Go See a Sarcoma Specialist!

Article Contributed By Sarcoma Alliance Medical Advisory Board Members:

William Tseng, MD, Surgical Oncology, University of Southern California, Keck School of Medicine, Los Angeles, CA (pictured above left)

Raphael Pollock MD PhD, Surgical Oncology, Ohio State University, The James Comprehensive Cancer Center, Columbus, OH (pictured above right)

               Soft tissue sarcomas (STS) are rare and complex cancers that should be treated by a specialist and ideally, at a dedicated sarcoma center. In total, STS represent only 1% of all cancers in adults and within STS are over 50-70 different subtypes, each with distinct genetics, behavior and nuances in treatment. In fact, we have previously published an analogy comparing STS subtypes to wine: “while most physicians know of red versus white, to the true sarcoma enthusiast, there are many more subtleties that deserve attention.” [1] STS most commonly develop in the limbs (60%) and then back of the abdomen (“retroperitoneum,” 20%), but these tumors can also develop in any location in the body, adding to the complexity of these cancers. Although there are exceptions, STS are overall potentially deadly cancers, emphasizing the importance of appropriate treatment by a specialist.

               First and foremost is having the correct diagnosis of STS and perhaps more importantly, the specific subtype of STS. These determinations, which impact subsequent decision-making, are made by close microscopic examination of tumor tissue obtained by either a needle biopsy or through surgery. Given the rarity of these cancers, making the correct diagnosis frequently requires a pathologist with a background or formal training in STS. In some cases, additional tests may be necessary to detect unique genetic features that definitively identify the tumor as one subtype versus another. Without an accurate diagnosis of STS and the subtype, the incorrect course of treatment may be given, sometimes unintentionally, to the patient’s detriment.  

               Having imaging studies interpreted by a radiologist with experience in STS is also important. In fact, even without pathology information from tumor tissue, an STS radiologist can sometimes make an  accurate diagnosis based on body location and features of the tumor seen on the imaging studies. Alternatively, the pathologist may even need to review the imaging studies with the radiologist to finalize their diagnosis being made based on tumor tissue. With a diagnosed STS subtype, the radiologist can then suggest additional imaging studies that may be needed to determine if the tumor has spread (“metastasized”) to other body locations. Many STS subtypes should be further studied (“staged”) with a chest CT, some may need a whole body PET scan, a select few need a brain MRI, while some do not need any further imaging at all since the tumor will never spread.

               Surgery is the main form of treatment for the vast majority of STS that have not spread. Although many surgeons can technically remove STS, it isn’t always simply just about “cutting or getting it out.” [2] The surgeon must balance knowledge of these rare cancers and the expected subtype-specific behavior with the proposed extent of surgery, considering the potential for complications and impact on quality of life. Surgery for STS is truly intellectual in the sense that often the decision-making before and during the actual operation can become as critical as the surgeon’s technical ability. For example, the extent of surgery – how much of the adjacent normal tissue do we need to take? – is a common question for which the answer depends largely on the STS subtype. Adequate surgery for STS can be a major operation. In fact for retroperitoneal sarcomas which develop in the back of the abdomen, these can be extremely challenging operations that may involve simultaneous removal of multiple organs and major blood vessels; or… for the appropriate patient and STS subtype, an adequate operation may just be removing the tumor alone and preserving organs and vessels! These complex decisions are best made by not just a cancer surgeon (surgical oncologist) but ideally, a cancer surgeon who specializes in STS.

               In some patients, the best treatment course for their STS subtype may include more than just surgery. Radiation therapy can improve local disease control, especially for STS in the limbs, which may potentially spare the patient of a more morbid or disfiguring surgery such as amputation. Systemic therapy, which includes chemotherapy and other drugs that reach the entire body, can improve both local and distant disease control. In patients whose tumors have already spread, systemic therapy is the front line treatment. Radiation and systemic therapy are given by radiation and medical oncologists, respectively. In STS, having specialists for these therapies is critical to match a patient’s subtype to the appropriate therapy. This is especially relevant for systemic therapy. The perception for a general medical oncologist that “chemotherapy doesn’t work in sarcoma” may take on a different light for a STS medical oncologist who has intimate knowledge of a drug that targets a specific genetic change found only in a specific STS subtype (“targeted therapy”). As an added advantage, these drugs may also have fewer side effects from less non-specific damage to normal body cells. To a general medical oncologist, many of these drugs are ones “I’ve heard about” (but not given) since they may not be used in more common cancers.

               Therefore from diagnosis to treatment, the STS patient benefits immensely from having specialists at every step. Not surprisingly, STS specialists often work together within an organized sarcoma program which is frequently - but not always - at an academic center. A sarcoma program offers patients direct access to the combined experience and ideally, expertise of specialists from each discipline: pathology, radiology, surgical, radiation and medical oncology. The cornerstone of a good sarcoma program is indeed multidisciplinary collaborative meetings, often referred to as “Tumor Boards.” In these meetings, individual patient cases are discussed and input is given by each STS specialist with the goal of achieving a mutually agreed upon, best course of treatment for that specific patient. These Tumor Boards should take place on a regular (e.g. weekly) basis, be focused on STS and can be led by, but should not be dominated by any one specialist. A good sarcoma program also has the advantage of offering access to STS-specific clinical trials and research studies. In fact, a firm indicator of a true sarcoma specialist and program is frequent medical publications of new research findings, to advance our collective knowledge of these rare and complex cancers.

               Not surprisingly, there is ample published research which demonstrates that outcomes are clearly better when patients with STS are seen by specialists and at sarcoma centers [3,4]. For these rare and complex cancers that arguably most non-sarcoma physicians have very little knowledge of, there seems to be no legitimate reason not to see a specialist. The often unfortunate reality is that with inappropriate STS treatment by a non-specialist, subsequent treatment even by a specialist can often become more challenging if not impossible or no longer effective. Alternatively, with lack of understanding of STS, patients may be given treatment that is either inadequate or overly aggressive. For all of these reasons and simply the reassurance of an already anxious STS patient - despite possible personal, financial/insurance or geographic constraints – Go See a Sarcoma Specialist!

References

[1] Tseng WW, Pollock RE, Gronchi A. The Trans-Atlantic Retroperitoneal Sarcoma Working Group (TARPSWG): "Red wine or white"? Ann Surg Oncol. 2016 Dec; 23(13):4418-4420

[2] Tseng WW, Pollock RE, Gronchi A. Retroperitoneal sarcomas: Big tumors that involve more than just "Getting it Out". J Surg Oncol. 2018 Jan;117(1):5-6

[3] Gutierrez JC, Perez EA, Moffat FL, et al. Should soft tissue sarcomas be treated at high-volume centers? An analysis of 4205 patients. Ann Surg. 2007 Jun;245(6):952-8.

[4] Bonvalot S, Gaignard E, Stoeckle E, et al. Survival impact of surgical management in reference centers for retroperitoneal sarcoma: A nationwide study of FSG-GETO and NETSARC. ASCO 2018 Annual Meeting (abstract)

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