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.