Study Shows Tuberous Sclerosis Complex (TSC) Tumors are Highly Vascular; Biology of TSC and Cancer Similar
PHILADELPHIA (April 4, 2002) — Benign tumors in patients with tuberous sclerosis complex are highly vascular. That is the result of a study published in the March 2002 issue of the Journal of the American Academy of Dermatology. Elizabeth P. Henske, M.D., a member of the Medical Science Division of Fox Chase Cancer Center was an investigator in study.
Tuberous sclerosis complex (TSC) is a genetic disorder that leads to benign tumors in multiple organs, including the brain, kidneys, heart, eyes, lungs and other organs. Progressive growth of the tumors, and malignant transformation of brain and kidney lesions constitute the major cause of morbidity and mortality in adults with tuberous sclerosis. In addition, growth of skin lesions may be disfiguring to patients.
The purpose of this study was to determine whether benign tumors in patients with tuberous sclerosis are angiogenic. Brain, kidney, and skin tumors from patients with tuberous sclerosis were stained with CD31, a specific marker of vascular endothelium. In addition, the researchers demonstrated that renal cells involved in TSC express the potent angiogenesis stimulator -vascular endothelial growth factor (VEGF).
"This study confirms that benign tumors in tuberous sclerosis involve excessive blood vessel growth. It is possible that existing therapies that stop angiogenic activity may be helpful in treating, or even preventing, these tumors. In addition, laboratory studies that determine how the tuberous sclerosis genes result in blood vessel growth could lead to a better understanding of blood vessel growth in cancer," explains Dr. Henske.
Cancers are also highly vascular and many express VEGF. This researcher points to a substantial overlap between the biology of cancer and the biology of benign tumors of TSC. Pathways involving cell proliferation, differentiation and signaling that are disrupted in TSC are also frequently involved in human malignant tumors (cancers). This research suggests that certain biologic therapies being developed for cancer patients may be effective for TSC patients.
TSC is characterized by seizures, most often beginning in the first year of life, and the condition can result in mental retardation or neurobehavioral problems. It is estimated that nearly 50,000 people in the United States are diagnosed with TSC and more than 1 million worldwide.
Fox Chase Cancer Center, part of Temple University Health System, is one of the leading cancer research and treatment centers in the United States. Founded in 1904 in Philadelphia as one of the nation’s first cancer hospitals, Fox Chase also was among the first institutions to receive the National Cancer Institute’s prestigious comprehensive cancer center designation in 1974. Fox Chase researchers have won the highest awards in their fields, including two Nobel Prizes. Fox Chase physicians are routinely recognized in national rankings, and the Center’s nursing program has achieved Magnet status for excellence three consecutive times. Fox Chase conducts a broad array of nationally competitive basic, translational, and clinical research and oversees programs in cancer prevention, detection, survivorship, and community outreach. For more information, call 1-888-FOX-CHASE (1-888-369-2427).