ISSN:
1435-5922
Keywords:
diffuse ganglioneuromatosis
;
plexiform neurofibroma
;
von Recklinghausen's disease
;
multiple endocrine neoplasia type IIb
Source:
Springer Online Journal Archives 1860-2000
Topics:
Medicine
Notes:
Abstract A rare case of diffuse ganglioneuromatosis with plexiform neurofibromas limited to the gastrointestinal tract in a 20-year-old man is reported. The patient had no cafe-au-lait spots or any apparent tumorous lesions affecting other organs. A fan-shaped resection of the mesentery, including 200 cm of the ileum, together with ileocecal resection was done, and the diagnosis was histologically confirmed. The relationship between this benign tumor and other neurogenic disorders is discussed, along with a review of the literature. Long-term follow up of this patient is required because of the possible development of von Recklinghausen's disease or multiple endocrine neoplasia type IIb.
Type of Medium:
Electronic Resource
URL:
http://dx.doi.org/10.1007/BF02389528
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