Solitary Liver Mass in a Patient with Hepatitis C
A 64-year-old man with a history of intravenous drug use and chronic hepatitis C virus (HCV) presented with shortness of breath, 1 month of severe right upper quadrant abdominal pain, and a 10-pound weight loss. He denied fever, nausea, or vomiting. He consumed alcohol rarely. Examination revealed a mildly tender liver, without jaundice, ascites, or asterixis.
Laboratory studies were significant for immunoglobulin G antibodies to HCV type 3a, aspartate aminotransferase 113 U/L (normal, 10–50 U/L), alanine transaminase 53 U/L (normal, 10–50 U/L), alkaline phosphatase 168 U/L (normal, 40–129 U/L), γ-glutamyltransferase 304 U/L (normal, 8–61 U/L), serum albumin 2.4 g/dL (normal, 3.5–5.2 g/dL), and international normalized ratio 1.6 (normal, 0.9–1.3).
Abdominal computed tomography scan identified a large, solitary hepatic mass, measuring approximately 15 cm in diameter, with areas of necrosis and calcification (Figures A, B). Because of the patient's history of HCV, alpha-fetoprotein was evaluated for suspected hepatocellular carcinoma (HCC) and was mildly elevated, 29 ng/mL (normal, 0–8 ng/mL).
Pathologic examination by H&E of a liver biopsy demonstrated a high-grade spindle-cell neoplasm characterized by short spindle cells showing focal short fascicles and hyperchromatic, pleomorphic nuclei with nondescript cytoplasm (Figure C). Immunohistochemical staining revealed well-developed vimentin reactivity (Figure D), as well as reactivity for CD99, CD56, FLI-1, α1-antitrypsin (Figures E–H, respectively), and α1-antichymotrypsin. On the basis of the staining for vimentin, in the absence of immunophenotypic evidence of epithelial or more specific mesenchymal differentiation, the neoplasm was ultimately classified as an undifferentiated sarcoma.
The diagnosis of primary sarcoma of the liver is exceedingly uncommon, representing <1% of all hepatic malignancies.1 This is especially true of patients with long-standing HCV infection, in whom HCC presents with an annual incidence of 1%–8%.2, 3 In contrast to patients with HCC, patients with primary sarcomas of the liver will have only mildly elevated or negative alpha-fetoprotein level.
Data on appropriate management of patients with primary sarcoma of the liver are limited; however, treatment options include surgical resection, radiation, and chemotherapy.1, 4 In spite of these interventions, however, the prognosis for patients with primary hepatic sarcoma is overwhelmingly poor, with a reported mean survival of 6–10 months after diagnosis.1, 4 In the present case, the patient was evaluated for surgical resection but opted for palliative care, and he died 2 months after his diagnosis. His presentation underscores the importance of arriving at a specific histologic diagnosis, even in the setting of a strong presumptive diagnosis, to formulate a prognosis and optimal therapeutic plan.