Targeting rare cancers
By Richard Saltus
Stubborn skin rashes, red bumps, scaly patches, and a small growth on his lip sent Kervork Tevekelian to a dermatologist, but it was nearly a year before his condition received a definitive — and frightening — diagnosis.
The retired dry cleaner from Belmont, Mass., had developed an uncommon skin cancer called cutaneous T-cell lymphoma (CTCL). It is rarer than malignant melanoma but can be equally deadly. The cancerous immune T cells in CTCL use a specific molecular code to get into skin, just like normal T cells that patrol the skin to fight infection. But the cancerous T cells become aggressive and cause severe inflammation; they may also become malignant and spread to other parts of the body.
Fortunately for Tevekelian, an expert in CTCL was close at hand in Thomas Kupper, MD, of Dana-Farber and Brigham and Women's Hospital. Between 1,000 and 1,700 cases of CTCL are diagnosed each year nationally, "and we are on target to see about 100 new patients annually, or 5—10 percent of all cases in the country," according to Kupper.
Thomas Kupper, MD, and Marianne Tawa, RN, (center) discuss treatment with Elaine Hornung.
Tevekelian's outlook is good; his form of CTCL hasn't invaded his body beyond the skin. When his symptoms were at their worst, however, they were painfully disfiguring. Hard, red patches marred his face, ears, and shoulder, his scalp became red, and his hair thinned. He hated to go out in public. Although he still has some signs of the disease, his appearance has improved, thanks to treatment that included interferon and electronbeam radiation. "I'm feeling more gregarious," says Tevekelian. "I don't feel like I'm some grotesque thing."
Dana-Farber's Cutaneous Oncology Disease Center also sees patients with a rare and dangerous form of skin cancer called Merkel cell carcinoma, a malignancy that begins in nerve cells that provide the sensation of touch. Only about 500 cases are diagnosed in the United States each year, but its 26-percent fatality rate is the highest of any skin cancer.
Paul Nghiem, MD, PhD, of Dana-Farber has a special interest in this disease, which is becoming more common and affects people with weakened immune systems.
Many Boston residents learned about Merkel cell carcinoma in 2003, when it was diagnosed in David Brudnoy, a popular radio talkshow host. If caught before spreading to the lymph nodes, it has a 90-percent cure rate, says Nghiem, but that number plummets if the disease has metastasized. Radiation is the most effective treatment, though other DFCI researchers are considering testing chemotherapy agents that have been used in cancers with some similarities.
"I believe Merkel cell carcinoma is often mismanaged; there are not many physicians who think about this cancer a lot," says Nghiem. "At Dana-Farber, we do."
