Pleomorphic Liposarcoma: What Is It & How Is It TreatedheadingContent
Dr. Adrian Legaspi, an internationally renowned surgical oncologist recently discussed pleomorphic liposarcoma, a rare tumor which can develop anywhere there is fat in the body. Former Toronto mayor, Rob Ford, had passed away from the disease. So what is it, how is it treated and what are the factors for developing this disease?
What is pleomorphic liposarcoma?
Pleomorphic liposarcoma is a malignant tumor that usually arises from fat in the body and is most commonly found in the abdomen. The other sites where it can develop are the extremities, the buttocks or the armpits. The term pleomorphic describes the tumor’s characteristics where cells have distinct and different densities within one mass. Malignant transformation happens at a different pace in the areas of the tumor where there is fat.
Why is pleomorphic liposarcoma so rare and fast growing?
Pleomorphic liposarcoma has a very broad range and rate of growth.
What determines the rate of growth? This is currently unknown, but we know the main reasons cancer occurs is a consistent mutation in the genes that regulate growth. Fat itself is not a tissue that divides rapidly. By contrast, the inner lining (mucosa) of the colon exhibits a rapid turnover of cells, which in part may be responsible for the fact the cancer of the colon is a more common type of cancer.
We still haven’t figured out what causes the gene mutation and why some pleomorphic liposarcomas behave more aggressively than their counterpart, myxoid liposarcomas. Myxoid liposarcoma is a rare variant of liposarcomas and constitutes about 18 percent of all liposarcomas.
Are there any warning signs for this type of pleomorphic liposarcoma?
The most difficult to detect is the retroperitoneal liposarcoma of the abdomen. The patient can have a tumor growing slowly for months. The patient may go see a doctor because of a vague abdominal pain or other unrelated abdominal symptoms and discover the tumor incidentally. When it comes to liposarcoma of the extremities it is a common occurrence to find a difference in the size of the thigh, the calf or the biceps when compared to the other extremity. This resultant asymmetry is a product of a tumor growing gradually. Sometimes the liposarcomas can start as a benign lipoma.
When you see a soft tissue tumor that’s under the skin become larger in size, or becomes firmer or harder, that is a strong indicator there has been a transformation of the nature of the mass and should lead to a physician consultation.
How can pleomorphic liposarcoma be treated?
The most effective and one of the best treatments is surgery to resect completely. After surgery, depending on the size of the tumor, radiation may improve the chances of cure by minimizing the possibility of recurrence. However, radiation may not be very effective if it’s in the retropardium but could be more effective if it’s in an extremity. Chemotherapy is usually reserved for tumors where you try and shrink it down before the operation or tumors that are not suitable for resection.
The truth is chemotherapy may be the least effective tactic of all the treatments that we have right now. As our knowledge improves in recognizing the common genetic mutations, there’s a new generation of systemic agent called PERK kinase inhibitors that may be useful once we recognize the type of mutations pleomorphic liposarcomas have.
How can it be removed, is there a certain procedure you use? Do you use robotic surgery?
Typically, it’s an open surgery. At the time of the surgery, the surgeon will customarily take samples of the tissue from the resected periphery of the tumor, to make sure there is no microscopic extension. Depending on the size of the liposarcoma, location in the abdomen, and skill and training of the surgeon, selected liposarcomas might be suitable to use minimally invasive robotically-assisted surgical techniques.
Former Toronto mayor, Rob Ford, passed away after his fight with this disease at the age of 46. Most people who have died from this disease have an average age range between 50 and 65 years. Could there be factors to why he might have developed pleomorphic liposarcoma at a younger age?
Mr. Ford had significant behavioral issues that were widely publicized in the media. The fact that he developed this malignancy at a young age, suggests the mutations that usually produce the tumor were accelerated or hastened by drinking or smoking. Obesity can make it difficult to detect this tumor. In fact, it is likely the liposarcoma had been present for some time but it was never detected. Not infrequently, the size of the abdomen is attributed to obesity. I think that lifestyle does play a role and for Mr. Ford, there were several behavioral issues that put him at a higher risk for a cancer.