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Lymphoma of the spinal cord

Lymphoma of the spinal cord is an uncommon manifestation of lymphoma.

Although lymphoma more commonly involves the vertebral body (vertebral body tumours) or epidural compartment, intramedullary lymphoma does rarely occur.  

Apparent intramedullary spinal cord lymphoma may often in fact represent secondary invasion of the spinal cord by leptomeningeal disease8.

Epidemiology

Intramedullary spinal lymphoma accounts for 3.3% of all CNS lymphoma, which constitutes only 1% of all lymphomas in the body 1.

The mean age at presentation is 47 years. Females are more commonly affected than males.

Risk factors for developing CNS lymphoma include: 

  • AIDS 
  • transplant recipients
  • congenital immune deficiency
  • Epstein-Barr virus infection

Clinical presentation

Clinical presentation is similar to that of other intramedullary spinal tumours, with pain, weakness and sensory changes common

Pathology

Primary lymphoma of the spinal cord is most often predominantly histiocytic or mixed histiocytic and lymphocytic 2. 85% are non-Hodgkin lymphomas 2.

The principal microscopic feature is a dense, perivascular, mononuclear infiltrate composed predominantly of large lymphocytes with prominent nucleoli. Most tumours are B-cell derivatives3.

Radiographic features

The most common intramedullary location is the cervical cord, followed by the thoracic cord then the lumbar cord 7. Most are solitary lesions, however there may be multiple lesions throughout the spinal cord.

MRI

Although spinal cord expansion is usually present, in some patients there is relatively minimal enlargement of the cord 4. The lesions are generally poorly defined and tumoural cysts are generally not a feature and secondary syringomyelia is rare 4. Lymphoma usually does not have a haemorrhagic component 8.

Reported signal characteristics include:

  • T1 : isointense to spinal cord
  • T2 : hyperintense (this is in contrast to the characteristic low T2 signal intensity that is seen in intracranial lesions)
  • T1 C+ (Gd) : usually solid and homogeneous enhancement 4

Treatment and prognosis

Radiotherapy is the primary therapy for potential preservation of neurologic function and extension of survival. Minimal data are available on the role of chemotherapy for primary spinal cord lymphoma9.

The prognosis for patients with intramedullary spinal lymphoma is poor. The survival rate at 2.5 years is less than 50%3.

Differential diagnosis

General imaging differential considerations include

See also

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