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

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 ner...

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Main Authors: Andreas Meyer, Michael Stark, Johann H. Karstens, Hans Christiansen, Frank Bruns
Format: Article
Language:English
Published: Wiley 2012-01-01
Series:Case Reports in Oncological Medicine
Online Access:http://dx.doi.org/10.1155/2012/789640
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author Andreas Meyer
Michael Stark
Johann H. Karstens
Hans Christiansen
Frank Bruns
author_facet Andreas Meyer
Michael Stark
Johann H. Karstens
Hans Christiansen
Frank Bruns
author_sort Andreas Meyer
collection DOAJ
description 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.
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spelling doaj-art-18b35884538c45b0bc5c2871d00ae57f2025-02-03T06:07:27ZengWileyCase Reports in Oncological Medicine2090-67062090-67142012-01-01201210.1155/2012/789640789640Langerhans Cell Histiocytosis of the Cranial Base: Is Low-Dose Radiotherapy Effective?Andreas Meyer0Michael Stark1Johann H. Karstens2Hans Christiansen3Frank Bruns4Department of Radiation Oncology, Hannover Medical School, Carl-Neuberg-Street 1, 30625 Hannover, GermanyDepartment of Radiation Oncology, Hannover Medical School, Carl-Neuberg-Street 1, 30625 Hannover, GermanyDepartment of Radiation Oncology, Hannover Medical School, Carl-Neuberg-Street 1, 30625 Hannover, GermanyDepartment of Radiation Oncology, Hannover Medical School, Carl-Neuberg-Street 1, 30625 Hannover, GermanyDepartment of Radiation Oncology, Hannover Medical School, Carl-Neuberg-Street 1, 30625 Hannover, GermanyIntroduction. 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.http://dx.doi.org/10.1155/2012/789640
spellingShingle Andreas Meyer
Michael Stark
Johann H. Karstens
Hans Christiansen
Frank Bruns
Langerhans Cell Histiocytosis of the Cranial Base: Is Low-Dose Radiotherapy Effective?
Case Reports in Oncological Medicine
title Langerhans Cell Histiocytosis of the Cranial Base: Is Low-Dose Radiotherapy Effective?
title_full Langerhans Cell Histiocytosis of the Cranial Base: Is Low-Dose Radiotherapy Effective?
title_fullStr Langerhans Cell Histiocytosis of the Cranial Base: Is Low-Dose Radiotherapy Effective?
title_full_unstemmed Langerhans Cell Histiocytosis of the Cranial Base: Is Low-Dose Radiotherapy Effective?
title_short Langerhans Cell Histiocytosis of the Cranial Base: Is Low-Dose Radiotherapy Effective?
title_sort langerhans cell histiocytosis of the cranial base is low dose radiotherapy effective
url http://dx.doi.org/10.1155/2012/789640
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