Sarcoma – An Introduction, with Dr. Robert Maki

Last Updated: July 5, 2017

Dr. Robert Maki talks about what sarcoma is and the scientific advances in the treatment of different types of sarcoma. In addition, he talks about finding a sarcoma expert.

More Information

Guide to Sarcoma – Soft Tissue

Guide to Sarcoma – Specific Organs

Full text transcript

Cancer.Net®: Doctor-Approved Patient Information from ASCO®

Sarcoma: An Introduction

What is Sarcoma?

Robert Maki, MD, PhD, FACP: Sarcoma is a tumor of connective tissue, and that involves tissues such as the bones, muscles, cartilage, fat, everything that holds us together.

So that’s to be distinguished from the most common cancers, such as breast, lung, colon and prostate cancer.  In those cases, those are called carcinomas.  Those are tumors of glands or of the lining of an organ.

And perhaps the easiest way to think about this is the intestine.  The lining of the intestine, such as the colon, is the part of the colon that gives rise to the cancer.

However, if a tumor arises from the wall of the colon, that's a sarcoma.  That's what distinguishes a sarcoma from the more common cancers such as carcinomas.

We classify sarcomas as a group of cancers.  However, it is a large family of tumors and there are at least 50 different subtypes of sarcoma of the soft tissue and about 20 different versions of sarcoma of bone.

So between those two, that's about 70, or maybe even more, different diagnoses, and that does not even include some of the specific molecular subtypes of each of these cancers.  So, as a result, it's a complex family of diseases.

Questions to Ask Your Oncologist

Dr. Maki: What are some of the first things you should know when you have a sarcoma?  Well, you should try and figure out what the stage of the tumor is, how aggressive is it and what is the risk of the tumor coming back.

Most of the time, sarcomas present as a single mass or a lump, and the idea there is how do you treat these things.  It’s usually surgery and sometimes radiation and sometimes chemotherapy.

So your questioning should include, you know, what will my treatment entail?  How long will I be treated?  And what is the follow up for that treatment after it's finished?

Treatment Advances for Sarcoma

Dr. Maki: Sarcoma is treated with three different types of treatment, and maybe even a fourth now, depending on how research studies turn out.

There's really no cure for most sarcomas without surgery, so surgery is nearly always a necessity for treatment of a sarcoma.

For tumors that are larger, more than about two inches in size, we usually talk about using radiation as well, because this will decrease the chance of the tumor coming back right where it started.

For some other patients, and it really depends on which of these subtypes of sarcoma are involved, there's some risk of the tumor showing up again elsewhere in the body.

And this is an important difference between sarcomas and some other cancers.  Sarcomas usually don't go to lymph nodes as other cancers will commonly do, but they do like to travel directly to the lungs.

So for some people we will offer chemotherapy to try and prevent the tumor from coming back elsewhere in the body as well.

Treatment Advances for Sarcoma

Dr. Maki:  What's on the horizon for sarcoma therapy?  Just as with other cancers, we're looking at a variety of different classes of medications.  And some of these so-called immune checkpoint inhibitors are of interest because they can be given -- just be given directly to people to activate preexisting immune responses to try and attack their cancers. 

So we're just beginning to investigate what's happening with those compounds in different types of sarcomas.

At the same time, there's an interest in kinase inhibitors, some novel therapeutics that way that have been developed over the last several years for a variety of cancers, and these are -- that pursuit is continuing right now as well.

And, then, perhaps, finally, epigenetic drugs, drugs that don't really damage the DNA like a lot of our traditional chemotherapy drugs do. 

These are drugs, rather, that affect some of the proteins or some of the other modifications on DNA.  Those have some possibility of some use in sarcomas, specific subtypes, again, but those are very much in their infancy in terms of being tested.

Finding a Sarcoma Expert

Dr. Maki:  We always recommend that you at least obtain a consultation with an expert center because of the heterogeneity of sarcomas.  Because there are so many different subtypes, the treatment for each one can be as different as the treatment is of breast cancer from colon cancer.

So it really pays to see an expert, at least once or even repeatedly as treatment goes on, to ensure that the treatment is the best it can be.

Oftentimes, these experts will be the people who have those new research studies that really may have an impact, especially if the tumor has come back.

Where to Get More Information

Dr. Maki:  Patients can certainly take a look at a variety of resources to find out more about sarcomas.  One of the most reliable sources that's been vetted by professionals is the Cancer.Net website. 

There's a nice section on sarcoma, and it gets into some of the subtypes of sarcoma, from some of the more common ones to the rare ones, and it's certainly worth a look.

[Closing and Credits]

Cancer.Net®: Doctor-Approved Patient Information from ASCO®

ASCO's patient education programs are supported by Conquer Cancer Foundation of the American Society of Clinical Oncology. ConquerCancerFoundation.org  

Special Thanks:

Dr. Mary Wilkinson, Dr. Raymund Cuevo, and the staff at Medical Oncology & Hematology Associates of Northern Virginia

Carolyn B. Hendricks, MD, The Cancer for Breast Health

Hasbro Children’s Hospital

Helen F. Graham Cancer Center at Christiana Care Health System

The Adele R. Decof Comprehensive Cancer Center at The Miriam Hospital. The Miriam Hospital is a teaching hospital of The Warren Alpert Medical School of Brown University

Video Footage and photography courtesy of:

St. Jude Children’s Research Hospital Biomedical Communications

Moffitt Cancer Center

University Hospitals Case Medical Center Seidman Cancer Center

The opinions expressed in the video do not necessarily reflect the views of ASCO or the Conquer Cancer Foundation.

Requests for commercial use of this video should be submitted to permissions@asco.org.

© 2017 American Society of Clinical Oncology®. All rights reserved

Sharing and personal publication of this video indicates your consent to the Terms of Use, viewable at: http://www.asco.org/VideoDisclaimer