Primary central nervous system NHL

At this time we have little to offer on this very rare type of lymphoma other than a link to a web site that all PCNSL patients should be made aware of.  It is about Blood-Brain Barrier Disruption, a new way to delivery chemotherapy directly to the brain, and getting around the blood brain barrier problem. If you have PCNSL please check this link and discuss it with your doctor.

Blood Brain Barrier Disruption website.

Diagnosis and prognosis

A diagnosis is usually made via a lumbar puncture. The prognosis for PCNSL remains challenging and overall survival is lower than for other aggressive types of NHL.

Treatments

The mainstay of treatment for PCNSL has been high dose Methotrexate, or Intrathecal Methotrexate. (Intrathecal means injected directly into the cerebral spinal fluid). Often that is accomplished using an Omaya reservoir, which is a port installed directly into the skull.

ARA-C (aka Cytarabine) is also a common treatment for PCNSL. Similarly to Methotrexate it is infused intrathecally. There is also a liposomal version of ARA-C called Depocyt. This form of ARA-C is encapsulated in a fat molecule. It will generally deliver more drug to the cancer and less to other normal tissues, thus reducing side effects. (Sometimes you will see it spelled Depocyte)

A recent study from the 2009 ASCO convention looks at the results of combining both Methotrexate and ARA-C. Here is the study.

Randomized phase II trial on primary chemotherapy with high-dose methotrexate (HD-MTX) alone or associated with high-dose cytarabine (HD-araC) for patients with primary CNS lymphoma (I.E.L.S.G. #20 Trial): Tolerability, activity, and survival analyses

Although not common practice at this time, some studies are looking at intrathecal Rituxan for central nervous system involvement of NHL. Note that CNS involvement is not the same as "primary" central nervous system lymphoma. Therefore these results may not be applicable.

Phase I Study of Intraventricular Administration of Rituximab in Patients With Recurrent CNS and Intraocular Lymphoma

Whole brain radiation is controversial. While it often results in better disease control it comes with significant risk of cognitive problems. Some studies are suggesting that it may not lead to better overall survival.

Primary central nervous system lymphoma: the role of consolidation treatment after a complete response to high-dose methotrexate-based chemotherapy

The next study looks at the issue and finds that although whole brain radiation is eventually needed for most patients, it may be beneficial to reserve for relapse to preserve brain function as long as possible.

High-dose methotrexate based chemotherapy with deferred radiation for treatment of newly diagnosed primary central nervous system lymphoma

Here is yet another study the finds an upfront SCT can yield some excellent results without the neurotoxicity. Nevertheless the mortality rate is high.

Thiotepa, busulfan, cyclophosphamide, and autologous stem cell transplantation for primary cns lymphoma: a single centre experience

Additional information

Here are some links to further information on Primary Central Nervous System lymphoma.

Blood-Brain Barrier Disruption and Intra-Arterial Methotrexate-Based Therapy for Newly Diagnosed Primary CNS Lymphoma: A Multi-Institutional Experience

E-medicine PCNSL page

National Cancer Institute PCNSL page-professional version

National Cancer Institute PCNSL page-patients version