Non-Hodgkin's lymphoma presenting with spinal epidural involvement

Abstract

Cord compression was noted at presentation in 10 of 453 (2.2%) previously untreated non-Hodgkin's lymphoma patients seen at the Northern Israel Oncology Center between 1968 and 1983. A prodromal phase of local back pain occurred in eight patients, persisting up to 1 year, followed by a second phase of rapidly progressive signs of cord compression. Five of the ten patients presented with primary spinal epidural involvement (Stage IE), whereas the others had Stage IIE and IIIE (one patient each) and Stage IV, with bone and bone marrow involvement (three patients). All patients had unfavorable histologic diagnoses, mostly of the intermediate grade malignancy types according to the Working Formulation. The patients were treated by radiotherapy (two patients), chemotherapy (three patients), or both modalities (five patients). Seven of the ten patients achieved complete remission, but four of them have subsequently had relapses (two patients in bone, one in central nervous system, and one in mediastinum). The 5-year actuarial survival and 3-year relapse-free survival were 66% and 32%, respectively. Median survival has not been reached after a mean follow-up of 34 months. Non-Hodgkin's lymphoma with spinal epidural involvement at presentation is an aggressive disease. An intensive treatment combining irradiation with chemotherapy, and surgery as needed, is suggested in order to achieve good local response and long-term survival.

Primary Epidural non-Hodgkin's Lymphoma in Clinical Stage IEA Presenting with Paraplegia and Showing Complete Recovery after Combination Therapy.

“…Primary epidural non-Hodgkin's lymphoma has been reported to affect older individuals and to predominantly have a B cell phenotype. The most prevalent primary site is the thoracic spine (6,9). The present patient had all of these characteristics.…”

“…The present patient had all of these characteristics. The proper management of patients with primary epidural non-Hodgkin's lymphoma is not yet certain and the value of laminectomy is unclear because non Hodgkin's lymphoma is a very radio-and chemo-sensitive Primary Epidural Non-Hodgkin's Lymphoma tumor (9). In this patient, laminectomy was performed to treat rapidly progressive paraplegia and to obtain a diagnosis, because of the lack of other sites of involve ment.…”

“…In this patient, laminectomy was performed to treat rapidly progressive paraplegia and to obtain a diagnosis, because of the lack of other sites of involve ment. Epelbaum et al have stated that when it is possible to obtain a tissue specimen from a site other than the spine and there is no rapidly progressive severe neurologic impairment, treatment based mainly upon intensive chemotherapy and irradiation may be sufficient (9). Recently, it was also suggested that chemotherapy alone may be a sufficient treatment for this disease (10).…”

Stage IV Hodgkin's Disease presenting with spinal epidural involvement and cauda equina compression as the initial manifestation: Case report

“…1,5 The incidence of spinal cord compression at presentation (0.8%) in HD is much lower than that described in newly diagnosed NHL patients, where cord compression without any preceding sign of disease accounts for 1.5 ± 2.2% of cases. 6,7 In the big series of Yale, consisting of more than 600 HD patients, only one instance of HD presenting with epidural disease and spinal cord compression as the only apparent site of initial clinical involvement was described. 8 The regions most commonly a ected by spinal HD involvement are the upper thoracic and lumbar spine.…”

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“…9 Two phases of clinical presentation have been described: in the first prodromal phase, they suffer from local pain in the back, sometimes accompanied by radicular pain to legs and abdomen, and this pain persisted for several months to 1 year; then the phase of spinal cord compression followed and, within 2 to 8 weeks, the rapidly developing signs of compression. 10 Based on these clinical manifestations, our case was in the second phase, which can lead us to think that the beginning of the pathology was at least 1 year ago.…”