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Expression of Wilms Tumor 1 Gene Distinguishes Vascular Malformations From Proliferative Endothelial Lesions
Leslie P. Lawley, MD;
Francesca Cerimele, MD, PhD;
Sharon W. Weiss, MD;
Paula North, MD;
Cynthia Cohen, MD;
Harry P. W. Kozakewich, MD;
John B. Mulliken, MD;
Jack L. Arbiser, MD, PhD
Arch Dermatol. 2005;141:1297-1300.
Background Vascular malformations and hemangiomas, which are endothelial lesions of childhood, may result in considerable morbidity because they can cause discomfort and functional impairment and have a negative affect on the patient’s appearance. Although vascular malformations may initially appear very similar to hemangiomas, they have distinct clinical courses. Infantile hemangiomas progress through 3 stages: proliferative, involuting, and involuted. The proliferative phase is characterized by clinical growth. Once hemangiomas reach their maximum size, they begin to regress or involute. Histologically, this stage is characterized by endothelial apoptosis. Finally, the involuted stage of the hemangioma occurs when the original lesion is replaced by a connective tissue remnant. In contrast to hemangiomas, vascular malformations do not involute but continue to enlarge as the patient grows.
Observations The biochemical differences between hemangiomas, which involute, and vascular malformations, which do not involute, are not well understood. We found that the transcription factor encoded by the Wilms tumor 1 (WT1) gene is expressed in the endothelium of hemangiomas but not in vascular malformations.
Conclusions Defects in WT1 signaling may underlie the inability of malformation endothelial cells to undergo physiologic apoptosis and remodeling. The availability of WT1 staining in hospital laboratories may allow the clinician to distinguish hemangiomas from vascular malformations and thus to give appropriate therapy to the patient.
Author Affiliations: Departments of Dermatology (Drs Lawley, Cerimele, and Arbiser) and Pathology and Laboratory Medicine (Drs Weiss and Cohen), Emory University School of Medicine, and Department of Dermatology, Veterans Affairs Hospital (Drs Lawley, Cerimele, and Arbiser), Atlanta, Ga; Department of Pathology, University of Arkansas School of Medicine, Little Rock (Dr North); and Department of Pathology (Dr Kozakewich) and Division of Plastic Surgery (Dr Mulliken), Children’s Hospital and Harvard Medical School, Boston, Mass.
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