患者女性,44岁,已婚已育,因“子宫肌瘤2次剔除术后8年,经量增多1年”于2022年10月29日入院,查体示:外阴发育正常,阴道通畅,宫颈暴露困难,光滑,宫体前位,如孕5个月大小,形态不规则,质中,活动可,无压痛。双侧附件区未扪及明显异常。辅助检...患者女性,44岁,已婚已育,因“子宫肌瘤2次剔除术后8年,经量增多1年”于2022年10月29日入院,查体示:外阴发育正常,阴道通畅,宫颈暴露困难,光滑,宫体前位,如孕5个月大小,形态不规则,质中,活动可,无压痛。双侧附件区未扪及明显异常。辅助检查:① 妇科超声:子宫前位,约15.8 cm × 11.0 cm × 10.2 cm,包膜欠光滑,外形欠规则,肌层回声不均匀,肌层内见多发低回声结节及团块,较大者右后壁下段8.8 cm × 5.8 cm (外凸),均边界清;② 血红蛋白:94 g/L。术后病理提示:家族遗传性平滑肌瘤。展开更多
静脉内平滑肌瘤病(Intravascular Leiomyomatosis, IVL)是临床中极为罕见的疾病,仅限于女性,好发于围绝经期或育龄期妇女,IVL源自子宫肌细胞或静脉血管平滑肌细胞,病理类型为良性肿瘤,但有不良的生物学行为,指向血管腔生长,会沿人体静...静脉内平滑肌瘤病(Intravascular Leiomyomatosis, IVL)是临床中极为罕见的疾病,仅限于女性,好发于围绝经期或育龄期妇女,IVL源自子宫肌细胞或静脉血管平滑肌细胞,病理类型为良性肿瘤,但有不良的生物学行为,指向血管腔生长,会沿人体静脉系统蔓延,累及腹盆腔静脉–下腔静脉–右心房–肺动脉。所以尽早诊断及准确评估是提高本病生存率的最好方法。影像学检查是早期发现病变及术后评估的最有效的无创方法。近年来临床对IVL的认知度逐渐提高,本文就笔者在临床中遇到的一例经病理证实的静脉内平滑肌瘤病患者,其病变生长延伸至左肺动脉,笔者对病例进行回顾性分析并进行相关文献复习,旨在为临床工作者提供相关诊断经验,提高临床医生对于IVL的认知水平与了解。Intravascular Leiomyomatosis (IVL) is an extremely rare clinical disease, limited to females and predominantly occurring in women during the perimenopausal or reproductive years. IVL originates from uterine myocytes or venous vascular smooth muscle cells, and pathologically is classified as a benign tumor. However, it exhibits unfavorable biological behavior, growing into the vascular lumen and spreading along the human venous system, involving the pelvic and abdominal veins, the inferior vena cava, the right atrium, and the pulmonary artery. Therefore, early diagnosis and accurate assessment are the best methods to improve the survival rate of this disease. Imaging examinations are the most effective non-invasive methods for early detection of lesions and postoperative assessment. In recent years, clinical awareness of IVL has gradually increased. This article reviews a case of IVL, pathologically confirmed in the clinic, with the lesion extending to the left pulmonary artery. The author conducts a retrospective analysis of the case and reviews relevant literature, aiming to provide diagnostic experience for clinical workers and to improve clinical doctors’ awareness and understanding of IVL.展开更多
文摘患者女性,44岁,已婚已育,因“子宫肌瘤2次剔除术后8年,经量增多1年”于2022年10月29日入院,查体示:外阴发育正常,阴道通畅,宫颈暴露困难,光滑,宫体前位,如孕5个月大小,形态不规则,质中,活动可,无压痛。双侧附件区未扪及明显异常。辅助检查:① 妇科超声:子宫前位,约15.8 cm × 11.0 cm × 10.2 cm,包膜欠光滑,外形欠规则,肌层回声不均匀,肌层内见多发低回声结节及团块,较大者右后壁下段8.8 cm × 5.8 cm (外凸),均边界清;② 血红蛋白:94 g/L。术后病理提示:家族遗传性平滑肌瘤。
文摘静脉内平滑肌瘤病(Intravascular Leiomyomatosis, IVL)是临床中极为罕见的疾病,仅限于女性,好发于围绝经期或育龄期妇女,IVL源自子宫肌细胞或静脉血管平滑肌细胞,病理类型为良性肿瘤,但有不良的生物学行为,指向血管腔生长,会沿人体静脉系统蔓延,累及腹盆腔静脉–下腔静脉–右心房–肺动脉。所以尽早诊断及准确评估是提高本病生存率的最好方法。影像学检查是早期发现病变及术后评估的最有效的无创方法。近年来临床对IVL的认知度逐渐提高,本文就笔者在临床中遇到的一例经病理证实的静脉内平滑肌瘤病患者,其病变生长延伸至左肺动脉,笔者对病例进行回顾性分析并进行相关文献复习,旨在为临床工作者提供相关诊断经验,提高临床医生对于IVL的认知水平与了解。Intravascular Leiomyomatosis (IVL) is an extremely rare clinical disease, limited to females and predominantly occurring in women during the perimenopausal or reproductive years. IVL originates from uterine myocytes or venous vascular smooth muscle cells, and pathologically is classified as a benign tumor. However, it exhibits unfavorable biological behavior, growing into the vascular lumen and spreading along the human venous system, involving the pelvic and abdominal veins, the inferior vena cava, the right atrium, and the pulmonary artery. Therefore, early diagnosis and accurate assessment are the best methods to improve the survival rate of this disease. Imaging examinations are the most effective non-invasive methods for early detection of lesions and postoperative assessment. In recent years, clinical awareness of IVL has gradually increased. This article reviews a case of IVL, pathologically confirmed in the clinic, with the lesion extending to the left pulmonary artery. The author conducts a retrospective analysis of the case and reviews relevant literature, aiming to provide diagnostic experience for clinical workers and to improve clinical doctors’ awareness and understanding of IVL.
