Twin Skin

Three TMC doctors. Two identical twin sisters. One extraordinary transplant.

 
Mary Jane Fields (left) donated her abdominal tissue to her identical twin sister, Marian (right), in a groundbreaking transplant and reconstructive plastic surgical procedure.

Mary Jane Fields (left) donated her abdominal tissue to her identical twin sister, Marian (right), in a groundbreaking transplant and reconstructive plastic surgical procedure.

The growth on Marian Fields’ back started out in the fall of 2011 as a lump the size of a half dollar, right along the center of her spine. At first, the 66-year-old and her twin sister, Mary Jane, thought it was an innocuous cyst. A dermatologist sliced it out, but within nine months, the growth grew back again. And again. And again. And again.

Marian endured six surgical procedures and 30 radiation treatments to remove the growth, but the tumor and radiation damage had sprouted into a painful, hard, leathery mass that engulfed nearly every square inch of her back. For years, she was unable recline in her seat or sleep on her back because of the pain. The radiation fibrosis “was like the worst sunburn you ever had,” Marian said. Mary Jane would continuously rotate through ice packs to help alleviate the burning on her sister’s back.

In October 2016, Marian went to see a plastic surgeon at the University of Kansas Hospital in hopes of finally freeing herself from the incessant nightmare on her back. But when the doctor saw the severity of her condition, his response crushed her.

“I walked out of the doctor’s office crying and scared to death,” Marian said. “He was very discouraging and said that he didn’t see how I would ever have anything beyond a major hole in my back. There’s no amount of skin that could cover what would have to be cut out. There was no way it could be done. My quality of life would be significantly diminished.”

Soft tissue sarcoma

After Thanksgiving 2016, Marian noticed that she had developed multiple lumps on her back, some of which had grown so quickly that they became necrotic, and the open sores exposed raw flesh. In a Hail Mary attempt for a cure, the Fields twins traveled in March 2017 from their home in Independence, Missouri, to The University of Texas MD Anderson Cancer Center, where sarcoma expert and surgical oncologist Keila Torres, M.D., Ph.D., diagnosed Marian with plexiform fibrohystiocytic sarcoma, an extremely rare and aggressive type of soft tissue cancer.

Soft tissue sarcoma accounts for less than 1 percent of all cancers, but Marian’s specific type of sarcoma is so rare that there have only been 150 reported cases since the 1980s, Torres explained.

The tumors grow in interlaced nodules that multiply on top and beneath the skin’s surface. Because of the rarity of the disease, coupled with the inconspicuous nodules, there is a 50 percent chance of the tumor recurring. But Marian’s tumor had grown back every single time, seven times.

“If the patient goes to a surgeon, they will look at the nodules that are on the surface, but they won’t realize that, underneath the skin, there may be other nodules that you cannot see,” Torres said. “Whenever they resect the margins of the specimen, it may have some tumor cells that were left behind.”

Torres and Jesse Selber, M.D., a reconstructive plastic surgeon at MD Anderson, devised a plan to first surgically remove the diseased flesh and then perform an autologous microvascular reconstruction—they hoped to transfer skin, muscle and tissue from Marian’s abdomen and thighs to cover the excision.

Unfortunately, Marian’s petite 5-foot, 115-pound frame was an issue. There simply wasn’t enough of Marian to go around. She had previous surgery on her abdomen, rendering the small amount of tissue there unusable.

“Because [Marian’s tumor] had recurred several times and had grown to epic proportions, it outstripped most reconstructive tools we normally use to perform cancer reconstruction,” Selber said.

The “suboptimal” alternative was to use muscle and skin from multiple parts of Marian’s body and patch them together like a quilt to cover the hole from the resection, but there was a high risk of chronic wound and pain management for years to come, Selber explained.

This option sounded bleak, but Marian’s twin was determined to help her sister.

“Just use me,” Mary Jane told the doctor.

Selber had heard that offer many times before: A patient’s loved one often volunteers his or her own tissue for transplant. Although it’s a noble and altruistic offer, it is rarely feasible because transferring tissue between two individuals requires immunosuppression, which is considered too risky in cancer patients.

Selber took a longer look at the two women.

“How are you two related?” he asked.

“Well, I’m her identical twin,” Mary Jane responded.

Selber raised his eyebrows. The sisters certainly didn’t look like identical twins. Mary Jane is taller and broader than Marian, with a larger body and head. She stands at 5’4.5″ and, at the time, weighed 44 pounds more than Marian.

It wasn’t the first time someone doubted they were twins. By the time Mary Jane entered college, she had grown an additional 4.5 inches beyond her sister and continued to gain weight. For 10 years, doctors couldn’t explain what was causing the mysterious changes in her body. Eventually, Mary Jane was diagnosed with acromegaly, a tumor in her pituitary gland that caused her to produce excessive growth hormone.

She went on steroid medication and had the tumor removed, but by then, her appearance and size had already dramatically changed to the point where she and Marian no longer looked like identical twins.

When Selber heard this, he realized a rare and unique opportunity had presented itself. If he could confirm that Marian and Mary Jane were, indeed, identical, Mary Jane’s larger build might actually provide Selber with enough tissue from her abdomen to reconstruct the hole in Marian’s back.

Selber ordered genetic tests that would determine if the sisters were 100 percent identical and a match for transplant. He enlisted the help of transplant surgery veteran Osama Gaber, M.D., director of the Houston Methodist J.C. Walter Jr. Transplant Center, to run further immunological tests. Gaber and Selber had collaborated two years earlier to perform the world’s first skull-scalp-kidney-pancreas transplant.

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Osama Gaber, M.D., is director of the Houston Methodist J.C. Walter Jr. Transplant Center.

Confirming an identical match was critical to the success of the surgery. Typically, a transplant recipient would be placed on immunosuppressive medication to prevent the rejection of the new organ. But with the high recurrence rate of Marian’s tumor, the doctors couldn’t risk weakening her immune system. If they were identical twins, there was a chance immunosuppression wouldn’t be necessary.

The doctors found that not only were the twins identical on six of the human leukocyte antigens usually measured, but they were a match on all 10.

“They were completely identical,” Gaber said.

But the twins didn’t need genetic tests to prove they were one. Their kinship ran deeper than the flesh and bone of their bodies or matching DNA on a lab report. Except for six years in their late 20s, Marian and Mary Jane have lived together their entire lives. Neither ever married or had any children. They have each other, and that’s all they need.

“We get along really well. It seems silly to force a separation. If we lived in separate houses, we would always be at each other’s house,” Marian said. “It’s just nice to have your best friend around all the time.”

“Good friendship is one soul in two bodies,” Mary Jane said, quoting Aristotle. “That’s us.”

What is life without quality?

Before the doctors could proceed with the surgery, Gaber put the twins through the routine process of consent. He had to make sure they understood all the risks and benefits. Although he, Selber and Torres supported the surgery, they had to make sure the twins knew every possible complication that could arise and all the challenges they might face. There was a chance the tumor could recur. The surgery might not completely relieve Marian of the neuropathic pain caused by the radiation. And Marian’s mobility and strength might be permanently limited from the surgery.

In addition, the procedure would not extend Marian’s life. She had an aggressive tumor, but because her type of sarcoma typically doesn’t metastasize to other organs—such as the liver, lung and bones—it wasn’t life-threatening, Torres explained.

There were risks for Mary Jane undergoing such a major surgery, as well. Doctors planned to cut out a mass of flesh from her abdomen to graft on to Marian’s back.

“When you counsel donors, you don’t tell them, ‘This is great. It’s all going to go well.’ You tell them what the risks and the benefits are,” Gaber said. “You may come across like you’re not sure you want them to do this, but I was from the very first moment really secure in the fact that this was a really good option.”

One thing was certain: If the surgery was successful, it would greatly improve Marian’s quality of life. When the doctors asked the twins one last time if they still wanted to go through with the surgery, Mary Jane responded: “What is life without quality?”

The twins were resolute.

“In my mind, from the beginning, going down [to MD Anderson], there was no other choice than some radical kind of surgery,” Marian said. “This was going to solve all of our problems. It was all good from the minute we went down there.”

Not only was the surgery risky, but it would be the hospital’s first living related donor transplant and the hospital’s first vascularized composite allograft (VCA)—a transplant of tissue other than an organ. It would also be one of the few VCAs ever performed between identical twins, and certainly the most extensive. It was “one of those lightning-striking-in-a-bottle type of situations,” Selber said.

“When I first proposed the transplant plan in detail, that it was going to require two simultaneous operating rooms, it was going to be a transplant that’s never been done for cancer reconstruction, everybody got a little nervous,” Selber said. “People start popping up to protest, which is what happens. It’s their job to make sure that surgical innovation is not a euphemism for human experimentation.”

Selber, who already established a reputation in innovative transplantation from performing the world’s first skull-scalp-kidney-pancreas transplant, leveraged the success of his leadership during that groundbreaking surgery to gain permission from the various regulatory groups, quality officers, perioperative service leaders and key stakeholders within the institution for the Fields sisters.

“The first person through the wall always gets bloody,” Selber said. “If you’re going to be the first person to do something, you have to be prepared to take criticism and defend your ideas with medical and scientific rationale and vision. But once you demonstrate the ability to build and lead a multidisciplinary, multi-institutional team for a very heavy lift like this, the medical community writ large has more confidence in your ability to conduct team medicine and surgical innovation.”

After Selber presented his meticulous plans for the surgery, arguing that although elaborate and unusual, it was the most logical and appropriate solution to an almost impossible problem and would give Marian a quality of life she hadn’t enjoyed for nearly six years, MD Anderson finally gave him the green light.

“MD Anderson sees the benefits of achieving great things in cancer care for our patients,” Selber said. “This is an institution that prides itself on being able to do things that nobody else can do. That’s what makes it the best cancer center in the world. If we can’t do this kind of thing here, where can it be done?”

14 Hours

On June 26, Marian went in for the first leg of the surgery. Knowing the recurrent nature of the disease, Torres needed to cut wide and deep into Marian’s back to make absolutely sure that the surrounding tissue was tumor-free. She carved out a large section of Marian’s skin, fat and muscle all the way down to her ribcage and worked with a spinal tumor surgical oncologist to shave off part of Marian’s spine from her neck down to her pelvis. It was paramount that no tumor cells remained.

Jesse Selber, M.D., connects the arteries and veins in Marian Fields’ back to the new tissue her twin sister, Mary Jane, donated. (Photo Credit: The University of Texas MD Anderson Cancer Center)

Jesse Selber, M.D., connects the arteries and veins in Marian Fields’ back to the new tissue her twin sister, Mary Jane, donated. (Photo Credit: The University of Texas MD Anderson Cancer Center)

What was left of Marian’s back was a gaping 20 by 30 centimeter hole—nearly 8 inches by 1 foot—that spanned from shoulder blade to shoulder blade and extended from her neck down to her pelvis, leaving her ribs and spine completely exposed.

It was a jarring sight, even for Torres.

“It sounds very morbid [because] it was a really big resection,” Torres said. “During the procedure, somehow it doesn’t feel right when you’re doing something like that because it’s so massive.”

The pathology team, led by Victor Prieto, M.D., Ph.D., then carefully analyzed the specimen to confirm that all margins were clear of any remaining tumor cells before moving onto the reconstruction. Everything was clear.

Four days later, on June 30, the twins moved into their respective operating rooms. Selber and a team of five additional plastic reconstructive surgeons worked on Mary Jane first. Selber drew incisions on her abdomen in the shape of a large oval, spanning from hip to hip and belly button to bikini line. He removed a slab of Mary Jane’s flesh and the tiny blood vessels that supplied it, measuring almost 10 inches wide by 22 inches long, pulled taut the walls of her abdomen and sutured the skin together—taking her from a size 14 down to a size 10.

“It was certainly the largest free tissue transfer that I’ve ever seen or been involved with,” Selber said.

Selber and the team then transferred Mary Jane’s abdominal graft to the other operating room down the hall, where Marian was lying on her stomach with her back muscles and fascia completely exposed. He placed the tissue from Mary Jane’s stomach on Marian’s hollowed back, and began to tease and coax the multitude of tiny arteries and veins until they were all precisely connected. The entire procedure took 14 hours.

“There is some poetic element to bringing a new level of closeness to two people whose bodies and minds were already so connected,” Selber said.

When the twins woke up from anesthesia, they both immediately asked Selber how the other was doing, before asking about themselves. Both procedures went smoothly, but Mary Jane had a slightly more challenging recovery than expected due to the 25 years of steroid medication for her pituitary condition.

“You can’t die. I can’t live with that guilt,” Marian said to Mary Jane over the phone from the room next door.

“Marian, I’m not going to die,” Mary Jane said. “I’m the stronger Catholic. You wouldn’t know how to plan my funeral.”

The Fields sisters were discharged in late July and have since returned to their home in Missouri, where they’ve regained a sense of normalcy neither had experienced since 2011.

Setting the stage

With two history-making surgeries under their belts, Selber and Gaber are pioneering a new frontier in innovative transplant and reconstructive plastic surgery at the Texas Medical Center.

Beyond the clinical significance of the Fields transplant, the entire surgical process with the twins proves that their previous collaboration during the skull-scalp-kidney-pancreas transplant “wasn’t a fluke,” Gaber said. Instead, it bears testament to the power of collaboration and continues to strengthen the need for a growing vascularized composite allograft program between MD Anderson, Houston Methodist and other institutions within the Texas Medical Center.

“It’s one of the really good examples of how collaboration in the Texas Medical Center can happen. You have the surgical expertise in a couple of institutions, the oncology expertise from MD Anderson … and the transplant expertise right across the street at Houston Methodist,” Gaber said. “It’s very exciting that we can work together to benefit more patients.”

Currently, the most common types of vascularized composite allografts involve hand and face transplants, but Selber and Gaber aim to tap into the high volume of clinical cases in the Texas Medical Center and offer creative solutions that take them off the beaten track of conventional reconstructive surgery, transplantation and cancer reconstruction.

“We’ve sort of cornered the market on really weird cases,” Selber said. “Where these unique reconstructive transplant cases fit in with certain complex clinical conditions, most doctors don’t recognize. But through this lens of understanding the interactions between reconstruction and allotransplantation, clinical solutions emerge that were previously unidentified. Hopefully, enough people have heard about this … that they’ll be able to identify us as a resource when these kinds of rare cases come around.”

Meanwhile, Mary Jane continues to inspire awe in everyone who hears about her donation to Marian. For the Fields sisters, though, it was a no-brainer.

“I’m not surprised by what she did,” Marian said. “She knows that if the tables were turned, it would be the same for me.”

After all, they are two halves of the same whole. When Marian and Mary Jane were still in the womb, the doctor listened to their mother’s protruding belly during checkups and heard one strong heartbeat. He concluded that one large baby was on the way. In reality, two hearts were beating as one. Sixty-six years later, their hearts continue to beat in perfect harmony.

“We were in sync even back then,” Mary Jane said. “We’re not just sisters and we’re not just twins. We’re best friends. What we have is special.”

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