Primary Hepatic Leiomyosarcoma: A Rare Diagnosis with an Anticipated Outcome

Abstract

Purpose: Primary hepatic sarcomas (PHS) represent <1% of all hepatic malignancies, and are classified according to their histologic features. Primary hepatic leiomyosarcoma (PHL) is one variant, which has poorly characterized prognostic factors and lacks an optimal management protocol due to its rarity. We present a case of PHS in a 34-year-old African-American male. He had a 7-month history of intermittent back pain before presenting with right upper quadrant abdominal pain, worsening dyspnea, and bilateral lower extremity swelling. On exam, he was found to have fever, abdominal distension with a palpable mass in the epigastric area, and +3 lower extremity edema. He had anemia (9.9 g/dL) and abnormal liver function tests. Radiologic imaging revealed a 17-cm complex mass in the right lobe of the liver. A liver biopsy was performed. Immunohistochemistry revealed tumor cells reactive for smooth muscle actin and desmin, which was consistent with high-grade leiomyosarcoma. He underwent right hepatic lobectomy via a thoracoabdominal approach. A 32 x 23 x 13.5 cm hemihepatectomy specimen was resected with portions of adherent diaphragm, leaving the diaphragmatic margin free of involvement. The patient subsequently received chemotherapy with Ifosfamide and mesna for residual tumor, which was well tolerated. The patient is doing relatively well 24 months after tumor resection, with no recurrence to date. This rare case of PHL is one of 30 cases reported in the English literature. Prognosis is influenced by the ability to resect the tumor completely. Liver transplantation (OLT) in such patients was accompanied by early high recurrence with poor outcomes after 1 year. In our patient, a good outcome was obtained with resection and chemotherapy, despite having advanced disease. International multi-registry efforts need to be established to assess the most effective therapeutic modalities for PHL.Figure: Primary hepatic leiomyosarcoma: 32 x 23 x 13.5 cm specimen.