伴有心脏受累的静脉内平滑肌瘤病一例

Intravenous leiomyomatosis with intracardiac extension:a case report

静脉内平滑肌瘤病是一种特殊类型的子宫肌瘤,其生长方式类似恶性肿瘤,达下腔静脉甚至累及右心腔者非常罕见,具有潜在的致命性。本文报道1例伴有心脏受累的静脉内平滑肌瘤病患者。患者41岁,女性,因"间断右上腹疼痛,伴双下肢轻度肿胀20天"入院。既往有子宫肌瘤及子宫次全切除病史。超声心动示:右心房内可见团块,与心腔无黏连,左室射血分数60.00。腹部CT示:下腔静脉全程、左侧髂总、髂内静脉内软组织肿物,病变上极达右心房,增强扫描示肿物有明显强化。妇科及盆腔血管彩超可见下腔静脉下段、左侧髂总静脉内占位,左侧附件区髂内、外静脉之间可见一分叶状团块,其内见营养血管,子宫次全切除术后。初步诊断:下腔静脉、右心房肿瘤。行胸腹联合心脏、下腔静脉全程肿瘤切除,左侧髂内静脉结扎术,术后未予激素治疗,病理示肿瘤细胞呈梭形,未见核分裂相,无凝固性坏死,免疫组化SMA(+++),desmin(+),PR(+),ER(+),S100(-),CD117散在个别细胞(+),CD34(-),Ki67<50。随访6个月未见肿瘤复发。该病临床表现无特异性,术前诊断率低,确诊主要依靠病理,鉴别心脏、下腔静脉原发肿瘤和血栓后,临床治疗主要是肿瘤彻底手术切除,术后短期未见复发。

SUMMARY Intravenous leiomyomatosis is characterized by a proliferation of benign smooth muscular tissue growing into uterine with malignant appearance. On extremely rare occasions, the tumor may grow out of the pelvis and extend into the inferior vena cava and the right atrium. We report a case of intravenous leiomyomatosis extending into the right atrium. A 41-year-old woman complained of 20 days of intermittent abdominal pain and lower limbs swell. Medical history of the patient revealed a previous hysterctomy operation 3 years ago due to uterine leomyoma. Echocardiography showed a homogenous mass extending from the inferior vena cava to the right atrium, without evidence of adherence to the right atrial wall, the left ventricular ejection fraction was only 60%. Computer tomography showed that a large mass arising from the left internal iliac vein and extending into the right chambers. Pelvic vascular ultrasound revealed the thrombotic material in the inferior vena cava and the left common iliac vein, and confirmed the presence of a complex mass in the left annex region. Based on the findings, the initial diagnosis was intracardiac and intravenous tumor. An operation was performed through a sternotomy and laparotomy to remove the whole tumor from the left common iliac vein to the right atrium, ligate left internal iliac vein meanwile. No hormonal therapy was administrated after the operation. Immunohistoehemieal studies revealed that the tumor cells were fusiform shape, there was no karyokinesis and necrosis, and the tumor cells were positive for smooth muscle antigen and desmin, as well as estrogen receptor and progesterone receptor. Six months postoperation follow-up revealed no signs of recurrence. The differential diagnosis of the disease compared with primary cardiovascular sarcomas and thrombus was difficult. The final diagnosis relied on immnnohistochemical analysis, however, the short-term result of operation was acceptable.