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December 1982

Ganglioneuroma and Acute Lymphoblastic Leukemia in Association With Neurofibromatosis

Author Affiliations

The Mount Sinai School of Medicine New York; Pascack Valley Hospital Westwood, NJ

Am J Dis Child. 1982;136(12):1090-1091. doi:10.1001/archpedi.1982.03970480056012

Neurofibromatosis (NF) is a neurocutaneous disorder that is inherited as an autosomal dominant condition or appears sporadically as a new mutation with variable expressivity. The diagnosis may be established by finding six or more cafe au lait spots greater than or equal to 1.5 cm in diameter, axillary freckles, and neurofibromas of peripheral, spinal, and/or cranial nerves.1 Tumors, both benign and malignant, occur with an increased frequency in patients with NF most commonly as a transformation of an existing neurofibroma or other tissue of neural crest origin; thus, schwannoma, neurilemoma, acoustic neuroma, optic glioma, meningioma, and ganglioneuroma have all been reported.2 In addition, Wilms' tumor,3 rhabdomyosarcoma,4 neuroblastoma,5 and acute leukemia6 have been described in association with NF. A recent epidemiologic survey of patients with NF and their families demonstrated that, of 266 individuals questioned, 31 reported at least one cancer and second tumors developed