Home >> ALL ISSUES >> 2013 Issues >> ALK-positive NSCLC—patient’s story opens eyes

ALK-positive NSCLC—patient’s story opens eyes

image_pdfCreate PDF

Anne Ford

September 2013—For good or bad, Matthew Hiznay seems to be an odds beater.

First, a minority of lung cancer patients have never smoked. He’s one of them, having been diagnosed with late-stage non-small-cell lung cancer in August 2011. Obviously, that’s the bad.

Second, only about five percent of NSCLCs express a rearrangement of the anaplastic lymphoma kinase (ALK) gene. Hiznay’s is one of them. That’s the very, very good.

That’s because on literally the same day that Hiznay received his diagnosis, the FDA approved crizotinib, a tyrosine kinase inhibitor that has been found to produce an objective response rate of 61 percent for ALK-positive NSCLC (Camidge DR, et al. Activity and safety of crizotinib in patients with ALK-positive non-small-cell lung cancer: updated results from a phase 1 study. Lancet Oncol 2012;13[10]:1011–1019).

Matthew Hiznay: “I kind of view myself as the poster boy” for molecular genetics’ role in cancer.

And for once, Hiznay is in the majority: His cancer responded quickly and dramatically to crizotinib. On Sept. 10, 2011, he took his first dose of the drug. Two months later to the day, scans revealed no traces of the disease.

“He had been at death’s door, and he had just a dramatic response,” says Raymond Tubbs, DO, professor of pathology and section head of molecular oncologic pathology in the Department of Molecular Pathology, Cleveland Clinic, and the pathologist who identified the ALK rearrangement in Hiznay’s cancer that was susceptible to crizotinib.

But this story is more than the account of one lucky young man and one effective medication. It’s also the tale of a pathology team that was able to alter a patient’s treatment in a powerfully specific way—as well as the account of a patient who helped that team realize just how crucial their work can be.

When Hiznay developed a persistent cough in July 2011, he assumed at first that it was related to his seasonal allergies. The busy 26-year-old, who had just completed his first year of medical school at the University of Toledo, decided to wait until the following month to have it checked out during his annual physical. That’s when an x-ray revealed a hazy left lung and an initial diagnosis of sarcoidosis.

Shortly afterward, he discovered a rapidly growing knot in his left trapezius muscle, which led to the correct diagnosis—stage IV lung adenocarcinoma—on Aug. 26. As he learned, the primary tumor was growing in his left lower lobe, and it had metas­tasized to his right lung, all media­stinal lymph nodes, lower cervical lymph nodes, sternum, and gastrohepatic ligament.

For treatment, Hiz­nay headed to the Cleveland Clinic, where he received two pieces of good news. First, the cancer had not spread to his brain. Second, the director of the lung cancer medical oncology program, Nathan Pennell, MD, PhD, was sending a biopsy of the cancer for molecular genetic testing, “because,” Hiznay remembers, “there were some new chemotherapy drugs coming on the market that were very specific for gene mutations. That was the first I’d heard about it. I didn’t realize they could target a specific mutation like that.”

Hiznay didn’t expect the genetic testing to yield anything useful. “So much had gone wrong up to that point that I thought it’d be too good to be true,” he says. “I wasn’t getting my hopes up. I thought: I’ve already defied the odds by getting lung cancer without being a smoker, so how am I going to be able to defy the odds twice?”

Besides, there was a new and sinister development to deal with. The tumor had begun to slither around his pericardium, eventually leading to cardiomegaly, bilateral pleural effusion, bilateral collapsed lower lobes, a collapsed right middle lobe, pneumonia, a massive clot in his superior vena cava, pulmonary embolisms in both lungs—and, on Sept. 2, 2011, cardiac arrest. Hiznay’s physicians considered a pericardial window to ease the stress on his heart, but by this point, Hiznay was so ill they feared he might not survive the procedure.

Dr. Tubbs

CAP TODAY
X