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.
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 spread throughout the fluid filled spaces of the brain -- the subarachnoid space -- 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 hematologic malignancies (lymphoma and leukemia). It is believed that cancer cells can get into the spinal fluid in one of a few ways:
- From the blood vessels that supply the leptomeninges
- From direct extension from tumors that have spread to the brain
- 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:
- Cerebral involvement -- headaches, nausea, seizures, encephalopathy
- Cranial nerve involvement -- double vision, hearing loss, facial numbness, decreased vision
- Spinal involvement -- weakness in the extremities, paresthesias (abnormal sensations or numbness), pain
- Blockage to the flow of CSF can lead to hydrocephalus causing increased pressure on the brain. This can lead to symptoms of headaches, vomiting, nausea, papilledema, sleepiness or coma.
How is LC diagnosed?There are two main ways that LC is diagnosed:
First, by lumbar punction to obtain CSF for histologic examination. 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 is not present.
How is LC treated?Untreated leptomeningeal metastases have a poor prognosis with patients usually succumbing within a few months. Treatment is therefore, for the most part, palliative. The objective is to preserve neurologic performance and to improve the quality of life for patients. With treatment that time may be extended up to 6-10 months.
Because the blood brain barrier prevents the penetration of most chemotherapy regimens in therapeutic ranges, treatment is often given intrathecally. Radiation therapy also plays an important role in the treatment of LC. External beam radiotherapy is indicated and beneficial for patients with large tumor burden. Corticosteroids used to treat symptoms associated with increased intracranial pressure and/or spinal cord compression.