Recurrent multisystem Langerhans cell histiocytosis involving the female genitalia: A case report

Abstract

BACKGROUND Langerhans cell histiocytosis (LCH) is a histiocytic proliferative disease caused by clonal proliferation of Langerhans cells, which is currently defined as an inflammatory myeloid tumor. It is rare in adults, with an incidence of 1–2 per million, and is highly heterogeneous in clinical presentation, with unpredictable disease progression and outcome. CASE SUMMARY A 52-year-old postmenopausal female patient presented to the gynecology department in July 2023 with bilateral vulvar masses. She was diagnosed with recurrent multisystem LCH. The patient had previously been diagnosed with a single-system and single-focal LCH in October 2021 due to a right maxillofacial mass, which resolved after surgical treatment. A chemotherapy regimen was developed after multidisciplinary consultation. Six cycles of chemotherapy resulted in partial remission, and maintenance chemotherapy is currently being administered. CONCLUSION Recurrent LCH involving the bilateral vulva has been poorly reported. Comprehensive imaging and pathological evaluation is important for diagnosis. The model of joint multidisciplinary specialist diagnosis and treatment is worthy of clinical application.