Yuan Chun-Yan, Zhang Zhi-Rong, Guo Ming-Fang, Zhang Na
Department of Gynaecology, Fengdu People's Hospital, Chongqing 408200, China.
Department of Gynecologic Oncology, Chongqing Key Laboratory of Translational Research for Cancer Metastasis and Individualized Treatment, Chongqing University Cancer Hospital, Chongqing Cancer Institute, Chongqing Cancer Hospital, Chongqing 400030, China.
World J Clin Cases. 2024 Oct 6;12(28):6222-6229. doi: 10.12998/wjcc.v12.i28.6222.
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.
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.
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.
朗格汉斯细胞组织细胞增多症（LCH）是一种由朗格汉斯细胞克隆性增殖引起的组织细胞增生性疾病，目前被定义为一种炎症性髓系肿瘤。它在成人中罕见，发病率为百万分之一至二，临床表现高度异质性，疾病进展和预后不可预测。
一名52岁绝经后女性患者于2023年7月因双侧外阴肿物就诊于妇科。她被诊断为复发性多系统LCH。该患者曾在2021年10月因右颌面肿物被诊断为单系统单灶性LCH，手术治疗后肿物消退。多学科会诊后制定了化疗方案。六个周期的化疗导致部分缓解，目前正在进行维持化疗。
双侧外阴复发性LCH的报道较少。综合影像学和病理评估对诊断很重要。多学科专家联合诊断和治疗模式值得临床应用。
