Primary epidural lymphocyte-depleted Hodgkin’s lymphoma of the thoracic spine – presentation of a rare disease variant

Abstract

BackgroundLymphocyte-depleted Hodgkin’s lymphoma is the rarest form of classical Hodgkin’s lymphoma, accounting for < 1% of all cases. Patients often have advanced-stage disease at the time of presentation with an aggressive clinical course. Even more uncommon is primary extranodal disease and rarely it will be presenting with spinal cord compression.Case presentationAn 88-year-old Caucasian female presented with a history of upper back pain for several months and new onset bilateral leg numbness and weakness. MRI of the spine showed a dorsal epidural lesion with cord compression at T1-T4 with involvement of the paraspinal muscles. The patient received urgent surgical decompression, with final histopathology showing a lymphocyte-depleted Hodgkin’s lymphoma. Systemic work-up did not show evidence of nodal disease. Following surgery, she received a course of radiotherapy with good outcome.ConclusionTo the best of our knowledge, this is the first reported case of primary lymphocyte-depleted Hodgkin lymphoma presenting as epidural spinal cord compression. Our report, in conjunction with a review of the literature, suggests that surgical intervention is clearly indicated in de novo disease followed by radiotherapy.

Spinal Manifestation of Malignant Primary (PLB) and Secondary Bone Lymphoma (SLB)

“…On the other hand, Peng et al reported that six of eight patients fully recovered after surgical decompression regarding their neurological impairment [26]. Others who have reviewed their patient data have come to similar conclusions [9,11,[27][28][29][30][31].…”

Hodgkin Lymphoma revealed by epidural spinal cord compression

“…1,2 Extra-nodal involvement of the disease is estimated at 10% of cases. [1][2][3][4][5] Epidural HL leading to a spinal cord compression occurs in 5% of cases, but usually at an advanced stage of the disease: it reveals the diagnosis in only 0.2% of cases. 4,6,7 Pathophysiology Epidural mass in HL may originate from hematogenous dissemination or more likely develops by direct spread from retroperitoneal or thoracic lymph nodes.…”

“…Hodgkin's disease usually presents with painless cervical adenopathy which may be accompanied by weight loss, long-term fever and night sweats. 2,3 Spinal cord involvement owing to an epidural mass is a rare complication that usually develops in the setting of a diffuse, undiagnosed disease or some time after the initial diagnosis. Then, the disease manifests itself with back pain and neurological deficits depending on the level and location of the lesion.…”

“…Then, the disease manifests itself with back pain and neurological deficits depending on the level and location of the lesion. 3,8…”

“…The gold standard treatment of Hodgkin Lymphoma is chemotherapy, however in case of primary disease presenting with spinal cord compression, surgical debulking is indicated and allows for adequate tissue sampling for a histopathological diagnosis. 3,6,8 Radiotherapy is useful for further local control of the epidural and paraspinal spread. 6…”