case_images: PMC10660422_01
case_images: Postoperative pathological results. (A) Hematoxylin-Eosin staining showed nested vascular tissue in the dermis (indicated by white arrow), confirming the diagnosis of TA. (B-D) Deep puncture biopsy of the paravertebral mass confirmed the diagnosis of KHE (B: CD34 (+), C: D2-40 (+), D: Glut-1 (-)). KHE,Kaposi hemangioendothelioma; TA, tufted angioma.
case_images: wjps-2023-000695f01.jpg
case_images: PMC10660422_01_wjps-2023-000695f01.jpg
case_images: F1
case_images: According to the medical history reported by her parents, she received surgical resection, and postoperative pathological examination revealed that there were nested vascular tissues in the dermis, which confirmed the diagnosis of TA (figure 1A). For further diagnosis, deep puncture biopsy of the paravertebral mass was performed in our clinic, and pathological analysis confirmed the diagnosis of KHE (figure 1B-D).
case_images: Case presentation of Cobb syndrome with Kaposi hemangioendothelioma/tufted angioma (KHE/TA). (A) patient presented with erythema surrounding the surgical incision on the left waist. (B) Preoperative MRI showed multiple abnormal signals in the left iliac, the left pedicle, transverse process and vertebral body of L2-S3 spine, as well as hyperplasia masses involving the left lower abdomen, soft tissue surrounding the left psoas major muscle and the left lumbosacral vertebral body. (C) Repeat MRI was performed 6 months into sirolimus therapy and revealed dramatic decreased of the dermis and near resolution of muscular invasion. KHE,Kaposi hemangioendothelioma; MRI, Magnetic resonance imaging; TA, tufted angioma.
case_images: wjps-2023-000695f02.jpg
case_images: PMC10660422_01_wjps-2023-000695f02.jpg
case_images: F2
case_images: However, 4 months after surgery, the patient presented with multiple, scattered masses with blue-to-purple color surrounding the surgical incision, and the erythema became progressively larger and darker (figure 2A). Magnetic resonance imaging (MRI) showed multiple abnormal signals in the left iliac, left pedicle, transverse process and vertebral body of the L2-S3 spine, as well as hyperplasia masses involving the left lower abdomen, soft tissue surrounding the left psoas major muscle and left lumbosacral vertebral body (figure 2B). Repeat MRI after 6 months of treatment showed that dermis was decreased dramatically and muscular invasion was nearly resolved (figure 2C).
case_images: The remission of pseudoscoliosis symptoms in patients. (A-C)The patient was additionally found to develop pseudoscoliosis and progressive claudication. (D-F) The patient had great improvement of pseudoscoliosis and claudication after treatment with sirolimus therapy for 6 months.
case_images: wjps-2023-000695f03.jpg
case_images: PMC10660422_01_wjps-2023-000695f03.jpg
case_images: F3
case_images: She was additionally found to develop complications secondary to KHE, including pseudoscoliosis and progressive claudication (figure 3A-C). By this time, her pseudoscoliosis and claudication had also greatly improved (figure 3D-F).