静脉内平滑肌瘤病的诊疗现状

Diagnosis and treatment of intravenous leiomyomatosis

静脉内平滑肌瘤病是一种罕见的、组织学上良性但生物学上具有侵袭性的肿瘤。该病起源于子宫平滑肌或子宫静脉壁,可突破子宫在盆腔内生长成团,也可沿子宫静脉延伸入静脉回流系统在静脉通道内爬行生长延长而不侵犯静脉血管壁本身。该病发生发展依赖雌激素,能随血流播散,除了延续性生长,部分呈现出静脉系统、心腔内跳跃性生长,或在双肺内播散性生长,却不发生远处转移。目前针对该病的研究较少,主要集中在个案报道、手术经验总结、与普通妇科肌瘤发病机制鉴别、影像学诊断经验等方面。发病起源主要有子宫平滑肌和子宫静脉平滑肌两种学说,一些基础研究验证了雌激素、孕激素受体相关通路和血管内皮生成在疾病发展过程中的作用。该病临床症状多样,因累及的范围不同而异,早期肿瘤累及盆腔仅出现一些妇科症状、盆腔器官受压迫症状,进展至下腔静脉、心脏后则出现静脉回流不畅、心脏梗阻和血流动力学相关症状。不同中心提出了不同的分期分型方法,有的根据病变累及范围、肿瘤各节段直径大小分型,以利于评估病情、学科间交流沟通和确定治疗方案;有的根据肿瘤大小、切除时需采取的手术策略分类,以利于在术前制定详细的手术计划。目前,手术治疗是本病的主要治疗手段,但具体手术方式、药物辅助治疗和预后仍需进一步探讨。

Intravenous leiomyomatosis is a rare type of tumor that is histologically benign but biologically invasive.It originates from the smooth muscle of the uterine or the uterine vein.It can grow through the uterus and extend into the pelvic cavity,or grow along the veins without invading the wall of the venous vessel itself.The tumors are estrogen-dependent and can metastasize through the bloodstream.Thus,in addition to continuous growth,some tumors exhibit isolated growths in the venous system and heart chambers or show disseminated growth in the lungs,although distant metastasis to other regions usually do not occur.Currently,there is limited research on this disease,the majority of which are case reports,surgical experience summaries,and differentiation from ordinary gynecological myomas in terms of pathogenesis and radiological diagnostic experience.There are two main theories on the origin of the disease:uterine smooth muscle and smooth muscle of the uterine veins.Some studies have verified the role of estrogen,progesterone receptor-related pathways,and angiogenesis in the development of the disease.The clinical symptoms of this disease are varied,depending on the affected area.In the early stages,when the tumor only affects the pelvic cavity,patients show mild symptoms resulting from pelvic organ compression.When it progresses to the inferior vena cava and heart,patients show more complex symptoms resulting from venous return obstruction,cardiac obstruction,and hemodynamics appearing.Different institutions have proposed different disease staging and classification strategies for different clinical purposes.Some are based on the affected area of the lesion;others are based on the size of the tumor.Although surgery remains the main treatment for this disease,the specific surgical approach,adjuvant drug therapy,and prognosis still need further exploration.