Abstract

INTRODUCTION

In this study, our aim was to analyse and discuss epidemiology, clinical and radiologic features, differential diagnosis, histopathologic characteristics, key elements of treatment strategies and follow-up results of Langerhans cell histiocytosis (LCH) in a 29-patient clinical series.

METHODS

All the patients who had been operated for multiple or solitary calvarial LCH between 2001-2018 in our clinic were included in our study. Patient discharge notes, radiologic workup, operation records, patholoy reports and follow-up data were retrospectively evaluated. Age, sex, location and size of the lesion; radiologic features, extent of resection, histopathologic features; adjuvant treatment, recurrence, reoperation and follow-up data were collected.

RESULTS

In total 29 patients (15 male, 14 female) were evaluated. Except one, all patients (97%) were under 18 years of age. Patients’ mean age at the time of diagnosis was 8.4±7.7 years (median 8; range 1-38). Most of the patients admitted with subdermal/palpable mass. In 5 patients there were multiple calvarial and/or other organ involvement. The most common location was parietal bone. In 3 patients with multiple lesions, only biopsy was performed; other patients undergone total extirpation of the primary/solitary lesion. Patients with multiple lesions and other organ involvement had chemotherapy. Most of the patients had a remission whereas two patients developed progression of residue in the follow-up period.

DISCUSSION AND CONCLUSION

Langerhans cell histiocytosis is a benign and rare calvarial lesion, in which total surgical excision is an effective and succesful treatmet. A thorough evaluation of a patient with suspected LCH is needed for differential diagnosis and in order to identify extracalvarial lesions. In cases where total excision is not possible and for patients with multiple and/or skull base involvement chemotherapeutics may be used.