- Vascular Malformations and Hemangiomatosis Syndromes
- Blue rubber bleb nevus syndrome
- Proteus syndrome
- Klippel-Trenauney Sydrome
- Kasabach-Merritt syndrome
- Blue rubber bleb nevus syndrome: facts
- Multiple subcutaneous as well as GI tract small tumors
- Vascular GI lesions will commonly bleed
- Can involve entire GI tract but most commonly small bowel
- Associated with increased incidence of medulloblastoma, CLL, RCC and squammous cell carcinoma
- Proteus Syndrome: facts
- Hemihypertrophy, partial gigantism
- Vascular tumors and AVMs
- Lipomas and fatty tumor proliferation
- splenomegaly
- Klippel-Trenaunay Syndrome: Facts
- Three key findings
–Port wine stain
–Abnormal vascular structures
–Osseous and soft tissue hypertrophy
- Klippel-Trenaunay Syndrome: Facts
- Patients have GI tract hemangiomas including bowel involvement with calcifications
- Kasabach-Merritt Syndrome: facts
- Consumptive thrombocytopenia and coagulopathy
- Can lead to DIC
- May have large hepatic or splenic hemangioma
- Association with angiosarcoma and AVMs
- Pulmonary Arteriovenous Malformations: Facts
- Congenital in origin
- 60-90% of patients have hereditary hemorrhagic telangiectasia (aka Rendu- Osler-Weber syndrome)
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