Langerhans cell histiocytosis in adults is associated with a high prevalence of hematologic and solid malignancies

Background 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. Methods 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. Results 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%). Conclusion 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.