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首页> 外文期刊>Annals of Indian Academy of Neurology >Langerhans cell histiocytosis of atlantoaxial joint in a middle-aged man presenting with deafness as first symptom and soft-tissue mass at neck showing excellent response to radiotherapy alone: Report of an extremely rare and unusual clinical condition and review of literature
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Langerhans cell histiocytosis of atlantoaxial joint in a middle-aged man presenting with deafness as first symptom and soft-tissue mass at neck showing excellent response to radiotherapy alone: Report of an extremely rare and unusual clinical condition and review of literature

机译：表现为耳聋为首发症状，颈部软组织肿块的中年男子的寰枢关节朗格汉斯细胞组织细胞增生症，仅对放疗即可表现出极佳的疗效和罕见的临床表现：文献报道

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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.
机译：朗格汉斯细胞组织细胞增生症（LCH）是一种主要发生于儿童的树突状细胞的克隆增殖疾病。脊柱受累很少见。当涉及颈部时，通常会表现出疼痛和斜颈。免疫组织化学的组织病理学证实。局部切除术或刮除术可用于局部疾病。放射疗法通常仅适用于部分病例。全身化疗是广泛性全身疾病的首选治疗方法。在本文中，我们提出了一种不寻常的表现，即伴有乳突的寰枢椎LCH导致中年男性患者的听力下降为首发症状和四肢瘫痪，这也与颈项和耳聋处的软组织肿块有关。该患者接受了根治性放射治疗，对这种疾病提供了出色的反应。中年男子累及寰枢关节和颞骨并伴有软组织肿块颈部和耳聋，是一种极为罕见的临床情况。

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