Section: Hepatobiliary Sub-section: Liver Curriculum: Curriculum, page 85
- Definition:
- Rare variant of HCC.
- Defined as well-differentiated polygonal hepatic tumour cells with an eosinophilic granular cytoplasm surrounded by a fibrous lamellar stroma
- Epidemiology:
- 1% of HCC.
- Younger age (20-35 years).
- Not due to chronic liver disease.
- Women > men.
- Presentation:
- Usually large at time of diagnosis (8-10 cm)
- Symptoms: palpable mass, abdominal pain, weight loss, malaise, anorexia
- Imaging:
- Large solitary hypervascular heterogenous liver mass with central hypodense region due to central necrosis or fibrosis.
- On MRI, central scar has low attenuation on T2 (as opposed to high attenuation in FNH).
- Well defined margins and calcification in 68%
- Pathology:
- Histology: Deeply eosinophilic, polygonal neoplastic cells surrounded by dense, layered fibrous stroma.
- Lymph node invasion within hepatic pedicle is frequent (60%).
- Treatment:
- Resection. Transplant has little or no place.
- Lymphadenectomy recommended.
- Prognosis:
- Better than HCC. 5-year survival after resection 50-75%
- Recurrence:
- High risk within liver, LNs or distant mets
- Repeat surgery reasonable option due to relatively indolent course of disease and inefficacy of non-surgical treatments
- Follow-up
- Close long-term follow-up mandatory. Recurrence and death beyond 5 years common.