During a grueling operation early last year, when the intractable five-pound tumor seemed to mock his skills, thoracic surgeon Joseph S. Friedberg felt buoyed by what he and his scrub-suited crew had already achieved.
The University of Pennsylvania team battles pleural mesothelioma, a rare, ferocious, incurable type of lung cancer.
Typically, patients die within a year of diagnosis. Yet more than two years after treatment at Penn, 27 out of 38 patients - 71 percent - were still alive, including four who had marked five years. These were advanced-stage cases, ostensibly hopeless, and they were defying the odds.
Friedberg, who was about to submit a study on those results for publication, knew there would be skepticism. The number of patients was small. And the treatment was almost as formidable as the disease. He spent up to 14 hours stripping out the cancer while preserving the patient's lung; then residual malignant cells were zapped with laser light therapy.
He was disappointed, but not surprised, when the Annals of Thoracic Surgery demurred at publishing the study. "The reviewers said the follow-up time was too short and we were overestimating" the projected survival time, he recalled.
The only way to address that concern was to let more time elapse.
Last month, with a fuller picture, the journal published the results, which are impressive.
This story is about those results, and how the 25 members of Penn's pleural and mesothelioma program are making remarkable progress against a dreadful disease.
It's also about the heartbreaking inadequacy of that progress.
"I don't particularly consider it a victory," said Friedberg, co-director of the program. " 'Good' for me would be 10 years. Every time these patients' cancer recurs, it kills me."
Caused by asbestos
Pleural mesothelioma is caused by exposure to asbestos, a once-ubiquitous building material. Decades after exposure, renegade cells arise in the mesothelium, a cellophane-thin membrane that lines the chest cavity, lungs, heart, and diaphragm.
By the time the classic symptom of breathlessness shows up, the cancer usually has a stranglehold, invading all surfaces and organs in the cavity.
Conventional treatments - chemotherapy, radiation, and surgery - have dismal outcomes. In the pivotal study of the only chemotherapy specifically approved for mesothelioma, 60 percent of patients did not respond. For the rest, it extended median survival just 13 weeks, to 12.1 months.
Mesothelioma has become a big specialty for liability lawyers, but with 3,000 new cases a year in the United States, it is hardly a hot research field.
Nonetheless, for more than two decades, Penn scientists have been doggedly pursuing innovations. They are combining conventional treatments with gene therapy, T-cell therapy, and the laser technology, called photodynamic therapy.
Patients come thousands of miles to Penn after exploring their options - or lack thereof.
William J. McQueen, 63, an ear-nose-and-throat doctor from San Antonio, Texas, is one of them. Because his cancer encased one lung - the typical pattern - and because he was in good overall health, specialists at several top hospitals recommended an "extrapleural pneumonectomy."
The harrowing surgery involves removing the lung, the lining of the chest, the heart's sac, and the diaphragm muscle, which controls breathing, then reconstructing certain tissues with high-tech fabric.
Up to 10 percent of pneumonectomy patients die of complications. The rest die of the cancer, which invariably comes roaring back, typically within months.
McQueen asked about preserving his lung but was told that would depend on the extent of his cancer - something that would be assessed on the operating table.
"I got the impression they would not take the time to strip the tumor out," he said. "They'd go in and take the lung out. That's what most surgeons do."
Sparing the lung
That's what Friedberg used to do.
Theoretically, taking the lung left minimal cancer for conventional therapies to mop up, thus lowering the chance of relapse. In reality, he could see it didn't work. It was like uprooting a garden to get rid of weeds, only to have the weeds grow back worse than ever.
