The terminology doctors use to describe different kinds of cancer can seem bewildering, but all cancers can be classified into a handful of major categories. Carcinomas form in the epithelial cells in your skin, mucus membranes, glands and organs. Leukemias and lymphomas are cancers that develop from white blood cells. Melanomas arise from cells that produce the pigment melanin. Sarcomas originate in muscle, fat, bone, cartilage and connective tissue. Low-grade fibromyxoid sarcoma is a rare, slow-growing cancer that tends to arise on the trunk and extremities, but may occur internally. The prognosis for a patient with LGFS is largely dependent on tumor size at diagnosis and whether the cancer has spread to distant organs.
Few Alarming Signs or Symptoms
Although a few cases of LGFS have been described in children, these tumors usually occur in young adults. Patients typically report having a painless, slow-growing mass on their thigh, shoulder or groin. Because these cancers grow slowly and do not cause discomfort, many people don’t seek medical attention until they become alarmed by the size of the mass. According to a case report and literature review in the July 2012 issue of "Case Reports in Nephrology and Urology," some patients with LGFS have delayed seeing their physicians for as long as 25 years. While this attests to the slow-moving nature of LGFS, such a delay can negatively affect your prognosis.
Treatment and Outlook
Prior to surgical removal, which is the initial form of treatment for most patients with LGFS, your doctor may order blood work, imaging studies or other tests, depending on where your tumor is located and how large it is. To offer the best chance for cure, your surgeon will probably remove tissue surrounding the cancerous tumor. This may necessitate grafting or reconstructive surgery to close the defect left by your initial procedure. According to the authors of the 2012 "Case Reports in Nephrology and Urology" report, the 5-year overall survival rate following surgery for LGFS is over 90 percent, with a more favorable prognosis associated with smaller tumors. This cancer has a tendency to recur, however, and it has been known to spread to distant organs many years after surgery.
According to a 2009 report in "Clinics in Orthopedic Surgery," complete surgical removal of an LGFS does not necessarily represent a cure. Recurrences at the site of the original tumor are common, with more than 50 percent of patients experiencing local recurrence. Spread to distant sites -- usually the lungs, the membranes surrounding the lungs or the chest wall -- occurs in approximately 6 percent of patients. Local recurrences of LGFS have been reported from a few months to 15 years after initial treatment, while distant spread has occurred from 0 to 45 years after surgery. Radiation therapy or chemotherapy may be necessary for patients whose tumors recur locally or spread to distant sites.
Long-Term Follow-Up Recommended
Low-grade fibromyxoid sarcoma is a deceptively slow-growing tumor. While long-term survival is good for most patients, LGFS can recur or metastasize up to 5 decades after its initial treatment. In the October 2011 issue of "The American Journal of Surgical Pathology," Dr. H. L. Evans of the M.D. Anderson Cancer Institute reviewed the histories of 33 patients who had been diagnosed and treated for LGFS. At that writing, 14 patients had died from their tumor between 3 and 42 years after diagnosis, with an average survival of 15 years. Nineteen patients -- 6 with cancer and 13 without -- were still alive between 5 and 70 years following diagnosis. Due to the tendency for LGFS to lie dormant for extended periods, patients with this cancer may need to be followed for several decades after their first treatment.
- Indian Dermatology Online Journal: Fibromyxoid Sarcoma of the Leg
- Case Reports in Nephrology and Urology: Low-Grade Fibromyxoid Sarcoma of the Renal Pelvis: First Report
- Clinics in Orthopedic Surgery: Low-Grade Fibromyxoid Sarcoma in Thigh
- The American Journal of Surgical Pathology: Low-Grade Fibromyxoid Sarcoma: A Clinicopathologic Study of 33 Cases with Long-Term Follow-Up (abstract)
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