The epidemiological and clinical characteristics of Langerhans cell sarcoma in the United States: A population study based on SEER data from 2000 to 2019

Abstract

Langerhans cell sarcoma (LCS) is a rare aggressive malignancy with a poor prognosis. Our knowledge about this condition is limited and mainly based on case reports, making it challenging to understand its epidemiology, clinical features, and patient outcomes. We conducted a retrospective study of LCS patients diagnosed between 2000 and 2019 using the Surveillance, Epidemiology, and End Results (SEER) database. The data were stratified based on age, race, stage, clinical pattern, and treatment method. Our study found that 57 LCS cases were reported in SEER registries between 2000 and 2019. Among these cases, most patients (50.9%) were over 60 years old and White (71.9%) with almost equal males to females ratio. About 45.6% of cases were localized while 47.4% were at distant stages. Of the patients, 50.9% underwent surgery, 45.6% received chemotherapy, and only 21.1% received radiotherapy. The overall survival rate for patients diagnosed with LCS in the United States is generally low with a 1-year overall rate of 63.8%. Certain factors can negatively impact prognosis, such as advanced stages of the disease, secondary tumors, or more than 1 tumor per patient. LCS is a rare disease with poor survival rates. Future research should incorporate global data for further statistically significant results. Moreover, investigating the molecular, genetic, and pathophysiological backgrounds of these tumors is crucial for developing targeted management strategies and improving prognosis.