Although rare cancers don’t occur often, they can affect people of all ages and genders. Greater awareness of rare cancers may lead to earlier diagnosis and management, and potentially better survival rates. Consider these facts about soft tissue sarcomas, one type of rare cancer.
Understanding Rare Cancers
Four facts to know about one type of rare cancer, soft tissue sarcomas
(Family Features) Although rare cancers don’t occur often, they can affect people of all ages and genders.
A rare cancer is defined as fewer than 15 new diagnoses per 100,000 people per year, according to the National Cancer Institute (NCI). Additionally, as noted by the American Cancer Society (ACS), the 5-year survival rate is lower for people diagnosed with a rare cancer than for people living with more common cancers. Greater awareness of rare cancers may lead to earlier diagnosis and management, and potentially better survival rates.
If you have recently been diagnosed with STS, it’s important to ask your doctor for more information about the specific sub-type you have. For example, if you received a diagnosis of undifferentiated sarcoma, ask your doctor for an integrase interactor-1 (INI1) test to see if you have a rare STS called epithelioid sarcoma (ES). (See sidebar for more on ES.)
Learning More About Epithelioid Sarcoma
A rare type of STS, epithelioid sarcoma (ES) accounts for less than 1% of all STS, which themselves account for approximately 1% of all cancers, according to research published in “Archives of Pathology & Laboratory Medicine.” ES can present as a lump or sore on the skin.
Notably, more than 90% of ES tumors do not express the INI1 protein, which when present acts to suppress tumor growth. INI1 loss plays an important role in the diagnosis of ES, according to researchers with “The American Journal of Surgical Pathology.”
Data from the NCI indicates that approximately 150-200 people in the United States are diagnosed with ES each year. Research published in “The Journal of Clinical and Aesthetic Dermatology” found the disease often occurs in young adults in their 20s and 30s. Because most ES patients are adolescents and young adults, there is a gap in the unique psycho-social needs for this patient population, including resources for patients who miss school while undergoing treatments, as well as fertility considerations later in life.
If you or someone you love is living with ES, you can find resources, information and the real-life perspective of an ES survivor at ESsentialsforES.com.
Content courtesy of Epizyme, Inc.
Photo courtesy of Getty ImagesSOURCE:
A Rare and Life-Threatening Disease with Common Symptoms: Highlighting Chronic Thromboembolic Pulmonary Hypertension (CTEPH)
(BPT) - Determination. Strength. Resilience. These are just a few qualities that may characterize people around the world living with pulmonary hypertension (PH), a sometimes debilitating disease of the lungs. But a particularly rare form of the disease, called chronic thromboembolic pulmonary hypertension (CTEPH), is often misinterpreted by patients and doctors alike.,
November marks PH Awareness Month, designed to shine a spotlight on the disease — and generate support for patients. On November 21, patient advocacy groups, healthcare professionals and caregivers focus specifically on CTEPH and the unique needs of this patient population.
Recognizing the signs and symptoms
PH diagnosis is frequently delayed by several months or years. This is because the most common symptoms, including breathlessness, fatigue and dizziness, are also associated with many other conditions. It is important to note that CTEPH, a subset of PH, is treatable and potentially curable through surgery when properly identified.,
CTEPH also has a connection to a much more common condition, called pulmonary embolism (PE), a blood clot in your lungs. Many people don’t realize that as many as 1 out of every 25 of previously treated patients with PE that does not resolve after three months of using blood thinners could go on to develop CTEPH*.,,
Spreading the word for healthcare professionals
In addition to raising awareness among potential patients, specialists who treat CTEPH, including Dr. Rajeev Saggar, executive director of advanced lung disease care at Banner — University Medical Center Phoenix and the clinical associate professor of medicine at University of Arizona College of Medicine — Phoenix, hope to increase knowledge of the disease among other clinicians.
“The goal of PH treatment is to lengthen survival time, reduce symptoms and improve quality of life for patients. Early, accurate diagnosis is increasingly important, particularly because medical care is steadily improving,” said Dr. Saggar. “It is vital that clinicians detect or rule out underlying diseases that may represent the cause of PH, including various heart and vascular diseases, human immunodeficiency virus (HIV), lupus, or as is sometimes the case with CTEPH, unresolved pulmonary thromboembolism, when a clot-like mass gets stuck to the lung’s blood vessel wall and blocks blood flow.”
According to Dr. Saggar, a ventilation/perfusion (VQ) scan is the preferred and recommended screening test for CTEPH. It is important for anyone diagnosed with the disease to see an experienced CTEPH practitioner who can determine candidacy for pulmonary thromboendarterectomy (PTE) surgery. Although PTE is the recommended treatment for CTEPH, not everyone is a candidate. It’s important for patients to speak with his/her doctor about options.
Despite efforts to ensure better education about the disease, its symptoms and methods of treatment, physicians like Dr. Saggar and those throughout the CTEPH community note that there is still progress to be made. That’s why November is an especially important time for the PH community to come together and raise awareness and generate additional support for research and improve care.
“Building awareness and enhancing education will help to extend and improve the lives of patients with CTEPH and all forms of PH,” says Dr. Saggar.
For more information about PH or to provide support visit http://www.phaware.global/. To learn more about CTEPH, visit www.CTEPH.com.
Dr. Saggar and phaware partnered with Bayer to help raise awareness about PH and CTEPH during PH Awareness Month. Dr. Saggar is a paid consultant to Bayer.
*Based on a study with 223 patients in which approximately 4% were diagnosed with CTEPH within 2 years of their first episode of pulmonary embolism (blood clot in the lung). CTEPH did not develop after two years in any of the remaining patients.
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 Pepke-Zaba et al. Chronic thromboembolic pulmonary hypertension: role of medical therapy. Eur Respir J. 2013;41:985–990.
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