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Juvenile-like (inflammatory/hyperplastic) mucosal polyps of the gastrointestinal tract in neurofibromatosis type 1.
MedLine Citation:
PMID:  24219125     Owner:  NLM     Status:  Publisher    
BACKGROUND: Diffuse neurofibromatosis/ ganglioneuromatosis, solitary/ plexiform neurofibroma, periampullary carcinoids and gastrointestinal stromal tumour (GIST) are the main gastrointestinal manifestations of neurofibromatosis type 1 (NF-1, von Recklinghausen disease). Inflammatory (juvenile-like) polyps still have not been recognized as specific gastrointestinal manifestations of NF-1.
METHODS, PATIENTS AND RESULTS: We describe 4 males aged 23-65 years with NF-1 and inflammatory (juvenile-like) gastrointestinal polyps and review the literature for similar cases. Two patients had single polyps (sigmoid colon and antrum, respectively), 1 had two polyps (left colon) and 1 had 3 polyps (distal esophagus and colon). Histology revealed variable appearance ranging from juvenile-like to granulation tissue-rich predominantly inflammatory and hyperplastic. Three lesions showed obliterative vasculopathic changes. None had neurofibromatous or ganglioneuromatous polyps. Review of the literature disclosed 11 similar cases. Most patients presented with severe gastrointestinal symptoms and/or anemia.
CONCLUSION: NF-1-associated inflammatory polyps probably represent specific GI manifestations of this disorder and should be thought of, particularly in patients with GI symptoms. They should be distinguished from inflammatory fibroid polyps and from juvenile-like changes associated with ganglioneuroma/tosis and neurofibroma/tosis. Their etiology remains obscure but different mechanisms including NF-1 inactivation, NF-1-associated vasculopathy and localized mucosal prolapse/damage caused by motility disorders might be involved. This article is protected by copyright. All rights reserved.
Abbas Agaimy; Inga-Marie Schaefer; Leopoldina Kotzina; Jürgen Knolle; Irith Baumann; Philipp Ströbel; Michael Vieth
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2013-11-13
Journal Detail:
Title:  Histopathology     Volume:  -     ISSN:  1365-2559     ISO Abbreviation:  Histopathology     Publication Date:  2013 Nov 
Date Detail:
Created Date:  2013-11-13     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  7704136     Medline TA:  Histopathology     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Copyright Information:
This article is protected by copyright. All rights reserved.
Institute of Pathology, University Hospital, Erlangen, Germany.
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