Are the 27 cases of Ewing sarcoma near Pittsburgh a cluster?

A photo of Braedyn Wasko, 12, recovering from Ewing sarcoma hangs on the wall of his room in Crucible, Pa. Braedyn was diagnosed in August 2016. He's in remission. (Andrew Rush/Post-Gazette)

There is no known cause for the rare childhood bone cancer but the number of cases demand attention, experts say.

Toss 250 pennies into the air above a large map of the United States. Some will fall far apart while others will land closer together.

Those clusters of coins may occur for no other reason than randomness.

Dr. Kelly Bailey, a UPMC physician and researcher who specializes in Ewing sarcoma, uses this example to explain why there might appear to be an unusual number of rare Ewing cancers in four counties in southwestern Pennsylvania flush with gas drilling and other industrial pollution when there’s no evidence to date that drugs or environmental exposures, including radioactivity, are causing this cancer of the bone or surrounding tissue.

With one case per 1 million population, Ewing sarcoma is rare. Yet, the Pittsburgh Post-Gazette has counted 27 cases over the past decade — 2008 to 2018 —  in Washington, Greene, Fayette and Westmoreland counties, which combined have a population of about 750,000.

On the face of it, with the national incidence rate of one Ewing case per million population, the expected rate in the four-county area would be 0.75 cases per year or about eight over a 10-year period. The four-county area actually has had 27 cases, more than three times that expected rate. But that total would require statistical analysis to determine if it still could be a random occurrence.

Each case also would need to be confirmed to be Ewing, Dr. Bailey said.

While Dr. Kurt Weiss, another UPMC cancer surgeon and researcher, agrees, he still finds the number troubling. “This ought to raise some eyebrows and [prompt us to] say, ‘What, if anything, is happening with this?’ ”

The Pennsylvania Department of Health last month determined that three cases in Washington County did not meet the definition of a cluster — which requires a known link to an environmental cause — but it did not count three other Ewing cases that also had been diagnosed in that area. Earlier, it had determined that a concentration of Ewing in Westmoreland County also was not a cluster.

The U.S. Centers for Disease Control and Prevention offers little detail about the rare instance when a cluster is confirmed, save for efforts to limit public exposure to the source causing the cluster. “Only a small fraction of cancer cluster inquiries might meet the … criteria to support a cluster investigation through all the steps outlined in this report,” it says.

Anya La Mar was an athlete who played youth softball throughout the area and often in Washington County, where six cases of the rare bone cancer have occurred among people living in the Canon-McMillan School District alone.

She was adopted at 15 months from Russian novelist Leo Tolstoy’s hometown of Tula and lived with her parents in North Fayette, a township with its own history of landfills, waste disposal and strip-mining. Diagnosed with Ewing sarcoma in 2014, Anya was 11 when she died Dec. 28, 2016.

Anya La Mar of North Fayette went on a Make-A-Wish Disney cruise in November 2015 while in remission from Ewing sarcoma. (Courtesy of the La Mar family)

Anya La Mar holds her new puppies in June 2016, three weeks after the recurrence of her Ewing sarcoma. She died on Dec. 28, 2016. (Courtesy of the La Mar family)

After Bob La Mar read a story March 28 in the Post-Gazette about Ewing sarcoma in the Canon-McMillan school district over the past decade, he reached out by email that his daughter should be included in that count. It’s a stretch, for sure, with the family living in Allegheny County, five miles east of the Washington County line.

“I know there isn’t a known cause for Ewing as of yet but I thought the data for the cases could be expanded to include more, as in my daughter’s case,” Mr. La Mar said in the email, later making the case during an interview.

As Anya’s case indicates, the Ewing sarcoma scare isn’t limited to people within the school district or as many as 14 cases in Westmoreland County since 2010.

The late Luke Blanock, Curtis Valent and Kyle Deliere, all residents of Cecil Township in Washington County, were good athletes, as is Mitchell Barton, a 21-year-old North Strabane man undergoing chemotherapy for Ewing. The two other cases within school district boundaries include Alyssa Chambers, 28, now of Shaler, and David Cobb, 38, diagnosed in June 2018 and living near Southpointe business park in Cecil Township.

What happens?

Carla Wasko-Hughes talks with her son Braedyn Wasko, 12, after dinner at their home in Crucible, Pa. Braedyn is in remission from Ewing sarcoma. (Andrew Rush/Post-Gazette)

Ewing sarcoma occurs when a gene in chromosome 22 breaks away and fuses to a gene in chromosome 11. What causes this “translocation” is not yet understood. Generally it “is not inherited but arises from a mutation in the body’s cells that occurs after conception,” according to the National Institutes of Health.

Early symptoms can include pain in the area of the tumor, most often in the hip bone, chest wall and legs, the American Cancer Society says. The person also can have shortness of breath if the cancer has spread to the lungs. Most bone tumors but especially the soft-tissue tumors cause lumps or swelling, which was the case in many of the local Ewing cases.

Five-year survival rates average 67%, the cancer society reports.

Shale gas drilling and fracking, occurring in all four counties, remain a concern among cancer victims and environmental activists, particularly in Canon-McMillan, which has many wells, gas compressor stations and one of the state’s largest cryogenic gas-processing plant in neighboring Chartiers, near Cecil Township.

The Marcellus Shale Coalition, the trade organization representing the shale gas industry in Pennsylvania, points out that there are “no known lifestyle-related or environmental causes of Ewing tumors …,” and David Spigelmyer, coalition president, has said any attempts to link Ewing sarcoma cases to the industry are without scientific or medical support.

Other pollution concerns in the area have included the U.S. Department of Energy uranium mill tailings disposal site, completed in the 1980s, in North Strabane near Canon-McMillan High School. The DOE reports that annual monitoring continues showing radiation levels at or lower than background radiation levels.

The six Ewing sarcoma victims within the Canon-McMillan School District boundaries are or were: (top, from left) Kyle Deliere of Cecil, Pa., who died at 27 in 2013; Luke Blanock of Cecil who died at 19 in 2016; (bottom, from left) Mitchell Barton, 21, of North Strabane; Curtis Valent, of Cecil, who died at 21 in 2011; Alyssa Chambers of Shaler, 28, who is an oncology nurse; and (not pictured) David Cobb of Cecil, 38.

There also is the long vacant ABB Inc. site in the village of Muse in Cecil Township, where the U.S. Environmental Protection Agency and Pennsylvania Department of Environmental Protection conducted a 2003 inspection to determine whether hazardous chemicals buried there were impacting the environment.

“The facility is currently inactive and no wastes are generated at the site,” the online EPA assessment states. “The facility manufactured synthetic resins for the foundry industry and compound chemicals for use in the foundry industry.”

A DEP spokesperson said most of the cleanup occurred in the 1980s and 1990s, that some pollution remains, but that no drinking water is at risk.

Researchers and physicians are cautious about identifying potential cancer clusters, given the high statistical and epidemiological bar required to prove that specific exposures are responsible for specific cancers.

Referring to Dr. Bailey’s analogy about the randomness of falling pennies, is there another reason so many pennies have fallen on this area?

“Families affected, of course, want answers and, of course, want to know what caused it,” Dr. Bailey said. “But the far majority of the time, the cause of the cancer is simply a random error during cell division.”

But she added, “From the pediatrician standpoint and mom standpoint, I always advocate for cleaner everything — air, water — everything,” she said. “When you have multiple families and multiple cancers, it is very logical and a worthy question to ask if something is causing this. But we have no evidence demonstrating that Ewing sarcoma is caused by any environmental exposures.”

Dr. Weiss — another UPMC cancer surgeon and researcher, specializing in osteosarcoma, the most common but still rare childhood bone cancer — echoed many of Dr. Bailey’s comments but said the number should be investigated.

“This is what I’m thinking, wearing many hats,” he said. “I’ve operated on, studied, and had osteosarcoma. And I have kids, so this is washed through all of those filters. Ewing sarcoma is a disease that has an incidence around one in a million. If a super rare disease might be clustering in a specific area, that should make us want to ask some questions.

“It is hard to pin it on any one thing but this ought to be investigated, whether it is the CDC or someone else.” he said.

Number of cases grows

Ewing sarcoma patient Mitchell Barton stands in front of his North Strabane home, just below a shale gas well pad. (Andrew Rush/Post-Gazette)

The Post-Gazette confirmed Ewing cases mostly through interviews with families, with three cases confirmed by newspaper stories, obituaries and Facebook postings.

One additional case in Washington County involves Aidan Knox, 16, of Bentleyville who was diagnosed in 2012. A previously identified Charleroi case involved a girl living there but diagnosed while living in Fayette City, Fayette County, where she returned to live.

She now represents one of four Ewing cases in Fayette County over the past decade.

“You never get solid answers, and that’s the worst part,” said Cheryl Potter, who lives near Perryopolis in Fayette County. Her son Joshua was diagnosed in 2010 at age 16 and died in May 2016.

“You just take it one day at a time as it comes, and there’s nothing you can do,” she said. “They even did genealogy tests and a genealogy study and looked for certain markers, and he didn’t have any markers for Ewing, so I just don’t know.”

The other two Fayette County cases include one each in Connellsville and Uniontown.

Elissa Spiker St. Clair of Dilliner, a village in southeastern Greene County where she’d lived most of her life, had just moved to North Carolina in 2013 when she was diagnosed with Ewing, said her aunt Janet Pennington, also of Dilliner. She died in September 2015 at age 30, “a beautiful life taken too soon,” she said.

Elissa Spiker of Greene County married Trevor St. Clair in 2012. She died of Ewing sarcoma in June 2015 at age 30. (Courtesy of Janet Pennington)

Also in Greene County, Braedyn Wasko, 12, of the village of Crucible in the Carmichaels Area School District, was diagnosed with Ewing in August 2016. He’s in remission.

“It’s scary to know all these children being diagnosed in the general area,” said his mother, Carla Wasko-Hughes. “We don’t know what is happening, but it is really scary.”

The state Department of Health has confirmed that 12 cases have been diagnosed in Westmoreland County since 2011, but has not classified this as a cluster.

Casey Jackson, the son of Belle Vernon Mayor Gerald Jackson and Kristen Jackson, wasn’t  included in the Westmoreland County totals because he was diagnosed in June 2011 while stationed at Fort Bragg, N.C., just 11 months after leaving Belle Vernon for the Army. But symptoms of the cancer began before he left for the military. He died in North Carolina in September 2012.

In an email, Mr. Jackson’s mother said she reported his case after the March 28 Post-Gazette story about Ewing sarcoma cases. “I called the Pennsylvania Department of Health, and they didn’t seem to be interested in Casey,” she said in the email.

Health department spokesman Nate Wardle said investigation of cases involves the location and address of the patient at the time of diagnosis, as well as the person’s current address, as recorded by the physician who enters the case into the cancer registry.

“It is important that we take steps to ensure that our work is consistent among the cases that are part of our investigations,” he said.

But another case may have been added to Westmoreland County’s total, pushing the official count to 13 — and 14 if the Jackson case had been included.

Dr. Kevin Bartolomucci, 32, who said he’s lived all but four years of his life in Greensburg, emailed the Post-Gazette — also after the initial March 28 article — that he’d been diagnosed with Ewing sarcoma in 2010 in Erie, during his first year at the Lake Erie College of Osteopathic Medicine.

In a follow-up interview, he said he contacted the DOH, which agreed that his case should be included in Westmoreland’s total.

“I wanted to provide researchers with my information to help them with analysis of Ewing sarcoma cases in the region,” said Dr. Bartolomucci in the original email, adding in the recent interview: “I understand there’s pollution out there that’s a concern for health but I dealt with my diagnosis from the approach that life happens.”

David Templeton: dtempleton@post-gazette.com or 412-263-1578. Twitter: @templetoons. Don Hopey: dhopey@post-gazette.com or 412-263-1983. Twitter: @donhopey.

Ewing sarcoma is named after a native Pittsburgh pathologist

James Ewing, a notable 20th century cancer researcher, discovered and described various cancers, including the one named after him.

By David Templeton | Pittsburgh Post-Gazette

Almost a century ago, a Pittsburgh native was trying to figure out the difference between a mysterious bone cancer and osteosarcoma, a more common childhood bone cancer.

His breakthrough came when he found that, unlike osteosarcoma, the rarer cancer responded to radiation therapy.

As a result of that discovery, the cancer was named after James Ewing —  the famous Cornell University pathologist who first described the rare sarcoma of concern in the Canon-McMillan School District, as well as Westmoreland County and other counties throughout southwestern Pennsylvania.

James Ewing, a famous pathologist at Cornell University, appears in this undated photo. (Courtesy of Medical Center Archives of New York-Presbyterian/Weill Cornell Medicine)

Beginning at age 14, the son of Pittsburgh Common Pleas Judge Thomas Ewing would spend two years in bed due to osteomyelitis, a serious bone infection that would cripple him for the rest of his life. But his interest in classical languages, a mounted butterfly collection that became a permanent fixture “at a Pittsburgh museum,” and his acquisition of a microscope, would lead to his role as one of the 20th century’s most famous cancer researchers, according to a 1951 National Academy of Sciences biography of Ewing.

His 1919 book, “Neoplastic Diseases,” became the world’s standard cancer reference textbook for the next 30 years, it says.

Most notably, he advocated for and ultimately created America’s first specialty cancer hospital, this one at Memorial Hospital in New York. Later he co-founded the American Cancer Society, along with discovery and description of various other cancers.

On Jan. 12, 1931, a drawing of Ewing was featured on the front cover of Time magazine as part of its coverage of “The cancer men” and their advances in cancer research.

To this day, the cause of Ewing sarcoma is unknown. What researchers do know is that the sarcoma results from a gene from chromosome 22 breaking away and fusing to a gene in chromosome 11. That translocation is not an inherited genetic malady, so the mystery of what causes the cancer-causing gene translocation remains unresolved.

Ewing died in 1943, at age 76, of bladder cancer.

His accomplishments were so vast that his National Academy of Sciences’ biography doesn’t even mention his discovery of the cancer bearing his name. But his other accomplishments helped change medical history.

“Ewing was an outspoken opponent of the idea that there could be an all-embracing cause or cure for cancer, maintaining that cancer does not represent a single disease but is a generic term covering a broad department of biology and a universal potentiality of tissue cells,” it says.

A rare cancer, Ewing sarcoma, was named for pathologist James Ewing after he discovered differences in the mysterious bone cancer compared to other more common cancers. (Courtesy of Medical Center Archives of New York-Presbyterian/Weill Cornell Medicine)

David Templeton: dtempleton@post-gazette.com or 412-263-1578. Twitter: @templetoons.


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