Objective  To summarize the clinical data of primary squamous cell carcinoma of the liver (PLSCC) and provide a reference for the diagnosis and treatment of PLSCC.

  Methods  The papers about PLSCC were searched from PubMed, Web of Science, CNKI and Wanfang data up to October 2020, and the reported cases were collected to make a pooled analysis if their follow-up information were available.

  Results  A total of 67 literatures including 75 patients were included in our study, with a ratio of male to female of 1.59:1, age ranging from 18 to 83 years, and an average of 57.2 years. Most of the patients were admitted to the hospital with a complaint of abdominal pain. Laboratory work-up and imaging were helpful in diagnosis. A total of 24 patients underwent surgeries, 16 patients received non-surgical therapy such as hepatic artery chemoembolization (TACE), 16 patients received surgery and non-surgical therapy, and 19 patients received conservative therapy. In 75 patients, 50 deaths were observed and survival time of 33 patients was no more than half a year. The mean survival time of all the patients was (17.73±4.02) months, the median survival time was 6 months, and the 6-month, 1-year, 2-year survival rates were 49.5%, 32.5% and 16.3%, respectively. Combination treatment is the most beneficial treatment for patients to improve their survival. Univariate analysis showed that the number of tumors, the maximum tumor size, treatment therapy and keratinization were associated with prognosis (P < 0.05). Multivariate analysis showed that treatment method was an independent factor affecting the prognosis of patients(P < 0.05). The prognosis of the cases reported from 2003 to 2020 was better than that reported from 1972 to 2002, and the difference was statistically significant (P < 0.001).

  Conclusion  The clinical manifestations of PLSCC lack specificity, and its etiology and pathogenesis are not clarified. If patient's condition permits, active treatment should be taken as much as possible.