This rare soft-tissue cancer requires early diagnosis and appropriate referral for treatment.
He waited patiently, as the long stream of mourners walked in solemnly, single file, to pay their respects. His loving wife had passed just one week prior. Her children played with their friends and cousins off to the side, unaware of the grief that weighed heavily on their father’s sagging shoulders.
As her doctor took a place in the line and gazed upon the father’s face, the question arose: Could the clinical course have been altered? Although not properly diagnosed before her initial surgical procedure, upon discovery of her sarcoma cancer she was appropriately referred to a specialty center. Unfortunately, her tumor reappeared on scans with a vengeance.
We lost her too soon. Her husband was now a widower and single father of two children. Two young children gazed on pictures of their mother that immortalized her smile, but lacked the infectious laughter that would have normally accompanied it.
In a country of 350 million people, 15,000 seems a relatively minuscule number. That is, until you or a loved one falls into that small group. Then that number becomes personal. The American Cancer Society estimates that 15,040 new cancers arising from the bones, joints or soft tissues will be reported in 2014. Of these, 6,200 patients likely will die from their disease this year. Collectively, these cancers — called sarcomas — comprise more than 70 subtypes. Each entity is rare, and it is estimated that some primary care physicians may see only a few in their lifetimes.
Cure can be achieved surgically when patients present with localized disease. However, in adults, when the disease has spread, treatment is aimed at prolonging life, with most recent clinical trials reporting 50 percent survival rates at 12 to 15 months. Therefore, there is a pressing need to diagnose patients early, before the disease has had a chance to spread.
The varied nature of the subtypes of sarcoma cannot be overstated. While proper surgical resection remains the mainstay of treatment for early disease, some subtypes require additional treatments such as chemotherapy and/or radiation. Due to the number of variables, it is important that the disease be diagnosed and treated at an experienced sarcoma center. The majority of soft-tissue sarcomas require evaluation of the primary site with magnetic resonance imaging (MRI) and the site of most common distant involvement, the lungs, with CT scan (or X-ray). However, some rare types may require more detailed imaging or studies.
Once the disease has been shown to be confined, proper resection in a surgical center with experience in managing sarcomas gives the best chance for survival. This often involves removing the lump in question with a wide margin to capture not just the visible tumor but also cells invisible to the naked eye. If a lump is treated as benign and later found to be sarcoma, the patient often requires additional surgery. Inadequate surgery for sarcomas may leave behind residual disease, which is known to put patients at increased risk for relapse and to threaten their long-term survival.
The key to proper management of sarcomas lies in early recognition and appropriate referral. The majority of lumps seen on the limbs and trunk are benign and do not need specialty care. However, failure to recognize the signs that suggest the presence of an underlying malignancy can have perilous consequences.
Patients, relatives and primary care providers are often the first to notice an abnormal lump. Physicians and families should be alert to any lumps that are deep, new or getting larger, especially those that are 2 inches (5 centimeters) or larger — anywhere in the body. These lumps can be painless or painful. This is important, because some painless lumps are written off as benign, and the time taken to observe a suspicious large lump may delay diagnosis.
These suspicious lumps should be imaged prior to biopsy, preferably with MRI, and referred for management in a high-volume sarcoma center. We recommend that attempting a surgical biopsy or removing a tumor that is deep-seated or greater than 2 inches be reserved for those who have training in sarcoma treatment.
As members of the Medical Advisory Board of the Rein in Sarcoma Foundation, we strongly advocate for adopting these principles, and we urge patients and caregivers to stop and consider a diagnosis of sarcoma. Perhaps if treated properly from the beginning, our young mother could have been saved.
Further information is available at the website www.reininsarcoma.org.
This article was signed by Dr. Nancy Mcallister of Children’s Hospitals and Clinics of Minnesota; Dr. Randy W. Hurley, medical director of the cancer program at HealthPartners; Dr. Scott Okuno of the Mayo Clinic; Steven Robinson, M.B.B.S., of the Mayo Clinic, and Drs. L. Chinsoo Cho, Jutta Ellermann, Emily Lipsitz Greengard and Christian M. Ogilvie of the University of Minnesota Medical Center.