Meyer Andreas, Stark Michael, Karstens Johann H, Christiansen Hans, Bruns Frank
Department of Radiation Oncology, Hannover Medical School, Carl-Neuberg-Street 1, 30625 Hannover, Germany.
Case Rep Oncol Med. 2012;2012:789640. doi: 10.1155/2012/789640. Epub 2012 Aug 16.
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.
引言。朗格汉斯细胞组织细胞增多症（LCH）是一种病因不明的罕见疾病，具有不同的临床特征。目前尚未确立标准化治疗方案。病例报告。我们报告一例28岁患者，最初表现为第五颅神经感觉减退和左耳疼痛。诊断显示肿瘤位于颅底，最大直径为4.1厘米。经活检组织学确诊为LCH。诊断性检查证实该颅部病变是LCH的唯一表现。给予总剂量9 Gy（单次剂量1.8 Gy）。放疗后6个月内症状完全消失；重复的CT和MRI扫描显示病变大小缩小，骨质再矿化。随访13年后，患者无症状，无复发，也无治疗的任何副作用。讨论。由于该疾病病程缓慢，自发缓解率高，治疗选择很大程度上取决于个体临床情况。在本病例中，低剂量放疗足以在关键结构和组织区域实现长期局部控制。
