| 摘要: |
| 目的 总结血管内平滑肌瘤病的影像学特点及临床治疗经验。方法 回顾性分析我院2014年1月至2019年12月确诊的5例血管内平滑肌瘤病患者的临床表现、影像学资料、手术治疗情况及术后病理和免疫组织化学染色等资料，总结血管内平滑肌瘤病的影像学特点及临床治疗经验。结果 5例患者均为女性，年龄为46～60岁（平均51.8岁），均有子宫肌瘤病史，其中2例有子宫及双侧附件全切除术史。2例患者平滑肌瘤侵及右心房，表现为心功能受损症状，余3例分别以右下肢肿胀不适、腹部肿块、下腹胀就诊。4例患者行超声及下腔静脉CT造影检查，2例行盆腔或腹部MRI平扫+增强检查，1例行血管造影检查。5例患者均行血管平滑肌瘤切除+血管成形术。2例病灶累及右心房的患者中，1例患者行一期手术治疗，1例行二期手术，术后均恢复良好。1例患者因术后4年复发再次手术。1例患者因术中出血较多，未切除右侧髂总静脉内肿瘤，行肿瘤大部切除+髂总静脉起始端结扎术。5例患者的免疫组织化学染色均表现为平滑肌肌动蛋白（SMA）（+）、钙调理蛋白（CALP）（+）、抑癌基因P53（－）、CD31（－）、细胞增殖相关抗原Ki-67（－）；仅1例患者雌激素受体（ER）、孕激素受体（PR）均为阳性，1例ER、PR均为部分阳性，1例为ER部分阳性、PR少量阳性，2例患者未见ER、PR染色相关资料。5例患者术后均定期复查，随访至2021年2月均未见复发。结论 血管内平滑肌瘤病无特异性临床表现，但影像学检查特征明确，结合临床表现及妇科病史可提高诊断准确性。 |
| 关键词: 平滑肌瘤 子宫肿瘤 血管肌瘤 血管内肿瘤 |
| DOI：10.16781/j.0258-879x.2021.07.0811 |
| 投稿时间：2021-02-19修订日期：2021-03-31 |
| 基金项目: |
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| Imaging features of intravascular leiomyomatosis: a report of 5 cases |
| ZHONG Jing-jiao1,SONG Tao1,YI Yue-ling2* |
(1. Department of Radiology, Changhai Hospital, Naval Medical University (Second Military Medical University), Shanghai 200433, China;
2. Department of Emergency, Changhai Hospital, Naval Medical University (Second Military Medical University), Shanghai 200433, China
*Corresponding author) |
| Abstract: |
| Objective To summarize the imaging features and clinical treatment experience of intravascular leiomyomatosis. Methods The clinical manifestations, imaging data, surgical treatment, postoperative pathology and immunohistochemical staining of 5 patients with intravascular leiomyomatosis diagnosed in our hospital from Jan. 2014 to Dec. 2019 were retrospectively analyzed, and the imaging features and clinical treatment experience were summarized. Results The 5 patients were all females, with an average age of 51.8 years (ranged 46-60 years), and all had a history of hysteromyoma, and 2 of them had a history of hysterectomy and bilateral adnexectomy. Two cases presented with leiomyoma invading the right atrium, showing symptoms of impaired cardiac function; the remaining 3 cases presented with right lower limb swelling, abdominal masses, and lower abdominal distension. Four cases underwent ultrasound and computed tomography (CT) angiography of the inferior vena cava, 2 cases underwent magnetic resonance imaging (MRI) plain scan + enhanced examination, and 1 case underwent angiography. All patients underwent angiomyoma resection+angioplasty. Of the 2 cases presented with leiomyoma invading the right atrium, 1 case underwent primary surgery, 1 case underwent secondary surgery, and both patients recovered well after surgery. One case was re-operated due to recurrence 4 years after surgery. One case underwent a major resection of the tumor + ligation of the common iliac vein due to excessive bleeding during the operation, and the tumor in right common iliac vein was not resected. In the 5 cases, the common features of immunohistochemical staining were smooth muscle actin (SMA) (+), calponin (CALP) (+), P53 (-), CD31 (-), Ki-67 (-); only 1 case was positive for estrogen receptor (ER) and progesterone receptor (PR), 1 case was partially positive for both ER and PR, 1 case was partially positive for ER and slightly positive for PR, and 2 cases had no ER or PR staining data. All the 5 patients were followed up regularly and no recurrence was found until Feb. 2021. Conclusion The clinical manifestations of intravascular leiomyomatosis are not specific, but the imaging features are clear. The diagnosis accuracy can be improved by combining clinical manifestations and gynecological history. |
| Key words: leiomyoma uterine neoplasms angiomyoma intravascular neoplasms |
