探讨原发性肝平滑肌肉瘤（PHL）临床病理特征及疗效。

回顾性分析2000年1月至2019年12月西安交通大学第一附属医院收治的3例手术切除并行病理检查确诊为PHL的患者临床病理资料，同时通过查阅国内外文献报道的46例经手术切除和病理确诊的PHL患者病例资料。采用HE和免疫组织化学法检测肿瘤内SMA、vimentin、desmin、Ki-67、CD34和actin表达，总结其临床病理特点及疗效。

共49例PHL病例资料纳入研究，其中男19例，女30例；年龄5个月～77岁，中位年龄50岁。表现为腹痛21例，腹部不适12例，发烧2例，打嗝1例，头晕1例，消化不良1例，9例无明显临床症状，未提供2例。肿瘤最大直径2.2～25.0 cm，中位直径为15.6 cm。超声检查表现为低回声，其内可见分支状血流信号；CT增强扫描不均匀强化；MRI表现为延迟增强。免疫组化法染色显示：SMA阳性率88%（35/40）、vimentin阳性率50%（20/40）、desmin阳性率45%（18/40）、Ki-67阳性率15%（6/40）、CD34阳性率5%（2/40）、actin阳性率5%（2/40）、而溶菌酶、a1-抗胰蛋白酶、CALP、HSP、P53及healdesmon阳性率均为2.5%（1/40）。本院收治3例获有效随访，中位生存时间22.5个月；随访期间复发1例，肝衰竭1例，转移1例。46例文献报道病例均获有效随访，其中单发病例中位生存时间为8个月，多发为17个月。死因为肝衰竭4例，多器官衰竭3例，复发5例，转移5例，腹腔内出血2例，肿瘤自发破裂出血1例，呼吸循环衰竭1例，大出血休克1例。

PHL临床罕见，临床表现及检测指标缺乏特异性，术前诊断困难，预后相对较差。增强MRI的延迟成像可能有助于PHL与其他肝肿瘤的鉴别诊断，确诊主要依靠病理学检查。手术切除是PHL主要治疗方案，与辅助化疗和（或）放疗联合可能获得较好的预后。

To investigate the clinicopathological characteristics and clinical efficacy of primary hepatic leiomyosarcoma (PHL).

Clinicopathological data of 3 patients who underwent surgical resection and were pathologically diagnosed with PHL in the First Affiliated Hospital of Xi'an Jiaotong University from January 2000 to December 2019 were retrospectively analyzed. Domestic and overseas literatures including, 46 patients undergoing surgical resection and pathologically diagnosed with PHL were retrieved. The expression levels of SMA, vimentin, desmin, Ki-67, CD34 and actin in tumors were detected by HE and immunohistochemical staining. Clinicopathological characteristics and clinical efficacy were summarized.

49 PHL patients were enrolled in this study, including 19 male and 30 female, aged from 5 months to 77 years, with a median age of 50 years. 21 patients presented with abdominal pain,12 cases of abdominal discomfort, 2 cases of fever, 1 case of burping, 1 case of dizziness, 1 case of dyspepsia, 9 cases without obvious clinical symptoms, and 2 cases of unknown data. The maximal tumor diameter was ranged from 2.2 to 25.0 cm, with a median diameter of 15.6 cm. Ultrasound examination showed hypoechoic sign, and branching blood flow signals could be seen within the hypoechoic sign. Enhanced CT scan revealed non-uniform enhancement. MRI showed delayed enhancement. Immunohistochemical staining showed that the positive rate of SMA was 88%(35/40), 50%(20/40) for vimentin, 45%(18/40) for desmin, 15%(6/40) for Ki-67, 5%(2/40) for CD34, 5%(2/40) for actin, and 2.5%(1/40) for lysozyme, a1-antitrypsin, CALP, HSP, P53 and healdesmon, respectively. 3 patients admitted to our hospital were subject to follow-up, and the median survival was 22.5 months. During subsequent follow-up, 1 case recurred, 1 case of liver failure and 1 case of metastasis. According to literature review, 46 cases were subject to follow-up. The median survival of single-onset patients was 8 months, and 17 months for multiple-onset cases. 4 cases died from liver failure, 3 cases of multiple organ failure,5 cases of recurrence, 5 cases of metastasis, 2 cases of intra-abdominal hemorrhage, 1 case of spontaneous rupture of the tumor, 1 case of respiratory and circulatory failure, and 1 case of hemorrhagic shock, respectively.

PHL is rare in clinical practice, lacking of specific clinical manifestations and detection parameters. It is difficult to make preoperative diagnosis and clinical prognosis is relatively poor. Delayed contrast-enhanced MRI may contribute to differential diagnosis of PHL from other liver tumors. Final diagnosis mainly depends on pathological examination. Surgical resection is the main treatment for PHL, and combination with adjuvant chemotherapy and/or radiotherapy probably yield better prognosis.