Primary Cerebral Lymphoma (Central Nervous System)

What is it?

Primary central nervous system lymphomas are rare tumors, accounting for about 0.5-1% of all brain tumors and not more than 1% of all non-Hodgkin's lymphomas. They result from an abnormal distribution of one of the types of white blood cells (lymphocytes, mostly B-type) in the central nervous system. In most cases, these are diffuse large B-cell lymphomas, but there are also cases of immunoblastic, lymphoblastic, Burkitt’s lymphomas and T-cell lymphomas with primary cerebral localization.

Causes and risk factors

The disease peaks between the ages of 60 and 70, but it can affect people of any age. It occurs more often in men. It affects about 0.5 out of 100,000 people and 9% of HIV-positive people. There has been an increase in incidence over time, so much so that this tumor may become the most common brain tumor in the coming years.

Causes are not known. Immunosuppression of viral (HIV), iatrogenic, or congenital origin has been associated with the emergence of primary brain lymphomas. It can occur as a second neoplasm after chemotherapy treatment. However, most patients do not suffer from immunodeficiency. It is not a hereditary disease, but in some families there may be a predisposition to this and other lymphoproliferative diseases.

Which are the symptoms?

Symptoms are associated with the presence of intracranial formation and are therefore nonspecific:

  • headaches
  • nausea and vomiting
  • convulsions (epileptic seizures)
  • personality change
  • eye symptoms
  • speech difficulties (aphasia)
  • difficulty moving parts of the body (hemiparesis, cranial nerve palsy)
  • motor incoordination (ataxia)

How is it diagnosed?

The diagnosis is made after appropriate instrumental and laboratory tests, such as:

  • Magnetic resonance imaging with contrast media
  • Examination of cerebrospinal fluid
  • Biopsy of suspected brain lesion

The diagnostic procedure is then completed with staged examinations:

  • Eye examinations
  • Computed tomography of the chest and abdomen
  • Testicular ultrasound (for male patients)
  • Bone marrow biopsy and bone marrow aspirate

Suggested exams

How is it treated?

Treatment usually consists of cycles of high-dose chemotherapy followed by radiotherapy of the brain. Elderly patients can also be treated with radiotherapy or chemotherapy alone. In well-selected cases, a bone marrow transplant procedure may be performed after chemotherapy.

Suggested procedures

Where do we treat it?

Within the San Donato Group, you can find Primary Cerebral Lymphoma (Central Nervous System) specialists at these departments:

Are you interested in receiving the treatment?

Contact us and we will take care of you.