Department of Radiation Oncology, Memorial Sloan Kettering Cancer Center, New York, New York.
Albert Einstein College of Medicine, Bronx, New York.
Cancer Med. 2019 Jan;8(1):58-66. doi: 10.1002/cam4.1844. Epub 2018 Dec 30.
Langerhans cell histiocytosis (LCH) is a rare disorder of histiocyte proliferation. Previous case studies suggest a higher prevalence of hematologic and solid malignancies among LCH patients, possibly due to treatment with tumorigenic agents such as etoposide. We report the first large, single-institution experience of adult LCH patients with additional malignancies to study the characteristics of these patients.
We identified 132 consecutive patients >18 years of age with histologically confirmed LCH at our center between 1990 and 2015. Demographics and detailed oncologic history were recorded to identify patients with additional malignancies.
Of 132 adult LCH patients, 42 (32%) patients had an additional malignancy. There were 53 malignancies among the 42 patients, with 31 (58%) preceding LCH diagnosis, 11 concurrent (≤3 months; 21%) with LCH diagnosis, and 11 (21%) after. Median age was 54 years (range 28-89) with a median follow-up of 3.7 years (0.1-22.2) for this cohort. OS at 3 years was 98% in patients with LCH alone and 82% among patients with additional malignancies, with 30 (71%) alive at last follow-up. Solid tumors, lymphomas, and other hematologic malignancies were observed as follows: 39 (74%), 9 (17%), and 5 (9%).
Our cohort of adult LCH patients demonstrates an unusually high number of additional malignancies. Our study includes predominantly malignancies diagnosed preceding or concurrent with LCH, suggesting a cause of malignancy independent of LCH treatment. Further exploration of the biology of this rare disease may elucidate the mechanism of frequent additional malignancies.
朗格汉斯细胞组织细胞增生症（LCH）是一种罕见的组织细胞增生性疾病。先前的病例研究表明，LCH 患者中血液系统和实体恶性肿瘤的患病率较高，这可能是由于使用依托泊苷等致瘤药物治疗所致。我们报告了首例大型单机构成人 LCH 患者合并其他恶性肿瘤的经验，以研究这些患者的特征。
我们在 1990 年至 2015 年间在我们的中心确定了 132 例经组织学证实的 LCH 大于 18 岁的连续患者。记录人口统计学和详细的肿瘤病史以确定患有其他恶性肿瘤的患者。
在 132 例成人 LCH 患者中，42 例（32％）患者患有其他恶性肿瘤。在这 42 名患者中，有 53 种恶性肿瘤，其中 31 种（58％）先于 LCH 诊断，11 种（21％）与 LCH 诊断同时发生，11 种（21％）在诊断后发生。该队列的中位年龄为 54 岁（范围 28-89 岁），中位随访时间为 3.7 年（0.1-22.2 年）。单独患有 LCH 的患者的 3 年 OS 为 98％，而患有其他恶性肿瘤的患者的 OS 为 82％，截至最后随访时，有 30 名（71％）患者存活。观察到的实体瘤、淋巴瘤和其他血液系统恶性肿瘤分别为：39 例（74％）、9 例（17％）和 5 例（9％）。
我们的成人 LCH 患者队列显示出异常高数量的其他恶性肿瘤。我们的研究主要包括在 LCH 之前或同时诊断出的恶性肿瘤，这表明恶性肿瘤的发生与 LCH 治疗无关。进一步探索这种罕见疾病的生物学可能会阐明频繁发生其他恶性肿瘤的机制。
