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July is Sarcoma Awareness month, symbolized by a yellow ribbon. Sarcomas are a rare group of malignant cancers of soft tissue or bone origin. Together soft tissue and bone comprise our connective tissues. Connective tissues include muscles, nerves, fat, fibrous tissues, bone, cartilage and blood vessels. This means that the disease can be found anywhere within the body. Less than 1% of all adult cancers are sarcomas while 10 to 15% of all pediatric cancers are comprised of sarcomas (Ryan & Meyer, 2020). Let’s explore sarcoma in more depth and bring awareness to this challenging disease.
The majority of new cases of sarcomas originate from soft tissue with lesser cases of bone. There are many subtypes of sarcomas categorized by those more common in adults versus children. Currently there are over a 100 different subtypes. A sarcoma diagnosis can be devastating to families. The disease can be highly aggressive and resistant to treatment.
Sarcoma Clinical Presentation:
The typical presentation for sarcoma is a gradually enlarging painless mass. The mass is usually deep and along tissue planes. Patients may complain of pain related to compression of nerves caused by swelling depending upon the location. The most common locations are the extremities, torso, head and neck, and abdomen. Sarcomas are more likely to spread to the lungs. They are less likely to spread to regional lymph nodes. Nodal involvement carries a poorer prognosis. Some subtypes have a chance of spreading to the central nervous system as well (Ryan & Meyer, 2020).
Diagnostics:
Various imaging studies are usually completed. These can include x-ray plain films depending upon location, CT, MRI, and PET/CT. CT of the chest is almost always recommended since the disease likes to spread to the lungs. The subtypes with likelihood of spread to the CNS will require a MRI brain. Inappropriate biopsy technique can lead to distant spread and further complications. It’s recommended to refer suspected patients to an appropriate specialized center for biopsy.
Sarcoma Prognosis:
Prognosis is dependent upon histologic grade, tumor size, and pathologic stage at the time of diagnosis. Some variants can be safely excised without further intervention, while others require extensive therapy.
Treatment:
Treatment usually consists of some combination of surgery, chemotherapy, and or/radiation. Depending upon the subtype, sarcomas can be highly resistant to radiation.
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Reference
Ryan, C.W., & Meyer, J.(2020). Clinical presentation, histopathology, diagnostic evaluation, and staging of soft tissue sarcoma. In S. Shah(Ed.) UpToDate. Retrieved July 15, 2020.