Radiology Department, Pakistan Institute of Medical Sciences, NUST, Islamabad, Pakistan.
Curr Med Imaging. 2022;18(7):764-767. doi: 10.2174/1871529X21666210924150832.
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.
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.
The history of the patient and multi-modality imaging utilization can help in early and accurate diagnosis of diseases leading to better prognosis.
Klippel-Trénaunay 综合征(KTS)的特征是静脉曲张、葡萄酒色斑和软组织或骨肥大三联征,如果存在这三个特征中的两个,则可诊断为 KTS。KTS 患者可发生各种部位的血管瘤,例如颅骨、脑、硬膜外和脊柱血管瘤、纵隔、结肠血管瘤、神经内/肌肉内血管瘤。
良性血管肿瘤可能很少发生恶性转化,正如 Bugarin-Estrada 等人报道的 Klippel-Trenaunay 综合征患者的乳腺血管肉瘤。我们报告了一例 40 岁女性,已知患有 Klippel-Trenaunay 综合征,左腿静脉曲张、皮肤痣,不幸发生深静脉血栓形成和右侧乳腺血管瘤显著增大。由于乳腺血管瘤的发病率低或早期检测缺乏,其诊断具有挑战性。
患者的病史和多模态影像学检查有助于早期和准确诊断导致更好预后的疾病。