Mondal Dodul, Julka P K, Jana Manisha, Walia Ritika, Chaudhuri Tamojit
Department of Radiation Oncology, DRBRAIRCH, All India Institute of Medical Sciences, New Delhi, India.
Department of Radiodiagnosis, DRBRAIRCH, All India Institute of Medical Sciences, New Delhi, India.
Ann Indian Acad Neurol. 2014 Oct;17(4):429-32. doi: 10.4103/0972-2327.144022.
Langerhans cell histiocytosis (LCH) is a disorder of clonal proliferation of dendritic cell mainly occurring in children. Spine involvement is rare. This usually presents with pain and torticollis when neck is involved. Histopathology with immunohistochemistry is confirmatory. Local curative therapy with excision or curettage is used for localized disease. Radiotherapy is usually reserved for selected cases. Systemic chemotherapy is the treatment of choice for widespread systemic disease. In this article, we present an unusual presentation of atlantoaxial LCH with mastoid involvement resulting in hearing loss as the first symptom and quadruparesis in a middle aged male patient, which was also associated with soft-tissue mass at the nape of the neck and deafness. The patient was treated with radical radiotherapy, which provided excellent response to the disease. Involvement of atlantoaxial joint and temporal bone associated with soft-tissue mass neck and deafness in a middle-aged man is an extremely rare clinical situation.
朗格汉斯细胞组织细胞增多症（LCH）是一种主要发生于儿童的树突状细胞克隆增殖性疾病。脊柱受累罕见。当累及颈部时，通常表现为疼痛和斜颈。组织病理学检查及免疫组化可确诊。局限性疾病采用局部切除或刮除的根治性治疗。放疗通常用于特定病例。全身化疗是广泛系统性疾病的首选治疗方法。在本文中，我们报告了一例不寻常的寰枢椎LCH病例，该病例累及乳突，导致一名中年男性患者以听力丧失为首发症状，并出现四肢瘫痪，同时伴有颈后部软组织肿块和耳聋。该患者接受了根治性放疗，对疾病产生了极佳的反应。中年男性寰枢关节和颞骨受累并伴有颈部软组织肿块和耳聋是一种极其罕见的临床情况。
