Experts recognize some 20 varieties of sarcoma, all of which originate in the connective tissue or muscle. Such tumors thus differ from the common solid tumors of the lung, colon, breast, or pancreas, that form in the epithelial lining of organs, or the immunological malignancies such as leukemia or lymphoma that affect the white blood cells. Sarcomas are rare, accounting for only 8800 cases in the United States in 2004. (1) Sixty percent appear first in the extremities and when localized, surgery can be curative. Once metastatic this cancer type – notoriously resistant to chemotherapy and radiation – usually proves fatal within a year.
Patient IG is a 58-year old woman who in the summer of 1993 first noticed a mass above her right ear. After the lesion became chronically irritated by her eyeglass frames, in August 1993 she opted to have it removed. The nodule, measuring about 1 cm in diameter, was found consistent with “malignant neoplasm, probably metastatic.” The slides were sent for review at the Mayo Clinic, where the pathologist classified the cancer as an epithelioid sarcoma. A subsequent third review of the slides confirmed the diagnosis of epithelioid sarcoma.
The patient then underwent a metastatic work-up. A bone scan in September 1993 revealed:
Single abnormal focus of uptake in the left occipital-parietal region, worrisome for metastatic neoplasm.
A skull series the same day showed a “9 mm geographic lucency in the left occipital bone, possibly representing a calvarial metastasis.” The report of a CT scan of the head a week later stated:
Images of the skull demonstrated one small lytic area… in the left occipital bone… It measures under a centimeter in size. It is in the medullary space of the bone but appears to affect the cortex also. No soft tissue component is noted.
A CT scan of the neck and chest showed a probable right thyroid cyst, and two areas of decreased attenuation in the liver compatible with either cysts or metastatic disease.
IG then met with a head and neck surgeon, who proposed wide excision with removal of much of her jaw followed by reconstruction. But when she was told she most likely would die of her disease anyway, she refused surgery. After investigating alternative approaches to cancer, she learned of our therapy and consulted with Dr. Isaacs in late September 1993. She thereafter followed her program diligently.
In June 1994, nine months after she began her nutritional regimen, she noticed a lump above her right ear in the same location as the original tumor. The nodule stabilized for two years, before it was resected in August 1996. The pathology report describes once again an epithelioid sarcoma. After Dr. Isaacs made some adjustments in the protocol, IG continued her therapy faithfully as before.
Over the years, IG has been very compliant with her regimen, and enjoyed improvement in her overall energy and sense of well being. Since the surgery of 1996, the disease has not recurred. When last seen by Dr. Isaacs in August 2006 she was in good health with no visible evidence of cancer.
Epithelioid sarcomas tend to be fairly aggressive. If localized, as with most sarcomas, surgery can be curative, but once metastatic, survival is usually measured in months. A review of epitheloid sarcomas (2) reported that “Median post-distant metastasis survival was 8 months.”
We don’t think the lesion that appeared after she began her therapy indicates global treatment failure. As mentioned previously, we find at times that tumors will recur in areas of prior surgery, though nowhere else. We suspect that in areas of such tissue disruption, the resulting fibrosis and scarification compromise blood supply to the area, and create a protected area where residual cancer cells can grow unhindered. We suspect such a scenario in this patient’s case. Regardless, today, 13 years from her original diagnosis of metastatic cancer she is in excellent health with no clinical evidence of her disease.
1. Kasper DL, Braunwald E, Fauci AS, Hauser SL, Longo, DL, Jameson JL. Harrison’s Principles of Internal Medicine, 16th Edition. New York: McGraw-Hill; 2005:559.
2. Spillane, AJ, Thomas JM, Fisher C. Epithelioid sarcoma: the clinicopathological complexities of this rare soft tissue sarcoma. Ann Surg Oncol. 2000;7:218-25. [Abstract]