Langerhans Cell Histiocytosis of the Cranial Base : Is Low-Dose Radiotherapy Effective?

Abstract EN

Introduction.

Langerhans cell histiocytosis (LCH) is a rare disease of unknown etiology with different clinical features.

A standardised treatment has not been established so far.

Case Report.

We report a case of a 28-year-old patient who initially presented with hypesthesia of the fifth cranial nerve and pain of the left ear.

Diagnosis showed a tumour localised in the cranial base with a maximum diameter of 4.1 cm.

The diagnosis of LCH was confirmed histologically by biopsy.

Diagnostic workup verified the cranial lesion as the sole manifestation of LCH.

A total dose of 9 Gy (single dose 1.8 Gy) was delivered.

The symptoms dissolved completely within 6 months after radiation; repeated CT and MRI scans revealed a reduction in size of the lesion and a remineralisation of the bone.

After a followup of 13 years the patient remains free of symptoms without relapse or any side effects from therapy.

Discussion.

Due to the indolent course of the disease with a high rate of spontaneous remissions the choice of treatment strongly depends on the individual clinical situation.

In the presented case low-dose radiotherapy was sufficient to obtain long-term local control in a region with critical structures and tissues.