Abstract
OBJECTIVES: Primary hepatic leiomyosarcoma (PHLMS) is an exceedingly rare malignant tumor originating from the liver with only a handful of cases reported in literature. Herein, we present five cases of PHLMS diagnosed and managed at our institute. RESULTS: The patients presented with non-specific symptoms, e.g., abdominal pain, lump, jaundice, etc., and imaging showed a space-occupying lesion in the liver. Hence, histopathological examination plays a pivotal role in diagnosis, revealing characteristic features of spindle-shaped cells arranged in fascicles or bundles with high mitotic rate and varying degrees of necrosis. Only 1 out of 5 patients underwent surgery. The remaining patients were planned with either palliative chemotherapy or best supportive care based on their performance status. The prognosis is however poor in such patients. CONCLUSION: Despite aggressive management, prognosis of PHLMS remains guarded. Surgical resection is the backbone of a radical treatment. This case series presents a rare entity providing insights into the diagnosis, management, and prognosis of the patients with PHLMS.