Langerhans cell histiocytosis of the female genital tract: A literature review

A. J. Montero, C. M. Díaz-Montero, A. Malpica, P. T. Ramirez, John J. Kavanagh

Research output: Contribution to journalReview articlepeer-review

26 Scopus citations


Langerhans cell histiocytosis (LCH) is a rare malignant disease involving the accumulation of a monoclonal proliferation of cells in various organs, that phenotypically resemble Langerhans cells (LC). LCH is not merely a hyperplasia of LC, as it typically affects organs that are outside of their normal physiologic distribution. Normal Langerhans cells are bone marrow-derived dendritic cells that populate the epidermis and are distinguished by the presence of Birbeck granules and cell surface protein CD1a. LC act as sentinels; they recognize, internalize, and process antigens encountered in the skin. Upon encountering an antigen, LC become activated with subsequent maturation and induction of their migratory capacity. Langerhans cells in patients with LCH are aberrant and profoundly differ from normal LC. The clinical spectrum of LCH is quite diverse; multiple organs can be affected. "Pure" genital Langerhans cell histiocytosis is a rare presentation, with only 12 previously reported cases. Due to the rarity of this disease, treatment of genital LCH is still very diverse. No modality is proven to be superior in improving patient outcome, and relapses frequently occur after surgery. Dramatic responses of cutaneous and ano-genital lesions to thalidomide and interferons have been reported. We advocate the use of immuno-modulating agents in LCH of the female genital tract first, rather than surgery.

Original languageEnglish (US)
Pages (from-to)381-388
Number of pages8
JournalInternational Journal of Gynecological Cancer
Issue number3
StatePublished - May 2003


  • Dendritic cell
  • Female genital tract
  • Histiocytosis
  • LCH
  • Thalidomide

ASJC Scopus subject areas

  • Oncology
  • Obstetrics and Gynecology


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