Imaging Pearls ❯ Musculoskeletal ❯ Soft Tissue Masses
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- “ Blue rubber bleb nevus, Proteus, Klippel-Trenauney, and Kasabach-Merritt syndromes are a diverse group of vascular malformation and hemangiomatosis syndromes. Both cutaneous and visceral vascular lesions are associated with these disorder. Accurate diagnosis of these syndromes is important because they can be associated with serious complications, including life threatening hemorrhage.”
Vascular Malformation and Hemangiomatosis Syndromes: Spectrum of Imaging Manifestation
Elsayes KM et al.
AJR 2008:190;1291-1299 - “ Blue rubber bleb nevus, Proteus, Klippel-Trenauney, and Kasabach-Merritt syndromes are a diverse group of vascular malformation and hemangiomatosis syndromes. Both cutaneous and visceral vascular lesions are associated with these disorder.”
Vascular Malformation and Hemangiomatosis Syndromes: Spectrum of Imaging Manifestation
Elsayes KM et al.
AJR 2008:190;1291-1299 - Vascular Malformation and Hemangiomatosis Syndromes
- Blue rubber bleb nevus syndrome
- Proteus syndrome
- Klippel Trenaunay syndrome
- Kasabach-Merritt syndrome - Blue rubber bleb nevus syndrome (BRBNS): Facts
- Usually presents at birth
- Multiple distinctive cutaneous and gastrointestinal venous malformations (usually small bowel)
- Can present with hematemesis, melena or hematochezia
- May present with life threatening bleeding
- Increased incidence of select tumors like medulloblastoma, CLL, renal cell carcinoma, and squammous cell carcinoma - Proteus Syndrome: Facts
- Varierty of malformations and overgrowths involving multiple tissue types
- May present as hemihypertrophy and partial gigantism with resultant deformity of the skull, hands and feet
- Imaging can show vascular malformations, regional fatty atrophy as well as splenomegaly and nephromegaly
- Exostosis, scoliosis and limb length discrepancy maay also be seen - Klippel Trenaunay syndrome: facts
Congenital disorder characterized by
- Port wine stain
- Abnormal venous structures (varicosities and venous malformations)
- Osseous and soft tissue hypertrophy
- GI tract involvement may occur in up to 20% of patients and presentation may be GI bleed. Splenic vascular lesions are common - Kasabach-Merritt syndrome:Facts
- Consumptive thrombocytopenia and coagulopathy
- May result in disseminated intravascular coagulation
- Often found in the setting of a hepatic or splenic hemangioma. Not a classic hemangioma and often referred to as tufted angioma or kaposiform hemangioendotheliomas
- May be associated with angiosarcoma or AVMs