ABSTRACT
Primary Hepatic Leiomyosarcoma are very rare Tumours with only 30 cases reported Worldwide1. Patient complains of nonspecific symptoms and often presents with Enlarged Liver. Diagnosis is usually delayed and patients have poor prognosis2. 35-year-old woman presented to Outpatient Department (OPD) complaining of dull aching pain and mass in upper part of abdomen for 6 months. On palpation liver was enlarged and lower border extended up to umbilicus. Ultrasonography of abdomen showed Liver enlarged 23 cm with multiple hypoechoic nodules noted in both lobes of liver. Triphasic Contrast-Enhanced Computed Tomography (CECT) abdomen multiple variable sized Peripherally Enhanced Hypodense Nodules in both lobes of Liver suggestive of Multifocal Hepatocellular Carcinoma (HCC)/ Metastasis. Tumour markers S alpha fetoprotein, S. CA 19-9, S. CEA was within normal limits. CECT Chest was normal. Positron Emission Tomography and Computed Tomography (PET-CT) was done, which showed Liver enlarged 24.5cm with FDG avid multiple nodules in both lobes of Liver and multiple enlarged FDG avid periportal, Portocaval and Peripancreatic Lymph Nodes. Rest of body organs were normal. Biopsy from the Liver showed Mesenchymal Tumour composed of spindle cells arranged in fascicular growth pattern, nucleus cigar shaped with atypia and mitotic figures seen. On Immunohistochemistry (IHC) Tumour was positive markers were Smooth Muscle Actin (SMA), VM, Desmin and H-caldestron and negative for CK, S-100, SOX-10, CD-117 and Dog-1. From above work up a diagnosis of primary Leiomyosarcoma of Liver was made. Palliative Chemotherapy was offered to the patient.