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Management of adult primary vulvar Langerhans cell histiocytosis: review of the literature and a case history.
MedLine Citation:
PMID:  22464205     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
Primary vulvar Langerhans cell histiocytosis (LCH) is extremely rare and there are no standard treatment options. This review of the published literature with a case report aimed to clarify the optimal treatment for patients with this condition. Medline and PubMed were searched and all cases of primary vulvar LCH reported as single case reports or small case series were reviewed. A patient with vulvar LCH treated in this department is also reported. Twenty-seven cases, including the reported case, were reviewed. First-line treatments included surgery, radiotherapy, chemotherapy, thalidomide and local treatment. The mean follow-up time was 21.1±17.7 months. Although no patient died from the disease, recurrence rates were high (62%) and the mean time to relapse was 10.9±11.8 months (range 1-36 months). Treatment with thalidomide was successful, resulting in long-lasting remission. Disease recurrence is likely after surgery and or radiotherapy, and these treatments together with chemotherapy affect the patient's wellbeing adversely. Although definitive conclusions await further work, thalidomide has minimal adverse effects, is easy to administer and should be considered as a first-line treatment or as maintenance therapy in some patients.
Authors:
Samer El-Safadi; Thomas Dreyer; Frank Oehmke; Karsten Muenstedt
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2012-3-29
Journal Detail:
Title:  European journal of obstetrics, gynecology, and reproductive biology     Volume:  -     ISSN:  1872-7654     ISO Abbreviation:  -     Publication Date:  2012 Mar 
Date Detail:
Created Date:  2012-4-2     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0375672     Medline TA:  Eur J Obstet Gynecol Reprod Biol     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Copyright Information:
Copyright © 2012 Elsevier Ireland Ltd. All rights reserved.
Affiliation:
Department of Gynaecology and Obstetrics, University Hospital Giessen and Marburg GmbH, Giessen, Klinikstrasse 33, D-35392 Giessen, Germany.
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