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Neurofibromatosis 1; Recognition and Management of Associated Neuroblastoma
Author(s) -
Hayflick Susan J.,
Hofman Karen J.,
Tunnessen Walter W.,
Leventhal Brigid G.,
Dudgeon David L.
Publication year - 1990
Publication title -
pediatric dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.542
H-Index - 73
eISSN - 1525-1470
pISSN - 0736-8046
DOI - 10.1111/j.1525-1470.1990.tb01028.x
Subject(s) - medicine , neuroblastoma , neural crest , neurofibromatosis , malignancy , neurofibroma , pheochromocytoma , physical examination , pathology , oncology , pediatrics , embryo , genetics , biology , microbiology and biotechnology , cell culture
Neurofibroma and neuroblastoma both arise from the neural crest, and there has long been speculation regarding a pathogenetic relationship between them. Clinical characteristics do not necessarily distinguish these tumors, therefore the diagnosis of neuroblastoma should be considered in all children with neurofibromatosis 1 (NF‐1) who have a rapidly growing or inaccessible mass. A careful physical examination, imaging studies, and urinary catecholamine measurement are indicated. In a child with NF‐1 and malignancy, direct tissue examination may be necessary to differentiate malignant from nonmalignant tumor and guide therapy. Furthermore, with the significantly increased risk of certain types of childhood cancer in these patients, we recommend evaluation for this common heritable condition in all patients with malignancy.