Background: Klippel-Trénaunay-Syndrome (KTS) is characterized by a triad of varicose veins, port-wine stain and soft tissue or bony hypertrophy, and the diagnosis of KTS can be made if any two of these three features are present. Hemangiomas in various locations, e.g., skull, brain, epidural and vertebral hemangioma, mediastinal, colonic hemangioma, intraneural/intramuscular hemangiomas, are reported with KTS.
Case Presentation: Benign vascular tumors may rarely develop malignant transformation as Bugarin- Estrada et al. reported breast angiosarcoma in a patient diagnosed as Klippel-Trenaunay-Syndrome. We reported a case of a 40-year-old female with a known case of Klipple-Trenaunay-Syndrome with left leg varicosities, cutaneous nevus, as well as unfortunate development of deep venous thrombosis and markedly enlarged right breast hemangioma. Due to low incidence or lack of early detection of breast hemangioma, its diagnosis is challenging.
Conclusion: The history of the patient and multi-modality imaging utilization can help in early and accurate diagnosis of diseases leading to better prognosis.
Keywords: Klippel-trenaunay-syndrome, deep venous thrombosis, breast hemangioma, mammogram, sonomammogram, magnetic resonance imaging.