M Alahmad Yaman, Mukdad Omar Al, Huneity Ahmad, Sayed Sarah, Adam Renan, Al-Taie Alaa
Radiology Department, Hamad Medical Corporation, Doha, Qatar.
Pathology Department, Hamad Medical Corporation, Doha, Qatar.
Radiol Case Rep. 2024 Dec 11;20(3):1289-1293. doi: 10.1016/j.radcr.2024.11.069. eCollection 2025 Mar.
Langerhans cell histiocytosis (LCH) is a rare disorder, especially among adults, characterized by abnormal accumulation of dendritic histiocytes in various tissues, presenting as either single- or multi-system disease. In adults, spinal involvement is less common than long bone, while central nervous system manifestations, such as pituitary gland enlargement and stalk thickening, affect about a quarter of adult patients and may lead to significant endocrine disorders. Salivary gland involvement is another extremely rare manifestation of LCH. We report a 46-year-old lady who presented with back pain along with sensory and motor deficits in the lower limbs. Her blood pressure was elevated. Computed tomography angiography spotted a T6 vertebral body compression fracture. Magnetic resonance of the spine showed T6 vertebral body pathological collapse with epidural soft tissue component compressing the spinal cord. Other imaging modalities showed features suggestive of central nervous system and salivary gland involvement. She underwent T4-T8 pedicle screws fixation with T6 decompressive laminectomy and biopsy of the epidural lesion, which revealed histopathological features of LCH. This is an extremely rare case of adult-onset LCH with multi-system involvement: musculoskeletal, salivary and central nervous systems. This case could serve as a crucial reference for both clinicians, radiologists and researchers, enriching the existing knowledge base on adult-onset LCH.
朗格汉斯细胞组织细胞增多症（LCH）是一种罕见疾病，在成年人中尤为少见，其特征是树突状组织细胞在各种组织中异常积聚，可表现为单系统或多系统疾病。在成年人中，脊柱受累不如长骨常见，而中枢神经系统表现，如垂体增大和垂体柄增粗，影响约四分之一的成年患者，并可能导致严重的内分泌紊乱。唾液腺受累是LCH的另一种极其罕见的表现。我们报告一位46岁女性，她出现背痛并伴有下肢感觉和运动功能障碍。她血压升高。计算机断层血管造影发现T6椎体压缩性骨折。脊柱磁共振成像显示T6椎体病理性塌陷，硬膜外软组织成分压迫脊髓。其他影像学检查显示有提示中枢神经系统和唾液腺受累的特征。她接受了T4 - T8椎弓根螺钉固定、T6减压椎板切除术及硬膜外病变活检，活检显示为LCH的组织病理学特征。这是一例极其罕见的成年起病的多系统受累的LCH病例，累及肌肉骨骼、唾液腺和中枢神经系统。该病例可为临床医生、放射科医生和研究人员提供重要参考，丰富现有的成年起病LCH的知识库。
