Tuberous sclerosis-associated lesions of the kidney, brain, and skin are angiogenic neoplasms.
BACKGROUND
Tuberous sclerosis is an autosomal dominant condition characterized by the development of benign neoplasms of the brain, kidney, and skin. Progressive growth 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.


OBJECTIVE
The purpose of this study was to determine whether benign tumors in patients with tuberous sclerosis are angiogenic.


METHODS
Brain, kidney, and skin tumors from patients with tuberous sclerosis were stained with CD31, a specific marker of vascular endothelium. In addition, we used Northern blot analysis to demonstrate that renal angiomyolipoma cells express the potent angiogenesis stimulator vascular endothelial growth factor (VEGF).


RESULTS
Brain, kidney, and skin neoplasms from patients with tuberous sclerosis are highly angiogenic. Renal angiomyolipoma cells produce the potent angiogenic factor VEGF.


CONCLUSIONS
Benign neoplasms of patients with tuberous sclerosis are highly vascular. Our results provide a rationale for antiangiogenic therapy in the treatment and prevention of tuberous sclerosis-associated neoplasms.
Arbiser
Jack L
JL
Department of Dermatology, Emory University School of Medicine, WMB 5309, 1639 Pierce Street, Atlanta, GA 30322, USA. jarbise@emory.edu
Brat
Daniel
D
Hunter
Steve
S
D'Armiento
Jeanine
J
Henske
Elizabeth P
EP
Arbiser
Zoya K
ZK
Bai
Xianhe
X
Goldberg
Gerald
G
Cohen
Cynthia
C
Weiss
Sharon W
SW
D000328:Adult; D018322:Angiofibroma / Q000473:pathology*; D018207:Angiomyolipoma / Q000473:pathology*; D001254:Astrocytoma / Q000473:pathology*; D015152:Blotting, Northern; D001932:Brain Neoplasms / Q000473:pathology*; D016228:Endothelial Growth Factors / Q000378:metabolism; D006801:Humans; D007680:Kidney Neoplasms / Q000473:pathology*; D008222:Lymphokines / Q000378:metabolism; D009389:Neovascularization, Pathologic / Q000473:pathology; D012878:Skin Neoplasms / Q000473:pathology*; D014402:Tuberous Sclerosis / Q000473:pathology*; D042461:Vascular Endothelial Growth Factor A; D042442:Vascular Endothelial Growth Factors
D016228:Endothelial Growth Factors; D008222:Lymphokines; D042461:Vascular Endothelial Growth Factor A; D042442:Vascular Endothelial Growth Factors