Endocrine Abstracts

Introduction: Langerhans cell histiocytosis (LCH) is a proliferative disease that origins from dentritic cell family. LCH most commonly affects bones and skin, but it can also involve the other systems and organs. Lytic bone lesions are the most common findings in LCH, LCH can involve any bone; the most common sites are skull and femur. In rare case, lytic osseous lesions occur in the spine. Diabetes insipidus (DI) is the most frequent endocrine abnormality associated with LCH and it can occur prior to, concomitant with or after the diagnosis of LCH. Here, we report a rare case of LCH at spinal vertebra in an adult patient with present neurological deficits.

Case Report: A 33-year-old woman presented with 3 months history of back pain, weakness in the lower extremities and unable to walk for 2 days. She had no history of fever, weight loss. The patient’s medical history was unremarkable for trauma or other bone diseases. Three years before admission, the patient was diagnosed with central DI and desmopressin treatment was started. The etiologic cause of DI could not be found. On physical examinations, she rated the back pain as visual analogue scale 8 and localized tenderness over the C7-T1 spinous process was showed. Muscle strength in lower limbs was slightly decreased (3-4/5). Pathological reflexes were negative for both legs. And there were no sensory abnormalities. Her body temperature was normal, and there was no inflammatory focus in other systems. Laboratory tests including blood cell count, serum electrolytes, renal and liver function tests, erythrocyte sedimentation rate, and C-reactive protein did not reveal any abnormalities. Computed tomography showed a defined osteolytic lesion involving the the vertebral body C7, T1 and L2. Magnetic resonance imaging revealed a continuous enhancing epidural lesion located C7-T1 level with spinal cord compression. Because of motor weakness, and cord compression, the patient underwent posterior laminectomy. Histopathological examination showed that neoplastic cells which were positive for S-100, CD 68 and CD-1a. These features confirmed the diagnosis of LCH. After surgery, the patient showed a progressive improvement in motor function. The patient underwent chemotherapy with prednisolone and vinblastine.

Conclusion: We reported a rare case of adult spinal LCH with epidural involvement and neurological compression. The patient was treated succesfully vertebral resection and chemotherapy. Although extremely rare, LCH should be considered when there are epidural lesions with spinal cord compression in adults and it can be seen years after DI diagnosis.