Non-Hodgkin Lymphoma in a Child Presenting with Acute Paraplegia: A Case Report

Abstract

Spinal cord compression is a rare presentation of non-Hodgkin lymphoma. Extradural location at onset is a rare but devastating event in pediatric oncology. The authors describe a girl with acute spinal cord compression due to epidural non-Hodgkin lymphoma, emphasizing the encouraging perspective for a complete recovery in children with this condition. A 5-year-old girl presented with pain followed by progressive hyposthenia and paraplegia after a trauma. CT scan and MRI showed homogeneous tissue extending from T2 to L4, occupying the entire vertebral canal and extending to the para- and peri-vertebral soft parts. Emergency surgical debulking was carried out through T6-L1 laminectomy. The patient began chemotherapy (LMB 89 Protocol) and the tumor quickly disappeared. The patient is maintaining a complete remission 42 months after diagnosis. Significant results may be obtained with the chemotherapy treatment of epidural non-Hodgkin lymphoma when the disease is promptly diagnosed. Considering the effectiveness of chemotherapy, the authors believe that a neuro-surgical approach should be employed only when rapid worsening of symptoms is observed or for diagnostic purpose.

Primary dural lymphoma: A novel concept of heterogeneous disease

“…The majority of the literature PDL cases originated in the cerebral dura. Spinal PDL was less common with only 38 cases (22%) of 166, including the two cases presented here . Interestingly, cerebral and spinal sites of PDL appeared constantly mutually exclusive.…”

“…Our literature review investigated ‘dural’, ‘epidural’ and ‘meningeal’ lymphomas, and identified many PDL cases, including 166 well‐documented cases . Particularly, PDLs diagnosed prior to the Revised European American Lymphoma (REAL) classification (1994), and where more recent reclassification was not possible, were not included.…”

“…Due to the paucity of cases in individual series, no definite treatment protocol for PDL has been fully‐established. Different treatment combinations associating local decompressive surgery, systemic chemotherapy (corticosteroids, CHOP and rituximab), intrathecal methotrexate, and adjuvant radiation therapy for local control of disease, have been tried in these cases with good outcomes over variable follow‐up periods …”

“…Most cases of dural lymphoma consist of secondary dural involvement in the context of systemic or other organs' primary lymphomas, and well‐documented cases of primary dural lymphoma (PDL) are less common. Primary dural lymphomas are distinct from primary lymphomas of the central nervous system (CNS) that encompasses brain, spinal cord and subarachnoid space .…”

“…Approximately 22% (37 cases) of the well‐documented previously reported PDL cases are confined to the spinal dura, the remainder being of cerebral location. Two cases of spinal PDL were diagnosed in our institution over a 3‐year period.…”