Spinal epidural involvement in adult Langerhans cell histiocytosis (LCH)

Abstract

Rationale: Spinal involvement in adult Langerhans cell histiocytosis (LCH) is rare, and epidural involvement is unusual. LCH is mostly indistinguishable from other spinal lesions such as infection, lymphoma, and metastasis. So, it could be easily misdiagnosed without suspicion. Patient concerns: We report a case of a 33-year-old man who complained of gait disturbance with weakness in both legs and severe back pain. Diagnoses: A continuous enhancing epidural lesion with cord compression from the T7 to L1 level was detected in magnetic resonance imaging. Laboratory analysis indicated the possibility of spinal infectious disease. We assumed that the lesion could be tuberculous spondylitis. Interventions and outcomes: The patient underwent posterior laminectomy with marginal excision of the epidural mass to relieve cord compression. Pathological examination confirmed the diagnosis of LCH. The 12-month follow-up evaluation revealed that the patient was neurologically intact and had no gait disturbance. Lessons: This case report presents a patient with epidural LCH of the thoracic spinal cord, which can mimic spinal infections such as tuberculous spondylitis with abscess formation. Therefore, LCH could be considered as a possible diagnosis when a patient presents with features of infectious spondylitis with vertebral involvement.

Multisystem Langerhans cell histiocytosis: Literature review and case report

“…Biopsy of the lesion was performed, and histological examination of the biopsy specimens verified vascular proliferation and the significant infiltration of CD1a-positive cells, suggesting a diagnosis of LCH. The patient was successfully treated with steroid therapy [17] . Montemurro et al.…”

Spinal Langerhans cell histiocytosis with cord compression and neurological deficits: A case report

“…In adults, the vast majority of cases with LCH (69 %) affect multiple systems, whereas only one third (31 %) of the cases involve a single system [ 5 ]. Lung is the most common organ affected in single system involved, constituting more than half of the cases (51 %) [ 5 ]. Bone and cutaneous involvement are the next most common systems involved in 38 % and 7 % of the cases respectively [ 5 ].…”

“…Lung is the most common organ affected in single system involved, constituting more than half of the cases (51 %) [ 5 ]. Bone and cutaneous involvement are the next most common systems involved in 38 % and 7 % of the cases respectively [ 5 ]. The most common bone locations affected are the skull, femur, and spine constituting 27 %, 15 %, and 6.5 %, respectively [ 5 ].…”

“…Bone and cutaneous involvement are the next most common systems involved in 38 % and 7 % of the cases respectively [ 5 ]. The most common bone locations affected are the skull, femur, and spine constituting 27 %, 15 %, and 6.5 %, respectively [ 5 ].…”

“…Spine LCH, despite being common in children, is very rare in adults, and only few adult cases with isolated spinal LCH were reported in the literature [ 5 , 6 ]. In this report, we present a rare case of thoracic spine LCH in an adult lady presenting symptomatic spinal cord compression and neurological deficits who was found to have asymptomatic pituitary and systemic involvement.…”