What is Spinal Cord Cancer (Lymphoma of the Spinal Cord)

Lymphoma of the Spinal CordCancers of the spinal cord may be of the Lymphoma type.

The spinal cord forms part of the central nervous system. It is a cylindrical continuation of the brainstem, commencing from the medulla (at the level of the foramen magnum at the base of the skull) and extending to the conus medullaris at the level of the L2 vertebra. It is located within the vertebral canal – the bony tube formed by the vertebral foramina. Below the level of L2, the remaining contents of the spinal canal are known as the cauda equina, the bundle of nerve rootlets in the subarachnoid space. Within the vertebral canal run the spinal cord, the spinal meninges, the cerebrospinal fluid, associated vessels and loose connective and fatty tissue.

Arising from the spinal cord are 31 pairs of spinal nerves that are the neural connections between the peripheries and the central nervous system. They attach to the spinal cord through ventral and dorsal roots and have both afferent and efferent properties. That is, the spinal cord acts as the main pathway for communication between the brain and the rest of the body.

Tumours of the spinal cord can be either primary or metastatic. The vast majority of spinal cord tumours are metastatic deposits from other primary sites. Tumours of the spinal cord can be divided into three groups, based on the anatomical location of the tumour mass.

Firstly, they are divided by their relationship to the spinal meninges, with tumours being classified as intradural or extradural. Furthermore, intradural tumours can be subdivided into those arising within the substance of the spinal cord itself – intramedullary tumours – or those arising in the subarachnoid space (extramedullary).

Extradural tumours most commonly represent metastases and usually arise within the vertebral bodies. These tumours most commonly cause spinal compression through extrinsic mass effect but can on occasion do so through intradural invasion. Symptoms from these tumours tend to be the slowly progressing features of spinal cord compression with initial predominantly motor loss followed by progressive sphincter dysfunction and ascending sensory loss. Extradural tumours represent the vast majority of spinal cord tumours.

Intradural extramedullary tumours tend to be nerve sheath tumours (neurofibromas) or meningiomas. They commonly present with nerve root involvement consisting of pain and progressive dysfunction due to spinal cord compression from the expanding tumour mass.

Intramedullary tumours usually represent gliomas ependymomas or astrocytomas, but metastatic deposits within the spinal cord itself are being increasingly recognised. These tumours result in a diffuse swelling of the spinal cord, often over several segments, characterised by loss of local function, pain and eventual loss of spinal cord function below the level of the lesion. A cyst may form in the spinal medulla, giving rise to a clinical picture of syringomyelia (predominant loss of spinothalamic function pain and temperature sensation – and blunted reflexes at the level of the lesion). Sacral sparing is a very late feature of intramedullary tumours but is pathognomonic.

The image shown above right is of an MRI scan that illustrates multiple vertabral metastases causing spinal cord compression.

Statistics on Spinal Cord Cancer (Lymphoma of the Spinal Cord)

Lymphoma of the Spinal Cord is rare. Lymphoma of the spinal cord most commonly represents metastatic disease but can occasionally occur as a primary malignancy. Primary spinal lymphoma represents less than 1% of all CNS (central nervous system) lymphoma and occurs with increasing age with sex incidence being approximately equal.

Geographically, the tumour is found worldwide.

Risk Factors for Spinal Cord Cancer (Lymphoma of the Spinal Cord)

Predisposing factors in the development of primary central nervous system lymphoma centre around immunodeficiency (including AIDS and following organ transplantation) when the disease can occur with increased incidence in younger patients. Sporadic cases of primary CNS lymphoma have been found in association with other diseases.

Progression of Spinal Cord Cancer (Lymphoma of the Spinal Cord)

This type of tumour spreads by infiltration of the cerebrospinal fluid and seeding within the central nervous system. In less than 10% of patients distant metastases occur to sites such as the viscera, bones and soft tissues.

How is Spinal Cord Cancer (Lymphoma of the Spinal Cord) Diagnosed?

General investigations may show anaemia of chronic disease or evidence of systemic lymphoma if the disease is due to metastatic deposits.

Prognosis of Spinal Cord Cancer (Lymphoma of the Spinal Cord)

Untreated primary lymphoma of the central nervous system is rapidly fatal with median survival of only months from the time of diagnosis. With appropriate treatment the 5 year survival can be as high as 30-40%. However, the disease tends to recur and be associated with a very poor prognosis. Independent prognostic factors are age and neurological performance status.

How is Spinal Cord Cancer (Lymphoma of the Spinal Cord) Treated?

Initial treatment of patients with suspected spinal cord compression is with corticosteroids. However, in the case of primary lymphoma these may delay or interfere with diagnosis and so should be delayed until pathological confirmation is received if the diagnosis is suspected.

Surgery in lymphoma of the spinal cord is usually limited to a sampling biopsy in order for a tissue diagnosis to be established. The mainstays of treatment are radiotherapy, corticosteroids and chemotherapy.

The symptoms that may require attention are those resulting from the neurological deficits associated with the tumour and the neurogenic pain from compressed nerve tissue. Patients with significant neurological deficits are best managed by a specialised neurological team using a multidisciplinary approach including physiotherapy, occupational therapy, nursing and medical practitioners.

Improvement in symptoms is an important measurement. Specific monitoring may be done by serial neurological examination and imaging of the spinal cord.

Spinal Cord Cancer (Lymphoma of the Spinal Cord) References

[1] Adams RD, Victor M: Diseases of the spinal cord. In: Principles of Neurology. 1993: 1078-116.
[3] Schutta HS: Spinal tumors. In: Joynt RJ, Griggs RC, eds. Clinical Neurology. 1997: 1-175.

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