Valerie Harper Has Terminal Cancer in Her Brain

TV sitcom icon Valerie Harper is battling a devastating cancer complication.

The 73-year-old star, known for her role as Rhoda Morgenstern on the 1970s television series The Mary Tyler Moore Show, and for her starring roles on the sitcoms Rhoda and Valerie, has been diagnosed with a rare brain cancer called leptomeningeal carcinomatosis.

Currently, Harper is undergoing chemotherapy to slow the progression of this incurable disease, which is probably a late complication of her lung cancer in 2009.

In January, while rehearsing for her one-woman show called Looped, she complained of numbness in her jaw. She was hospitalized and a spinal tap made the diagnosis. Doctors estimated that she has less than 3 months to live.

Harper told People magazine that when she got the diagnosis:

I was stunned…. And in the next minute I thought, “This could draw attention to cancer research. I think there’s an opportunity to help people.”

Ironically, friend and co-star Mary Tyler Moore recently revealed that she had been treated for a tumor in her brain, in Moore’s case a benign meningioma.

What is Leptomeningeal Carcinomatosis?

Leptomeningeal carcinomatosis (LC), sometimes called neoplastic meningitis, is a condition in which a cancer widely spreads to the leptomeninges, part of the coverings of the brain.

The meninges is a system of membranes which covers the central nervous system. The meninges consist of three layers: the dura mater, the arachnoid mater, and the pia mater. The primary function of the meninges and of the surrounding cerebrospinal fluid (CSF) is to protect the central nervous system. The leptomeninges (literally thin meninges) is a term used to refer to the pia mater and arachnoid mater.

In LC, cancer cells are introduced into the cerebrospinal fluid and then can be spread throughout the fluid filled spaces of the brain- the subarachnoid space (the space between the arachnoid mater and the pia mater) and the ventricular system around and inside the brain and spinal cord. The most common cancer cells to be spread this way include breast and lung cancer, melanoma and blood cancers (lymphoma and leukemia).

It is believed that cancer cells can get into the spinal fluid in one of a few ways:

1. From the blood vessels that supply the leptomeninges
2. From direct extension from tumors that have spread to the brain
3. Along the path of the major nerves (cranial nerves) to the brain

How common is LC?

Although believed to be uncommon, the occurrence of seems to be increasing. This is felt to be due to both the improved long-term survival of cancer patients, and improvement in the diagnosis of the disorder. According to Dr. R Andrew Sewell, approximately 1-8% of patients with cancer are diagnosed with LC. It is present in about 20% of those with cancer and neurological signs and symptoms.

What are the symptoms of LC?

The symptoms of LC are related to the specific brain structures affected by the tumor:

1. Cerebral involvement- headaches, nausea, seizures, encephalopathy (altered mental state)
   

   Hydrocephalus

2. Cranial nerve involvement- double vision, hearing loss, facial numbness, decreased vision
3. Spinal involvement- weakness in the extremities, paresthesias (abnormal sensations or numbness), pain
4. Blockage to the flow of CSF can lead to hydrocephalus (“water on the brain”) causing increased pressure on the brain. This can lead to symptoms of headaches, vomiting, nausea, papilledema (swelling of the optic nerve), sleepiness or coma.

How is LC diagnosed?

There are two main ways that LC is diagnosed:

First, physicians will do a spinal tap (otherwise known as a lumbar puncture) to obtain CSF . During a spinal tap, a hollow needle is inserted into the subarachnoid space in the region of the lower back. Fluid is withdrawn and sent to the lab to be examined under the microscope. Finding cancer cells in the CSF is diagnostic of LC, unfortunately only about 45% of patients will have cancer cells seen on a first spinal tap. Repeat spinal taps may increase that percentage.

A special type of MRI (magnetic resonance imaging) called Gadolinium-enhanced multiplanar MRI is another way to diagnose LC. Although 70% of patients with LC will have it show up on this type of MRI, the absence of findings on the MRI does not mean that LC does not exist.

How is LC treated?

Untreated leptomeningeal metastases have a poor prognosis with patients usually succumbing within a few months. With treatment that time may be extended up to 6 - 10 months. Treatment can consist of chemotherapy (usually injected directly into the CSF) or radiation therapy.