Published online Apr 27, 2022. doi: 10.4254/wjh.v14.i4.860
Peer-review started: January 10, 2022
First decision: January 22, 2022
Revised: January 22, 2022
Accepted: March 6, 2022
Article in press: March 6, 2022
Published online: April 27, 2022
Processing time: 101 Days and 11.8 Hours
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.
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.
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.
Core Tip: Sarcomas comprise only 1%-2% of all primary liver malignancies, and leiomyosarcoma is even rarer. We report a rare case of primary hepatic leiomyosarcoma accidentally diagnosed and surgically treated. The diagnosis requires a high level of suspicion because the clinical scenario and cross-imaging are not specific. Thus, histological examination is the only way to reach the diagnosis. In our case, the tumor probably originated from the left branch of the portal vein or the round ligament. Radical hepatectomy is the cornerstone of treatment. However, this tumor has aggressive metastatic potential and is usually diagnosed in locally advanced or metastatic disease. Two years after surgical resection, our patient is alive and with no evidence of tumor recurrence, likely because the diagnosis was established at an early stage and the surgery achieved a tumor-free margin.