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Pediatr Pathol. 1986;5(1):65-78. doi: 10.3109/15513818609068849.

Neurofibromatosis and associated neuroectodermal tumors: a congenital neurocristopathy.

Pediatric pathology

S J Qualman, W R Green, C Brovall, B G Leventhal

PMID: 3014462 DOI: 10.3109/15513818609068849

Abstract

The synchronous occurrence of neurofibromatosis and neuroblastoma has been labeled in the recent literature as a chance event. We report 2 cases of newborn infants with congenital neurofibromatosis and a similar midline pattern of multiple Schwann cell and neuroblastic tumors; other types of ectomesenchymal tumor differentiation are documented, along with supportive ultrastructural and immunohistochemical studies. The tumors may take an aggressive, fatal course despite maximal multimodality antitumor therapy. These 2 cases are reported, with additional literature review, to document a clinically recognizable neurocristopathy that links neuroblastic tumors and neurofibromatosis.

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