Fox Chase scientist Joseph R. Testa said the finding could lead to new drugs. But more immediately it should enable early detection and better survival of a disease whose course is usually all too swift, said Testa, a study coleader with Michele Carbone of the University of Hawaii.
Patients whose mesotheliomas are detected in later stages, as most are, tend to die within a year, he said. Yet those whose cancers are caught early, before the tumor has spread, have a median survival of about five years, Testa said. He knows one man who lived 14 years.
Curiously, in one of the families studied, two people with a BAP1 mutation had a different rare cancer called uveal melanoma, a tumor in the eye. One of those two people also had mesothelioma.
The researchers then studied 26 more mesothelioma patients whose disease was sporadic - not hereditary - and found two more cases of the rare eye cancer. The odds of a person having both cancers are so small that the researchers think the same mutated gene plays a role in both.
Moreover, in the original two families, the mutation also appeared to be linked to several other cancers, including of the breast and kidney. "This would be called a cancer syndrome," Testa said.
Testa urged that people with the rare eye cancer get a CT scan of the lungs in case of mesothelioma. Also, those exposed to asbestos and related to a mesothelioma victim should consider a scan.
Though asbestos is no longer widely used, many are still exposed in older homes with deteriorating insulation, or on the job. The work got some funding from folks in the latter category: Local 14 of the International Association of Heat and Frost Insulators & Allied Workers, in Philadelphia. - Tom Avril