Langerhans cell histiocytosis of the female genital tract: a literature review with additional three case studies in China.

PURPOSE: To summarize the clinical and pathological features of all reported Langerhans cell histiocytosis (LCH) cases of female genital tract and to provide our additional three cases, and the emphasis is put on the pathological diagnosis to remind both gynecologists and pathologists of this rare disease.

METHODS

Literatures available are reviewed and the routine hematoxylin and eosin stained sections and immunohistochemical stained sections are studied for the diagnosis of our cases.

RESULTS

Only 18 female genital tract LCH cases have been previously reported in medical literatures. The three cases we provide are diagnosed as LCH by both morphology and immunochemical staining after biopsy. All the patients presented papulous or ulcerative lesions on vulva or cervix, and the following systemic laboratory and radiologic examinations demonstrated no other affected site. After various treatments based on surgery and chemotherapy, most patients showed no signs of local or systemic recurrence.

CONCLUSIONS

'Pure' LCH of female genital tract without any other spreading is a quite rare disease and it might be misrecognized as some other diseases, so both gynecologists and pathologists should keep it in mind when encountered with such cases. The three patients we reported appeared to be the first cases having 'pure' lesion of female genital tract in China.