Langerhans cell histiocytosis in adults is associated with a high prevalence of hematologic and solid malignancies Journal Article
| Authors: | Ma, J.; Laird, J. H.; Chau, K. W.; Chelius, M. R.; Lok, B. H.; Yahalom, J. |
| Article Title: | Langerhans cell histiocytosis in adults is associated with a high prevalence of hematologic and solid malignancies |
| Abstract: | 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. © 2018 The Authors. Cancer Medicine published by John Wiley & Sons Ltd. |
| Keywords: | histiocytosis; eosinophilic granuloma; langerhans cell histiocytosis; langerhans; second malignancies |
| Journal Title: | Cancer Medicine |
| Volume: | 8 |
| Issue: | 1 |
| ISSN: | 2045-7634 |
| Publisher: | Wiley Blackwell |
| Date Published: | 2019-01-01 |
| Start Page: | 58 |
| End Page: | 66 |
| Language: | English |
| DOI: | 10.1002/cam4.1844 |
| PUBMED: | 30597769 |
| PROVIDER: | scopus |
| PMCID: | PMC6346231 |
| DOI/URL: | |
| Notes: | Article -- Export Date: 3 June 2019 -- Source: Scopus |
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