Neurofibromatosis and associated neuroectodermal tumors: a congenital neurocristopathy.
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.
D000310:Adrenal Gland Neoplasms / Q000151:congenital / Q000209:etiology / Q000473:pathology; D005260:Female; D005315:Fetal Diseases / Q000150:complications*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008175:Lung Neoplasms / Q000151:congenital / Q000209:etiology / Q000473:pathology; D008297:Male; D008479:Mediastinal Neoplasms / Q000151:congenital / Q000209:etiology / Q000473:pathology; D009373:Neoplasms, Germ Cell and Embryonal / Q000151:congenital / Q000209:etiology / Q000473:pathology*; D009423:Nervous System Neoplasms / Q000151:congenital / Q000209:etiology / Q000473:pathology; D009432:Neural Crest; D009456:Neurofibromatosis 1 / Q000151:congenital / Q000209:etiology / Q000473:pathology*; D009918:Orbital Neoplasms / Q000151:congenital / Q000209:etiology / Q000473:pathology; D010524:Peripheral Nervous System Neoplasms / Q000151:congenital / Q000209:etiology / Q000473:pathology; D011247:Pregnancy; D011471:Prostatic Neoplasms / Q000151:congenital / Q000209:etiology / Q000473:pathology; D013564:Sympathetic Nervous System