Twenty-pound tumor sheds light on liposarcoma, rare form of soft tissue cancer

But such was the case for the retired Staten Island resident, husband, father and grandfather. Lingering pain from a fall last winter prompted Mr. Bollettieri to visit his local primary care physician, Dominic Pompa, MD. But the CAT scan of his lower spine showed much more -- a clearly defined, substantial growth in his left retroperitononeum, the rear, anatomical area of the body from the rib cage to the hip. In fact, the mass was so large it prevented a definitive image of its origin or its impact on other vital organs.

Mr. Bollettieri was quickly referred to Joseph Raccuia, MD, Clinical Assistant Professor in the Department of Surgery at NYU Langone Medical Center, with particular expertise in cancers of the hepato-biliary and gastroinstestinal regions. Even Dr. Raccuia, a veteran of many extraordinary cases, was taken aback.

"You rarely come across tumors of this size and magnitude that are, for the most part, asymptomatic, particularly given Mr. Bollettieri's physical stature," Dr. Raccuia said, referencing to the patient's tall, slightly slender build. "One thing I can say with almost complete certainty: The fall that he took last winter saved his life."

Because the tumor took up so much space in Mr. Bollettieri's retroperitoneum, Dr. Raccuia suspected it possibly to be the result of kidney cancer. He asked his NYU Langone colleague William Huang, MD, Assistant Professor of Urologic Oncology and an expert in genitourinary malignancies, to consult on the case. Following extensive testing and back-and-forth discussion of a possible root cause, the two physicians determined that is was either a kidney cancer or a form of sarcoma, potentially a liposarcoma, a very rare form of cancer that often results in large, bulky fat-cell malignancies in deep soft tissue, such as inside the thigh or in the retroperitoneum. A final determination would only be made once they were able to examine it close up.

Drs. Raccuia and Huang decided to dissect the tumor as a team. They determined beforehand that surgery would almost assuredly require the removal of Mr. Bollettieri's left kidney and possibly other organs as well. Teamwork proved to be the right call. It was, indeed, a liposarcoma. While both physicians worked to remove the tumor, Dr. Huang also removed Mr. Bollettierri's kidney and left adrenal gland, while Dr. Raccuia removed his spleen -- all organs that Mr. Bollettieri can survive without. In total, over 20 pounds of tumor and various affected organs were taken from the patient!

"It was an amazing procedure, one of the largest and most solid tumors I have ever encountered in my surgical career," says Dr. Huang. "We know that liposarcomas can be quite large and very dense because of their physical composition of fatty tissue and, in some cases, even bone. But what we removed from Mr. Bollettieri was unprecedented, even for this type of malignancy."

Dr. Raccuia added,"What also made this liposarcoma even more uncommon was that there were characteristics of osteosarcoma and chondrasarcoma as well -- elements of cartilage and bone within the tumor."

Mr. Bollettieri will be carefully monitored every three months for the rest of his life. Although CAT scans from his first post-surgical visit to NYU Langone oncologist Gerald Rosen, MD, showed no sign of tumor recurrence, this is often the reality with liposarcomas.

"Mr. Bollettieri benefitted tremendously from Drs. Raccuia's and Huang's aggressive surgical approach, so no post-surgical chemotherapy or radiation is required at this time," he said. "However, we have to keep a watchful eye on Mr. Bollettieri, given his age and what we know about tumor recurrence with this type of cancer."

So, what does the patient have to say about his extraordinary experience? "I owe so much to my NYU physicians," Mr. Bollettieri said, reflecting that this past Father's Day had special meaning to him. "To still be here with my family… I can't put my gratitude sufficiently into words. I am so lucky to be alive."