Expression of Wilms Tumor 1 Gene Distinguishes Vascular Malformations From Proliferative Endothelial Lesions

Abstract

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.

Snrk-1 is involved in multiple steps of angioblast development and acts via notch signaling pathway in artery-vein specification in vertebrates

“…22 In addition, the exclusivity of the snrk-1 mutations in venous or lymphatic malformation patient samples argues that snrk-1 may be an important molecular marker like wt-1 that helps distinguish vascular malformations from proliferative endothelial lesions. 23 In summary, this study reports a novel cytoplasmic signaling member of the Ser/Thr kinase family that plays a critical role in angioblast development in vertebrates in vivo.…”

NHERF-2 silences the silencers

“…10 Interestingly, WT-1, a major activator of podocalyxin, is highly expressed in hemangioma cells, as well as highly invasive tumors (ie, glioblastoma). 11 Thus, NHERF-2 represents a novel target for pharmacologic therapy, and drugs …”

“…Cebpa-Cre;Dicer1 fl/fl mice died shortly after birth, likely due to defective Cebpa-dependent induction of the lung epithelium in late gestation, 10 similar to mice with conditional deletion of Dicer1 driven by a Sonic Hedgehog transgene. 11 To circumvent the perinatal lethality observed in this model, Alemdehy et al transplanted fetal liver cells from mutant and control embryos into lethally irradiated recipient mice. No quantitative differences in Lin-Sca-1 neg Kit pos , common myeloid progenitors (CMPs), GMPs, or megakaryocyte erythroid progenitors (MEPs) were observed in Dicer1-deficient mice.…”

Clinical and Molecular Biology of Angiosarcoma

“…However, only Kaposi sarcoma are positive for the presence of Kaposi's sarcoma causative virus (Human Herpes Virus 8) by immunohistochemistry using antibodies against Latency nuclear antigen-1 (LANA-1) or by amplifying viral DNA by PCR (reviewed in (Mesri et al 2010;Schmid & Zietz 2005)). Cutaneous AS may also be confused with irregular growths of superficial vasculature or benign hemangiomas, mostly seen in infants (Lawley et al 2005). These vascular malformations are commonly referred to as port wine stains or angel kisses.…”

“…These vascular malformations are commonly referred to as port wine stains or angel kisses. These may be distinguished from visceral AS by immunohistochemistry with antibodies against the Wilms tumor 1 transcription factor (Ge et al 2011;Lawley et al 2005). The main discernible clinical sign of splenic AS is splenomegaly (Neuhauser et al 2000;Thompson et al 2005).…”