Schwartz Tyler R, Elliott Leighton A, Fenley Heather, Ramdas Jagadeesh, Scott Greene Joseph
Department of Otolaryngology-Head and Neck Surgery, Geisinger Medical Center, Danville, PA, USA.
Division of Hematology/Oncology, University of Florida Department of Medicine, Gainesville, FL, USA.
Ann Otol Rhinol Laryngol. 2023 Apr;132(4):431-439. doi: 10.1177/00034894221098466. Epub 2022 Jun 3.
Retrospectively analyze head and neck Langerhans Cell Histiocytosis at a rural tertiary referral center and compare results with previously published data.
Electronic health record review was performed from 2003 to 2019. Patients with biopsy proven LCH with primary head and neck involvement were included. Demographics, presentation, imaging characteristics, treatment modality, delay in diagnosis (DD, ≥60 days), and outcomes were analyzed and reported.
Twenty-four patients were included. The most common presenting symptoms were otorrhea (n = 6) and scalp pain or swelling (n = 6). All patients had bony involvement. The most common site was facial or skull lesions (n = 20). Most skull lesions (75%) demonstrated CNS risk. Six patients were treated with primary surgery, 15 with primary chemotherapy, and 3 with surgery plus adjuvant chemotherapy. Nine patients experienced relapse of disease with median time to documented relapse of 11.4 months; all were treated with salvage chemotherapy to achieve complete remission (median follow-up: 72 months). Patients most likely to relapse were those with multisystem disease (5/7, 71.4%), temporal bone lesions (4/7, 57.1%), and DD (7/12, 58.3%). Of the 9 total patients who experienced relapse, 78% had a delay in diagnosis.
LCH is a complex disease process in which diagnosis can be delayed if not considered in the differential. Within the head and neck, the skull, including isolated temporal bone involvement, is the most common site of involvement. Treatment modality does not appear to have an influence on relapse rates. Relapse was more likely to occur in the first year after treatment and close monitoring is required.
回顾性分析一家农村三级转诊中心的头颈部朗格汉斯细胞组织细胞增多症，并将结果与先前发表的数据进行比较。
对2003年至2019年的电子健康记录进行回顾。纳入经活检证实为原发性头颈部受累的朗格汉斯细胞组织细胞增多症患者。分析并报告患者的人口统计学资料、临床表现、影像学特征、治疗方式、诊断延迟（DD，≥60天）及预后情况。
共纳入24例患者。最常见的临床表现为耳漏（n = 6）和头皮疼痛或肿胀（n = 6）。所有患者均有骨质受累。最常见的部位是面部或颅骨病变（n = 20）。大多数颅骨病变（75%）显示有中枢神经系统受累风险。6例患者接受了一期手术治疗，15例接受了一期化疗，3例接受了手术加辅助化疗。9例患者疾病复发，记录到的复发中位时间为11.4个月；所有患者均接受挽救性化疗以实现完全缓解（中位随访时间：72个月）。最易复发的患者是患有多系统疾病的患者（5/7，71.4%）、颞骨病变患者（4/7，57.1%）以及诊断延迟的患者（7/12，58.3%）。在总共9例复发患者中，78%存在诊断延迟。
朗格汉斯细胞组织细胞增多症是一个复杂的疾病过程，如果在鉴别诊断中未被考虑，诊断可能会延迟。在头颈部，颅骨，包括孤立的颞骨受累，是最常见的受累部位。治疗方式似乎对复发率没有影响。复发更有可能在治疗后的第一年发生，需要密切监测。
