Management of adult primary vulvar Langerhans cell histiocytosis: review of the literature and a case history.

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.