A Rare and Aggressive Liver Cancer With Atypical Presentation: Primary Hepatic Angiosarcoma Presenting With Hypercalcemia

Introduction: Primary hepatic angiosarcoma (PHA) is a rare and aggressive form of liver cancer. It accounts for 0.1%-2% of all primary hepatic malignancies. PHA primarily originating from vessels or lymphatic endothelial cells and composed of abundant vasculatures. Prognosis is poor in inoperable cases and majority patients die in 6 months of diagnosis. We report a patient who presented with lethargy and fatigue related to hypercalcemia finally found to have primary hepatic angiosarcoma. Case Description/Methods: A 68- year-old man with diabetes mellitus and hypertension presented with fatigue, nausea and abdominal discomfort of 2 weeks duration. On examination: weak and lethargic patient with sign of dehydration. Normal vital signs except mild tachycardia. Laboratory work up showed elevated liver enzymes; AST 116, ALT 80, ALP 178 and T bilirubin of 1.2. High serum calcium (14.8) and ionized calcium of 1.93 level. Parathyroid hormone slightly low, otherwise normal parathyroid related hormone, Vitamin D-25, D1,25 and complete blood count. Immunoglobulins IgG, IgA, IgM beta and alpha globulins were normal. Tumor markers AFP, CA19-9, CEA and PSA were all normal. CT abdomen and pelvis was notable for innumerable bilobar poorly enhancing liver lesions (Figure 1). Liver biopsy showed angiosarcoma. For completion of work up patient underwent CT chest and head were done and all negative. EGD and colonoscopy were unremarkable except diminutive hyperplastic polyp of transverse colon. Patient was hydrated, calcitonin and zoledronic acid were given to stabilize his calcium. His lesions are not amenable for resection and patient was started on chemotherapy. Discussion: Primary hepatic angiosarcoma is a rapidly progressive liver cancer with non-specific presentation. Due to the non-specificity of the symptoms, diagnosis can be challenging and can delay early diagnosis and intervention, ultimately leading to death. Imagining and biopsy are important modality in diagnosis, though sensitivity may not be absolute. High degree of clinical suspicion, early diagnosis and surgical intervention remain best way in reducing risk of mortality.Figure 1.: CT abdomen and pelvis showing innumerable poorly enhancing liver lesions in both right and left lobes.