Hematological neoplasms with first presentation as spinal cord compression syndromes: a 10-year retrospective series and review of the literature

Abstract

Context: The epidural space is a well-known, albeit uncommon, location for lymphomatous involvement, estimated to occur in less than 3% of all systemic lymphomas. Initial presentation of the patient with disease in this site (i.e. primary spinal epidural lymphoma) has been considered to be "rare". When it has been reported, many studies have emphasized the occurrence of aggressive lymphomas with a poor prognosis.

Design: A 10-year retrospective search of our files generated 7 patients who presented initially with back pain, incontinence and/or lower extremity weakness, and by neuroimaging studies were found to have masses causing spinal cord compression syndromes necessitating neurosurgical intervention.

Results: The 7 patients included 4 males and 3 females with thoracic and lumbar epidural masses. Tumor types included high-grade non-Hodgkin lymphoma, B cell type (n = 4), indolent B cell lymphoma (n = 1), nodular sclerosing Hodgkin lymphoma (n = 1) and plasmacytoma (n = 1). Advanced disease (stage 4) was subsequently identified in all 7 patients. Despite this, survival varied greatly with therapy, from 3 weeks to almost 6 years, underscoring the need for correct classification of the lymphoma in order to optimize chemotherapeutic choices. The epidural space was the site of presentation of disease in 4% of all lymphomas diagnosed at our institution.

Conclusions: Combining all reports in the literature, epidural presentation of lymphoma is not rare. Epidural lymphomas are distinct from both primary central nervous system lymphomas and from primary dural lymphomas. A broad range of systemic hematological tumor types can present as epidural masses. A full work-up for lymphoma classification may only be possible from the tissues received at the time of the neurosurgical decompression or biopsy procedure.