Adult Langerhans Cell Histiocytosis: A Rare Etiology of Spinal Cord Compression

Abstract

Langerhans cell histiocytosis is a rare disease involving clonal proliferation of langerhans cells seen in children and young adults. Clinical presentation is variable, ranging from a single location in the bone to severe multivisceral involvement. Moreover, spinal involvement causing myelopathy is even rare and unusual. We report a rare case of adult Langerhans cell histiocytosis in the dorsal spine causing a spinal cord compression associated with a pulmonary process treated by surgery, radiotherapy and systemic therapy with good evolution.

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

“…In adults, spinal involvement is very rare. Only few cases were reported in the literature [ 5 , 6 ]. Thoracic spine is the most common location for vertebral LCH, followed by lumbar and cervical spines [ 5 ].…”

“…Asymptomatic cases have been reported [ 1 ]. Low back pain was the only presentation in other cases, and a wide variation of neurological deficits (e.g., sensory, motor, bowel, or bladder dysfunction) was described in several reported cases [ 2 , 5 , 6 , 9 ]. The treatment and prognosis also vary among the cases [ 10 ].…”

“…Kouhen et al [ 6 ] have also reported a case of spinal thoracic LCH in an adult 25-year-old male with a long vertebral and epidural lesion extending from T1 to T5. The lesion was symptomatic causing paraparesis, bowel and bladder dysfunction, and sensory deficits [ 6 ].…”

“…Kouhen et al [ 6 ] have also reported a case of spinal thoracic LCH in an adult 25-year-old male with a long vertebral and epidural lesion extending from T1 to T5. The lesion was symptomatic causing paraparesis, bowel and bladder dysfunction, and sensory deficits [ 6 ]. This patient had evidence of systemic involvement in the form of asymptomatic lung LCH, discovered accidently during work-up [ 6 ].…”

“…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.…”