Primary Hepatic Leiomyosarcoma: A Case Series Highlighting a Rare Malignancy

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.