Sarcoma is cancer of the connective tissues of the human body. Connective tissue are the parts of our bodies that hold bodies together and make our bodies function mechanically. A few examples of these tissues are: Muscle, bone, fat, blood vessels, fibrous tissue, just to name a few. In general, cancer refers to any cell in our body that has gone out of control and has gained the ability to grow, divide, and spread without any regulation from itself or the rest of the body. When any connective tissue in our body becomes cancerous, the result is called a “sarcoma”. Because connective tissues are typically tissue types that are found in the arms and legs, and “musculoskeletal system” of our bodies, these cancers are typically treated by specially trained orthopaedic surgeons who are experts in treating conditions of the musculoskeletal system.
In this way, sarcoma is a category of cancers, rather than one specific cancer. Within the category of sarcoma, there are many specific diagnoses that are based on which connective tissue the cancer arose from. Overall there are over 100 types and subtypes of sarcoma, making the management of these tumors a specialty within itself. Sarcomas are also rare cancers compared to other cancers. The more common cancers are called “carcinomas” and are generally derived from organs in our bodies. (In reality “carcinoma” refers to cancers of glands in our bodies, which are tissues that secrete a particular product that our bodies need. Some of these gland tissues make up our organs) For comparison, there are about 10,000 new cases of soft tissue (meaning not bone) sarcomas per year in the United States, and about 3,000 new cases of bone sarcomas. This is compared to 300,000 new cases of breast cancer per year in the US, as well as nearly 250,000 new cases of lung cancer per year in the US as of 2022.
The rarity of these tumors makes the management extremely complex. This is due to a number of reasons, but among them is the fact that most physicians, even cancer specialists, have very little experience with sarcoma, given that there are very few sarcomas that develop compared to other cancers. A physician who has very little experience with sarcoma, whether they are a radiologist, pathologist, medical oncologist, or surgeon, will be at a significant disadvantage in treating these cancers, compared to a specialist in these cancers. This has been proven clinically, as studies have shown that outcomes for patients with sarcoma are significantly better when treated by sarcoma experts. This is why it is extremely important at every step of the way, for patients with a sarcoma, or with a mass that is potentially a sarcoma, to be treated by a sarcoma expert right from the beginning. Surgical management of musculoskeletal sarcoma has been the focus of Dr. Abraham’s career for nearly 20 years, and he developed Abraham Orthopaedics specifically to optimize the management of these rare tumors, using his extensive expertise and network of highly trained and sarcoma-specialized colleagues.
The first step in management of a mass that could potentially represent a sarcoma is getting adequate imaging. In general, Magnetic Resonance Imaging, or MRI, is the best modality for imaging musculoskeletal tumors. However, other types of imaging can be extremely important for adequate evaluation of a musculoskeletal or extremity mass. Each imaging modality gives the orthopaedic oncologist additional information that can all be put together to come up with a reasonable estimate of what a lesion is. For bone lesions, a plain Radiograph (X-Ray) is extremely important. A commonly quoted statistic is that an expert orthopaedic oncologist can determine close to 85% of the time what a bone tumor is likely to be by Xray alone. The xray demonstrates some important features that cannot be as easily seen on an MRI, like what the tumor is doing to the bone, and what the bone’s response to the tumor is. This helps the orthopaedic oncologist determine what the behavior of the tumor is, and therefore what the diagnosis is likely to be. For soft tissue masses, The MRI is excellent at telling us what the extent of the tumor is and where precisely it is located, but it is far more difficult to determine the aggressiveness of the lesion in the soft tissues. For this reason, a surgical wisdom pearl that Dr. Abraham can be often heard quoting is: “For a bone lesion, I can tell you 85% of the time what it is, but for a soft tissue lesion, I can tell you 100% of the time where it is”. It is important to know that orthopedic oncologists in particular receive specialty training in reading and interpreting all the imaging modalities of the musculoskeletal system, to best be able to decipher the patients imaging. This type of training is not part of the education of other types of surgeons and physicians, and is an important reason why patients with musculoskeletal cancers are best treated by an orthopaedic oncologist. The well-trained and experienced orthopaedic oncologist will be able to interpret a patient’s extremity imaging more accurately than most non-specialty radiologists. It is for this reason that Dr. Abraham will always ask for all your images on a disc, so that he can review them personally. He will never make any decisions based on the radiology report alone, because in a study that he performed, he found that non-specialist radiology readings of musculoskeletal imaging contained some sort of error or inaccuracy at least 50% of the time. In cases where there is some uncertainty in what the imaging is showing, Dr. Abraham will review the cases with a panel of carefully selected musculoskeletal radiology experts in the region, and a discussion will help determine the next best steps, be that more imaging, a period of observation, or an invasive procedure.
The biopsy is a critical part of the management of sarcoma. However, this does not mean that every lesion requires a biopsy. For benign lesions that are clearly recognizable by Dr. Abraham on imaging, a biopsy is usually not recommended. This is because every procedure, including biopsy, has risks and can have side effects, and so the decision to undergo a procedure should only be made if there is some clear benefit to getting the procedure. In the cases where the tumor is recognized by imaging alone to clearly be a benign tumor, the biopsy will not be recommended. The question is often asked, “How do you know for sure that the lesion is what it looks like?” The reality is that we can never know anything for sure, sometimes even after a biopsy, and so we use our best judgment, experience, and education to come up with the most sensible plan. In cases where the tumor appears benign but is not clearly recognizable, a period of observation followed by repeat imaging is often very helpful. In fact, we usually use this tactic for all benign appearing tumors, to make sure their behavior is what we expect. If it is, then nothing further other than continued imaging may be recommended. If there is some discrepancy, like for instance a tumor that we expect to stay stable in size shows evidence of growth on follow up imaging, then we may update the plan based on that new information and make a different recommendation at that point, such as biopsy. In cases where the tumor appears to potentially be cancerous, a biopsy is recommended. The biopsy is not only important to help distinguish benign from malignant tumors, but even more importantly to let the treatment team know precisely what type of sarcoma it is. As there are over 100 types and subtypes of sarcoma, it is important to get this information in order for the treatment team to be able to construct the best treatment plan for the patient.
How a tumor biopsy is performed
There are two types of biopsies for extremity lesions: a needle biopsy or an open surgical biopsy. The open surgical biopsy is still considered the “gold standard”, meaning it is the option that is most likely to give the most accurate answer. However, there are significantly increased risks associated with the open surgical biopsy. Some of these risks include infection, bleeding, and if the biopsy is not done properly, there is risk of spreading the tumor around locally. For these reasons, the first line biopsy technique in nearly all experienced centers in the country is the needle biopsy. This is a procedure where the surgeon or a radiologist uses some imaging guidance, such as ultrasound or CT scan, to pinpoint the tumor and place a specially designed needle into it. The needle has a chamber that can be opened and closed, and once the needle is inside the tumor, the chamber is opened to allow the tissue to enter it. Then it is closed, cutting a piece on the tumor and trapping it inside the needle. This is called a “core” of the tumor. The needle is removed and the core is emptied onto a sterile pad, and the procedure is repeated. In most cases, 5-7 cores will be taken to be considered an adequate biopsy. In some cases more samples may be taken for additional testing, outside consultations, or research purposes. The doctor performing the procedure will go over this in detail with you. Once the biopsy is completed, the patient can return home the same day.
How the tumor biopsy cores are processed
Once the cores are removed from the patient’s tumor, they are sent to the pathology lab, for preparation. The preparations include “fixing” the tissues, which prevents it from getting distorted over time, and “staining” the tissue, which highlights certain features that help distinguish one tumor from another. This process, followed by the pathologists’ review of the slides, can often take up to a week or more. Bone samples have an additional step: Before the tissue can be fixed or stained, it has to be decalcified for up to 5 days, which removes the calcium from the bone and turns it into a more manageable consistency which can then be cut and stained normally. When you add the decalcification time to the regular processing time, it can amount to 10-14 days prior to getting a result from a bone tumor! This is unavoidable and is the same regardless of where the biopsy is performed. It is important to note that this is very different from biopsies of other parts of the body, like the breast, in which a biopsy results may be returned in a few days. Because many of the radiology technicians are much more familiar with these cancers, they may unknowingly tell the patient that their sarcoma biopsy result will be back in 2-3 days. This creates anxiety and frustration when the patient later finds out that in reality it will be 10-14 days. The bottom line is that you should trust the information given to you by your sarcoma doctor, who has the most experience dealing with your type of problem
Once the slides are finally ready, they are sent to the pathologist to review. This is an absolutely critical step in the management of sarcoma. Although many sarcomas are treated in similar ways, each one has its particular nuances or “personality” that makes it different from the others. It is critical for the treatment team to know what these nuances are, which comes from having the correct name of the tumor. Since sarcomas are rare to begin with, and there are over 100 subtypes, and since most community pathologists will not encounter many sarcomas, it is absolutely critical to have an expert sarcoma pathologist evaluate the slides. Saromas all come from similar types of tissue (ie connective tissue) so at first glance nearly all 100 subtypes look relatively similar, which further complicates the pathology evaluation. For this reason, the highly trained and specialized eye of a sarcoma pathologist is needed in order to ensure that the patient gets the best treatment plan for their particular tumor.
For this reason, it is best to have the procedure performed at an institution recommended by your orthopaedic oncologist. Dr. Abraham has developed orthopaedic oncology programs in several institutions in the Philadelphia area, and has hired and helped train a number of pathologists in the region to be able to expertly diagnose these rare tumors. In addition, he has maintained connections with some of the best sarcoma pathologists in the country with whom he trained, and frequently has those pathologists do a second confirmatory review of his patients slides. These national colleagues of Dr. Abraham have made commitments to quickly turn around his consult slides so as to avoid significant delays usually associated with second opinion pathology reviews. This ensures that all the patients that Dr. Abraham treats have the absolute highest level of pathology evaluation available, which makes all subsequent treatment much more effective.
Where to get a tumor biopsy
The question frequently comes up: “Can I get my biopsy done closer to home?” The short answer is that although you can, it is usually not advisable. This is because even if the radiologist is an expert at performing biopsies and can do it without contamination, the likelihood is that there is not a sarcoma pathologist at that institution. This can not only cause an error in diagnosis, it can also cause significant delays, because after the 10-14 day pathology review time has eclipsed, another period of waiting will have to happen while the slides are sent to an expert sarcoma pathologist for review. These consultations often take far longer than the original pathology review, because the consult pathologist is not the primary pathologist, and so significant delays can occur. For this reason, in most cases, it is advisable to get the biopsy performed at the institution that Dr. Abraham recommends, even if there is some travel involved. What we want for you is the best outcome possible, and that is what our recommendations are based on.
Diagnostic review of pathology report
The pathology report can be confusing and is best interpreted by Dr. Abraham, who has familiarity not only with the terms, but also the nuances of the specific language used by the pathologists. For many of the pathologists that Dr. Abraham recommends, he has been working with them for nearly 20 years! This means he knows exactly how to “read between the lines” and get to the bottom of what the report is saying. In recent years there has been a trend at many facilities to release critical reports such as pathology reports to the patient via portals even before the ordering physician has seen it. This can create a lot of anxiety, frustration, concern, and fear on the part of the patient, because they don’t know exactly what the report means. Dr. Abraham reviews every pathology report in the office personally face-to-face with the patient. This allows him to not only give a detailed explanation of what the report means, but also to answer any questions and discuss the treatment plan. This is by far the best way to discuss the pathology reports. If you find that you have gotten your pathology report in advance of Dr. Abraham, our suggestion would be to call the office and let us know that you have the report, but would like to make your appointment to go over it with Dr. Abraham (if you don’t already have one). We always try our best to prioritize this type of office visit, and we are usually able to review the report with you within a few days of it being released. If hardships or extenuating circumstances exist, Dr. Abraham may go over the pathology report with you on the phone or via telehealth, but in general he will require you to come to the office for the best possible communication of the result.