| Summary: | Primary hepatic leiomyosarcoma is extremely rare among cases of liver tumors in adults, with an incidence of 0.1 and 1%. This paper describes the case of a 55 year-old man with a clinical evolution of five months consisting of abdominal pain, a large hard lump, weight loss, shortness of breath and fever.
A three-phase computed tomography (CT) showed a hypercaptive mass at its periphery during hypodense arterial phase at its center, located in segments VI and VI, without a plane of separation of the liver. Due to the symptoms, the patient underwent an exploratory laparotomy, finding a cerebroid mass of 40 x 40 cm; a lumpectomy without hepatectomy was performed, leaving free surgical margins.
The diagnosis was largely made through a histopathological assessment, finding stromal multinucleated pleomorphic forms, desmin (+), SMA (smooth muscle actin) and MSA (muscle specific actin) (+), Ki67 (+) and negative for S100 (protein S100) and CD117 antibody, which confirmed the high grade pleomorphic leiomyosarcoma diagnosis. The patient was discharged 16 days after admission once his condition improved, and was referred to the oncology department for adjuvant chemotherapy.
Given the size of the mass, the prognosis was bleak, which left surgery as the only option to offer survival expectations through regulated or "atypical" hepatectomies along with safety margins and liver transplantation. With this in mind, the first option was chosen; six months after surgery, with clinical improvement and adjuvant therapy, the patient, still with unfavorable prognosis, remained stable attending multidisciplinary medical management controls.
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