Abstract

Langerhans cell histiocytosis (LCH) is a very rare disease of female genital tract, most commonly seen in vulva and unusual in postmenopausal period. Herein, we report the 8th case of pure vulvar LCH in a postmenopausal woman. We pay attention to the differential diagnosis in postmenopausal state, features of pathologic diagnosis, and treatment options.

Highlights

  • Langerhans cell histiocytosis (LCH) is characterized by clonal neoplastic proliferation of bone morrow derived Langerhans cells in various tissues [1]

  • The patient was informed about the treatment options, and she was offered surgical treatment combined with radiotherapy according to the Gynecologic Oncology Council opinion

  • We reported a very rare pure vulvar LCH in a postmenopausal woman

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Summary

Introduction

Langerhans cell histiocytosis (LCH) is characterized by clonal neoplastic proliferation of bone morrow derived Langerhans cells in various tissues [1]. LCH is previously known as histiocytosis X, which includes a broad spectrum of clinical manifestations named eosinophilic granuloma, Hand-SchullerChristian disease, and Letterer-Siwe disease, depending on the involved tissues [1]. The most commonly involved tissues include bone, skin, lymph nodes, brain, and lungs. It is hard to recognize and diagnose LCH of the vulva both for the clinician and the pathologist. For this reason, we want to call attention to the diagnosis of LCH by reporting a case of pure vulvar LCH in a postmenopausal woman

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