The signs and symptoms of Primary Central Nervous System Lymphoma (PCNSL) are typically a symptom of the disease, such as headaches, increased intracranial pressure, seizures, or other neurological signs. These symptoms are often accompanied by changes in the patient's personality or changes in vision. Although neurologic symptoms are the most common manifestations of PCNSL, systemic symptoms can occur before a patient experiences the typical neurological signs and symptoms. Patients can also develop fever, night sweats, and weight loss.
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The imaging pattern for primary CNS lymphoma is characterized by a T1 sequence with gadolinium contrast. A single homogenously enhancing lesion is visible on the T1 sequence. Fluid-attenuated inversion recovery imaging (FAI) demonstrates a narrow edema surrounding the mass lesion. Diffusion-weighted imaging (DWI) demonstrates limited diffusion within the tumor.
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A CNS lymphoma treatment is effective when the cancer has not spread outside the cerebrum, the largest part of the brain. Patients must be younger than 60 years old, be able to maintain daily functions, and be free of AIDS or other illnesses that weaken the immune system. In addition, chemotherapy can be combined with other treatments such as growth factors, which increase the patient's blood count, or antibiotics, which can treat infections and other problems.
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If a CNS lymphoma has spread to the eye, symptoms may include blurred vision, floaters, and weakness. While floaters are not characteristic of lymphoma, a CNS lymphoma with spread to the eye can result in paralysis and weakness. It may even extend to the tummy and arms. A biopsy of the affected tissue is performed to confirm the diagnosis.
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A person's immune system may be weakened by a number of factors, including organ transplants. People with compromised immune systems are at higher risk for developing CNS lymphoma. Chronic immunosuppression, such as organ transplants, can reduce the immune system's ability to fight infections. However, the incidence of PCNSL has fallen dramatically in recent years. The most common symptoms of PCNSL are headaches, double vision, hearing loss, facial weakness, and seizures.
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After a diagnosis of Primary Central Nervous System Lymphoma, patients may undergo diagnostic tests to determine the extent of the disease, and to assess response to treatment. Blood tests can detect the presence of white blood cells (WBCs) or low levels of red blood cells (RBCs).
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A diagnosis of PCNSL requires a high level of clinical suspicion, as it can involve any area of the CNS. The cancer can spread throughout the brain and spinal cord, causing focal neurologic deficits, neurological problems, and neuropsychiatric symptoms. If it grows into an inoperable tumor, the affected person will experience psychosis, seizures, and even personality changes. If left untreated, it could be fatal.
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While PCNSL is uncommon in immunocompetent patients, it can occur in both immune-competent and immunosuppressed individuals. It represents only two to four percent of newly diagnosed intracranial tumors. Most PCNSLs are diffuse large B-cell lymphomas, with only a small percentage being T-cell or low-grade lymphomas. Although it is rare, PCNSL has an increasing incidence, especially in older patients. There are approximately 1,500 new cases of PCNSL every year.
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Though the survival rate of PCNSL has improved significantly since the 1960s, it still lags behind that of systemic lymphoma. Survival is estimated at 14 months, and the relative 5-year survival is 31.2%. Treatment with methotrexate, although still not optimal, improves survival in nearly half of patients. However, methotrexate can cause late neurotoxicity. Therefore, if PCNSL recurs, the prognosis is even worse.
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While the symptoms of CNS lymphoma depend on the location of the tumor, they are commonly accompanied by other symptoms such as nausea, vomiting, arm weakness, seizures, and headaches. A patient may also develop ring enhancement in the brain. Further, patients with CNS lymphoma may experience back pain, incontinence, or incontinence.
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Further, CNS lymphoma may affect the patient's ability to work, and can lead to paralysis.
The aggressive form of NHL is primary central nervous system lymphoma. This type of CNS lymphoma develops in the brain and spinal cord. It may also affect the eye, the spinal cord, and leptomeninges. It is rare, but it can cause focal neurological deficits, including seizures, confusion, and blurred vision. Patients should undergo an MRI if they have any of these symptoms.
