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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MSK Authors
  1. Joachim Yahalom
    423 Yahalom
  2. Benjamin H Lok
    55 Lok
  3. Jennifer Ma
    34 Ma
  4. Monica Rose Chelius
    12 Chelius
  5. James   Laird
    13 Laird
  6. Karen Chau
    20 Chau