This open letter is addressed to all Sarcoma patients and written by the founder of the Northwest Sarcoma Foundation, Dr. Ernest "Chappie" Conrad on December 3, 2017.
Dear Sarcoma Friends,
Our support for our Sarcoma patients and families is critical because Sarcomas are an uncommon tumor that are very difficult to diagnose and treat. Approximately 15,000 to 20,000 sarcomas occur each year in the U.S., which is a small incidence compared to other more common solid tumors (breast, lung, prostate, colon, etc.). Sarcomas are the only solid tumors that occur in any age group and in any anatomic location of the body, making its timely diagnosis difficult even for the best healthcare providers. The fact that most sarcomas present as a painless soft tissue tumor make the diagnostic challenges even greater. Lastly, sarcomas are difficult to diagnose by either MRI or under the microscope, and there are more than 50 pathologic/histologic subtypes that require an experienced, sarcoma pathologist for an accurate diagnosis. Scientific reviews of sarcoma pathologic diagnoses have documented histologic/pathologic “errors” in grading (growth rates) and sarcoma subtypes of greater than 30% and another 30% of patients present for treatment after inadequate, contaminated surgical resections, referred to as “ Whoops procedures.” Therefore, the timely and accurate initial biopsy and resection are both challenging and critical, because the first treatment may determine the final patient outcome.
A sarcoma patient, by the nature of their tumor, has a risk of local recurrence at the location where the tumor was first diagnosed, and a risk of metastasis via their bloodstream. Sarcomas are divided into fast-growing, “high grade” and high-risk subtypes that have a significant risk of spreading elsewhere (metastasizing to the lungs, lymph nodes or liver), or “low grade” sarcomas that have a much lower risk of metastasis. Patients with high grade, fast-growing subtypes have an approximate 50% risk of metastasis, while low-grade sarcoma patients have a 10-20 % (or lower) risk of metastasis. High grade and high-risk sarcomas get chemotherapy and low-grade sarcomas typically do not.
Despite being an unusual and difficult tumor to diagnose, there are effective treatments even for the most aggressive high-grade Sarcoma subtypes based on “molecular“ targets that are specific to the subtypes. In addition, there are new immunotherapy protocols that are directed towards the patient’s own immune system and towards their sarcoma cells. All of these treatments require an accurate and timely diagnosis, in addition to accurate, carefully planned surgical resection with adjuvant radiation therapy. Making these challenging diagnoses and treatment decisions requires a dedicated, experienced, “Multi-Discipline“ Sarcoma team that includes Medical Oncology, Sarcoma Surgeons, experienced Radiation Therapists, pathologists and radiologists.
The “average” 5-year survival rate “for high-grade Sarcomas is approximately 50% if the tumor has not metastasized, and 25% if it has metastasized, usually involving a lung metastasis. A patient’s subsequent prognosis and survival will be determined by the following important factors:
Tumor “Grade” (or growth rate ) and “subtype“. Tumor size and location. Tumors greater than 7-8 cm’s are more difficult to treat as are pelvic, abdominal and chest/pericardial tumors. Metastatic or “Recurrent “ tumors or tumors that have failed their first surgical resection, chemotherapy or radiation therapy.
My “ Sarcoma Advice” to anyone dealing with a Sarcoma treatment issue:
1 - ALWAYS BIOPSY YOUR TUMOR BEFORE TUMOR EXCISION/RESECTION!!
Get an expert and experienced Sarcoma opinion before resection or chemotherapy. An accurate diagnosis and review of your case is more important than a quick, convenient, but contaminated resection. Remember that most sarcoma treatment decisions are made on a monthly, not a weekly basis, because even high-grade sarcomas usually show an increase in size or metastasis every three to four months, not every three-four weeks.
2 - GET A SARCOMA EXPERT OPINION BEFORE YOUR TUMOR RESECTION
Don’t have a “ Whoops “, contaminated surgery. Find a clinic that has sarcoma expertise in your city or nearby. The best Sarcoma Clinics have a dedicated weekly Clinic and Conference (attended by Medical Oncology and a Sarcoma surgeon). If you have problems getting an appointment into the clinic via their scheduling process, ask to speak to an RN or a manager in that clinic about your situation. Most Sarcoma Clinics will be able to see you within 7 days, or your case may also be reviewed remotely by many Sarcoma clinics without going to their clinic!
3 - CHAMPION YOUR CARE
Take a family member or friend with you to your appointment. Write down your questions before the appointment. A good sarcoma provider will give you an understanding of the usual behavior of your tumor and your treatment choices
(resection vs. chemo vs. radiation). If in doubt regarding your treatment plan or communications ask for a second opinion. Review National Sarcoma guidelines on the NCCN - National Comprehensive Cancer Network website or via the NIH Comprehensive Cancer Center Network.
If you are a resident of Washington, Oregon, Alaska, Montana, and Idaho the Northwest Sarcoma Foundation will support you and your family with our “Helps” program. This is for all patients that have their sarcoma diagnosis confirmed. A $500 check will be provided to the patient to assist with getting an expert opinion or paying for living expenses. Contact the Northwest Sarcoma Foundation for more information.
Dr. Ernest “Chappie” Conrad
Founder & Board Member, Northwest Sarcoma Foundation