Incidence,Clinical Features,and Outcomes of Langerhans Cell Sarcoma in the United States |
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| Institution: | 1. Department of Internal Medicine, University of South Carolina School of Medicine, Columbia, SC;2. Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN;3. Division of Hematology, Mayo Clinic, Rochester, MN;1. Department of Radiation Oncology, Houston Methodist Hospital, Houston, Texas;2. Department of Radiation Oncology, University of Nebraska Medical Center, Omaha, Nebraska;3. Department of Radiation Oncology, University of Texas Health Science Center San Antonio, San Antonio, Texas;4. Department of Radiation Oncology, Houston Methodist Hospital, Houston, Texas;6. Department of Radiation Oncology, University of Maryland Medical Center, Baltimore, Maryland;1. Department of Medical Oncology, Mayo Clinic, Rochester, MN, United States;2. Department of Internal Medicine, University of South Carolina School of Medicine, Columbia, SC, United States;3. Department of Gastrointestinal Oncology, H. Lee Moffitt Cancer Center, Tampa, FL, United States;1. Department of Medicine, School of Medicine, University of South Carolina, Columbia, SC, USA;2. Department of Oncology, H Lee Moffitt Cancer Center & Research Institute, Tampa, FL, USA;3. Department of Oncology, Mayo Clinic, Scottsdale, AZ, USA;4. Department of Oncology, Mayo Clinic, Rochester, MN, USA;1. Department of Medicine, University of South Carolina School of Medicine, Columbia, SC, USA;2. Department of Oncology, H. Lee Moffitt Cancer Center, Tampa, FL, USA;3. Department of Oncology, Mayo Clinic, Rochester, MN, USA;4. Section of Hepatobiliary and Pancreatic Surgery, Division of Subspecialty General Surgery, Department of Surgery, Mayo Clinic, Rochester, MN, USA;5. Department of Oncology, Mayo Clinic, Phoenix, AZ, USA;1. Division of Urology, Maine Medical Center, Portland, Maine;2. Center for Outcomes Research and Evaluation, Maine Medical Center, Portland, Maine;3. Tufts University School of Medicine, Boston, Massachusetts;4. Division of Urology and Center for Surgery and Public Health, Brigham and Women’s Hospital, Harvard Medical School, Boston, Massachusetts;5. Vattikutti Institute Center for Outcomes Research, Analytics and Evaluation, Henry Ford Health System, Detroit, Michigan;1. Department of Medicine, University of South Carolina School of Medicine, Columbia, SC, USA;2. Department of Oncology, Mayo Clinic, Rochester, MN, USA;3. Section of Hepatobiliary and Pancreatic Surgery, Division of Subspecialty General Surgery, Department of Surgery, Mayo Clinic, Rochester, MN, USA |
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| Abstract: | BackgroundLimited knowledge exists on the incidence, treatment patterns, and long-term outcomes of Langerhans cell sarcoma (LCS) in the United States.Patients and MethodsWe performed a retrospective study of LCS patients diagnosed between 2001 and 2014 using the Surveillance, Epidemiology, and End Results (SEER) and National Cancer Data Base (NCDB) databases. Incidence was calculated from SEER, and treatment patterns and outcomes were calculated from NCDB.ResultsA total of 25 and 52 cases of LCS were reported to SEER and NCDB, respectively. The overall incidence of the disease was 0.2 per 10,000,000 and did not differ by race (P = .56) or sex (P = .33). The median age at diagnosis was 62 (range, 19-90) years. Of the 52 patients from NCDB, 20 (39%) received chemotherapy as first-line therapy, 24 (46%) received surgery, and 15 (29%) received radiotherapy. The 1-year overall survival (OS) rate was 62%, and the median OS was 19 months. After censoring the patients with bone marrow and reticuloendothelial system involvement, no significant difference in OS was noted between the patients who were managed with or without surgery (P = .75). Postsurgical radiation or chemotherapy were not associated with improvement in median OS (P = .25). Patients who were managed with radiotherapy had a better OS compared to those who received no radiotherapy (P = .03).ConclusionThis dual-national registry study shows that LCS is extremely rare and has a poor prognosis. Radiotherapy may offer a survival advantage to patients with locoregional disease without bone marrow and reticuloendothelial system involvement. |
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| Keywords: | Histiocytes NCDB Overall survival Radiation SEER |
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