Twin Saves Sister from Rare Cancer with Radical Procedure

James Radke

In March 2017, in the office of Jesse Selber, M.D., microvascular reconstructive surgeon at the University of Texas MD Anderson Cancer Center in Houston, Texas, Marian Fields inquired about her options to remove a rare skin cancer called pexiform fibrohistiocytic sarcoma.
In regard to surgical possibilities, she was limited. Almost all of her back had to be removed, and, as small woman, finding enough soft tissue from other parts of Marian’s body that could be used to patch together a back would require a miracle.
Enter Mary Jane, Marian’s twin sister.
This past week, Rare Disease Report spoke with Dr. Selber, and he explained that in his first encounter with the 2 sisters during a consult with Marian about her options, it was clear that the 2 have had several medical problems that make them looks slightly different. When Dr. Selber mentioned that there was not enough tissue on Marian to transfer to her back, he did not think the other person in the room, Mary Jane, was a possible tissue donor.
When Mary Jane suggested that the procedure take some of her tissue, Dr Selber politely asked the 2 women if they were related. Because of the type of surgery, only identical twins with the same DNA would be a good match. Mary Jane replied by saying that they were identical twins, and Marian’s options suddenly looked less bleak.
Once confirming the 2 were genetically compatible, surgery was planned that would involve removing the cancerous tissue from Marian’s back, testing the remaining intact tissue to make sure no cancer cells were remaining, removing a large section from Mary Jane’s stomach, and transplanted it to Marian’s back.
A 2-part surgery was conducted over a 4-day period. In addition to Dr Selber’s team, the surgery involved a group of oncology specialists led by Keila Torres, M.D., whom RDR also spoke with about the procedure.

Dr. Selber and his team during the surgery

“My job was to make sure we would remove the tumor completely,” said Dr Torres. This was not the first time this cancer had been excised from Marian’s back, as the tumor first appeared in 2012 and several times after that, each time being removed by a surgeon. Unfortunately, none of the doctors to have previously worked on the twin excised the cancerous tissue in its entirety.
Pexiform fibrohistiocytic sarcoma is a very rare cancer and removing it requires going beyond the cancerous tissue to the margins to make sure all remnants of the cancer are removed. Otherwise the cancer will come back like it did with Marian, to the point that it covered most of her back.
Dr. Torres noted that due to the extreme rarity of this cancer (150 cases in the U.S.), most oncologists would not be aware of its idiosyncrasies. It wasn’t until Marian was sent to MD Anderson, a large research center familiar with that rare cancer, that she was treated properly. 
“We wanted at least 2 centimeters of clean margin and I did that because of her history of so many recurrences. The tumors have a propensity to come back,” said Dr Torres. Ultimately, tissue from Marian’s neck to lower back was removed and areas where samples were taken for biopsy were marked and those samples were taken away to determine by numerous doctors to confirm that no cancer remained.
Dr Torres said, “The surgery had to be done in 2 days because we wanted to make sure that the samples were negative.”
If even 1 cancerous cell remained, the cancer would come back and all of the efforts performed would be for naught. The lab results, however, confirmed that all the cancer had been removed and then the team was ready to begin part 2 of the procedure – adding Mary Jane’s tissue to Marian’s back.
Dr. Selder said: “So, the first part of the surgery was removing the tumor but also setting up the blood vessels. There are not a lot of blood vessels in the back big enough so we had to bring in some blood vessels from other areas.”

For the transplant, the sisters were in adjacent rooms. “We started on Mary Jane, procuring her tissue and with a staggered start, we began on Marian to get her vessels prepared and moving some tissue around,” said Dr Selber.  Several hours later, the tissue, approximately 50 x 20 cm, from Mary Jane’s tummy was free and they carried it over to Marian and attached the tissue and the blood vessels. Dr. Selber noted, “that process took about 13 hours.”
Today, the twins are doing very well. Mary Jane was discharged 5 days after surgery and Marian stayed in the hospital for about 10 days, then moved to a nearby rehabilitation center. Dr. Selber noted there were positional issues early on in which some of the tissue was turning blue but that has been resolved. Last week, the twins were seen by the MD Anderson team and were clear to go back home to Missouri.
When asked if anything could have been done early on in course of Marian’s illness to prevent this radical procedure, Dr Torres noted that because of the extreme rarity of the cancer involved, it is very unlikely that the doctors who treated Marian were aware of its aggressive nature. Approximately 40-50% of these cancers relapse, but that is likely because the cancer is not completely removed during the surgery.
Dr Torres said: “This cancer has multiple nodules all interlaced. So if the doctor is not aware of how the tumor develops and the importance of having a wide margin, excising beyond the tumor, and taking normal tissue, and that is what happened to Ms Fields. She had 6 unsuccessful surgeries.”
Regarding what advice to give clinicians who come across this type sarcoma, Dr. Torres was quick to say that they need to do a biopsy. “If there is any suspicion of a mass it should be biopsied and that is with a needle, not a knife. Before going to surgery, you need to know what kind of disease you are dealing with.”
Dr. Torres added that if the biopsy confirms it is a sarcoma the hospital is not familiar with; they should reach out to larger research centers that may be more familiar with rarer types of cancer. If the pathology confirms it is a rare cancer, “I strongly recommend that the patient is referred to a center that has enough experience with that cancer; that will benefit the patient. They will be evaluated by multiple specialists and typically, these type of rare tumors are treated using a multidisciplinary approach.“
Below is a video produced by MD Anderson in which Dr. Selber talks about this unique procedure:

Images provided by Dr. Selber and the MD Anderson Cancer Center.

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