Neurofibromatosis and associated neuroectodermal tumors: a congenital neurocristopathy.
Pediatric pathology / affiliated with the International Paediatric Pathology Association(2009)
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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Key words
associated neuroectodermal tumors,congenital neurocristopathy
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