Abstract
BACKGROUND: Primary hepatic leiomyosarcoma is a very rare entity that originates from smooth muscle. Preoperative diagnosis requires a high degree of suspicion due to atypical clinical presentation and non-specific imaging features. CASE SUMMARY: We report the case of a 42-year-old man, with no relevant past medical history, accidentally diagnosed with a nodular liver lesion on a routine abdominal ultrasound. Liver function tests and hematology parameters as well as tumor markers were normal. A contrast-enhanced abdominal computed tomography scan revealed a heterogenous hepatic lesion measuring 40 mm 30 mm, adjacent to the left branch of the portal vein and the round ligament. Due to the unclear diagnosis, the patient underwent surgical resection. Histopathological and immunohistochemical examinations confirmed complete (R0) resection of a hepatic leiomyosarcoma. The patient remains without any signs of tumor recurrence for more than 2 years. CONCLUSION: We report a rare case of accidentally diagnosed primary hepatic leiomyosarcoma originating from the portal vein or the round ligament. Although this tumor has aggressive metastatic potential, a tumor-free resection margin is essential to improve survival.