid-cncr2820541241>3.0.co;2-4
Subject(s) - medicine , vincristine , autopsy , neuroblastoma , peripheral , pathology , cyclophosphamide , lesion , neoplasm , vanillylmandelic acid , urinary system , neural crest , chemotherapy , homovanillic acid , embryo , receptor , biology , microbiology and biotechnology , serotonin , genetics , cell culture
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 of age. The authors observed the course of a 3‐month‐old girl who presented with an enlarging mass in the left arm and manifested hepatic metastases at the time of diagnosis. The urinary level of vanillylmandelic acid (VMA) was moderately elevated. The primary lesion was excised and metastatic foci showed response to a regimen of vincristine, cyclophosphamide, Adriamycin (doxorubicin), and cisplatin. However, tumor recurred in the brain and liver and the child died 14 months after diagnosis. At autopsy, there was no involvement of adrenal glands or sympathetic ganglia and the liver contained numerous involuted lesions as well as active metastases. It is suggested that this is a unique neoplasm, derived from neural crest but distinct from neuroblastoma, which can be characterized by peripheral origin, a histologic pattern of confluent pseudorosettes, and aggressive clinical behavior.