Renal Angiomyolipomas, Cysts, and Cancer in Tuberous Sclerosis Complex

Overview

Journal Semin Pediatr Neurol

Specialties Neurology
Pediatrics

Date 1999 Jan 6

PMID 9874854

Citations 21

Authors

H P Neumann

G Schwarzkopf

E P Henske

Affiliations

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Abstract

Tuberous sclerosis is a multisystem syndrome characterized by neurological symptoms and tumors in multiple organs, including kidney, brain, skin, eyes, heart, and lung. The kidney and brain are the two most frequently affected organs in TSC, and renal disease is a leading cause of death in TSC patients. Three types of tumors occur in TSC kidneys: (1) angiomyolipomas, which are benign tumors composed of smooth muscle, fat, and vessels; (2) epithelial cysts; and (3) malignant tumors. This review focuses on the clinical, pathological, and molecular features of these tumors.

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