Revision 32 for 'Lymphoma of the spinal cord'

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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 tumors) or epidural compartment, intramedullary lymphoma may rarely occur.  

Apparent intramedullary spinal cord lymphoma may often, in fact, represent a secondary invasion of the spinal cord by leptomeningeal disease 8.

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

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 tumors, 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 tumors are B-cell derivatives 3.

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 tumoral cysts are generally not a feature and secondary syringomyelia is rare 4. Lymphoma usually does not have a hemorrhagic 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 lymphoma 9.

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:

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