Huge tumors are dramatic, and rare. They can also be deadly, yet research remains underfunded. Fortunately even “orphan diseases” have their champions.
By: Dr. William Tseng
“It got harder to tie my shoes,” he told me. It wasn’t just the effects of age we all suffer. Mike new something wasn’t right. He noticed he was getting full quickly during meals, and soon it became harder to take deep breaths. Everything else seemed fine. He was still working. But just to be sure, Mike went to see his primary care doctor. The doctor was concerned with his weight gain and with swelling on the left side of his abdomen. A CT scan was done, revealing that an enormous tumor had engulfed the left kidney, pushed the stomach, pancreas, and spleen up toward Mike’s chest, and moved the left colon to the right. The report concluded suspicious for malignancy, such as sarcoma.
Soft-tissue sarcomas are cancers of the connective tissues (fat, muscle, etc.) that together represent only 1 percent of all cancers in adults, with just more than 10,000 new cases diagnosed per year compared to 250,000 for breast cancer. They can develop anywhere in the body, including the back of the abdomen as in Mike’s case, or more commonly in the limbs or trunk (chest or abdominal wall). What adds to the complexity of sarcoma are the 50 to 70 subtypes, each distinct from one another in terms of genetics and behavior. Although there are exceptions, overall, sarcomas are aggressive and potentially deadly.
I met with Mike in my clinic. We looked at his CT scan together so that he could see and understand exactly what we were dealing with and what was on my mind regarding his treatment. It was only at this point that Mike fully realized how large the tumor was. Interestingly, a small portion of the tumor appeared aggressive (“high grade”) and we discussed whether it might be worthwhile to give chemotherapy first, then surgery.
“If the tumor is a multi-unit apartment complex, we can use chemo to wipe out the tenants inside and the few tenants that may have strayed elsewhere in your body before I remove the whole building with surgery,” I told him. However, Mike’s symptoms were getting worse and impacting his quality of life, so we decided to move forward with surgery.
Mike’s surgery was not easy. For these types of tumors, known as retroperitoneal sarcoma, surgery never is. For a tumor this size, we couldn’t remove it through small incisions; we had to make a big incision to get exposure. The tumor was touching upon many important organs and major blood vessels, and we had to make important decisions on what we needed to remove versus what we could keep, balancing adequate control of the cancer with potential complications. Six hours later, the tumor came out in one piece, along with Mike’s left kidney, but we had managed to save everything else around it. The final weight for this “beast” was 40 pounds—on the far end of the spectrum, but not too far for retroperitoneal sarcoma, the largest tumors in the human body.
While massive tumors understandably inspire some macabre interest among the public, they can be deadly—even with successful surgery, sarcoma is notorious for coming back and spreading to other parts of the body—yet the relative rarity of the disease makes funding research a challenge, compared to the more common cancers. Such underfunded cancers are sometimes referred to as “orphan diseases.” I am nevertheless optimistic that with further research and collaboration, the future is bright for patients with this challenging disease.
Meanwhile, two months out from surgery, Mike is cancer-free and feeling great. “Thanks, Dr. Tseng,” he says. “I can tie my shoes now.”William W Tseng, MD is Assistant Professor of Surgery at the Keck School of Medicine at USC. He obtained his undergraduate and medical degrees from Northwestern University, under the Honors Program in Medical Education. He did residency training in General Surgery at the University of California at San Francisco and then completed subspecialty fellowship training in Surgical Oncology at the University of Texas M.D. Anderson Cancer Center.
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