Pathology, Classification, Clinical Manifestations and Prognosis of Langerhan’s Cell Histiocytosis: A Single Center Experience

Objective: The aim of the study is to raise awareness about clinical features, histopathological and radiological analyzes and treatment details of this rare disease. Methods: A total of 55 Langerhans cell histiocytosis patients, diagnosed between the years 2006 and October 2020 in our department were included in the study. The patients were evaluated in terms of age, gender, tumor localization, risk groups, treatment modalities, recurrence, and disease outcome. Results: Twenty-three out of 55 patients were children and 32 were adults. The ages of the patients were between 7 months and 72 years. Thirty-seven of the cases were male and 18 were female. The most common clinical complaint in both groups was pain and swelling. The duration between the onset of the patient complaints and admission to the hospital varies between 7 days-12 months in children, and 10 days-23 years in adults. Forty-three of the cases had single-organ involvement and 12 had multiorgan involvement. The most frequently affected organ in both groups was bone. Forty of the 55 patients had follow-up data and the treatment modalities are as follows: Nine patients radiotherapy, 8 patients chemotherapy+steroid, 7 patients chemotherapy, 2 patients chemotherapy+radiotherapy+steroid, 1 patient steroid, and 2 patients chemotherapy+radiotherapy. Eleven patients were followed up without additional treatment after surgery. Median follow-up from the time of biopsy was 45.9 months in children and 41.9 months in adults. Conclusions: As a result, diagnosis requires a high degree of suspicion and final diagnosis is based on the histological examination of the lesions and biopsies