embryonal sarcoma of liver

Epidemiology: - 6% of primary hepatic tumors of childhood - occurs between 5 and 20 years of age - rarely middle and old age Etiology: unknown Pathology: - malignant stellate cells or spindle cells - compactly or loosely arranged in myxoid stroma - multinucleated giant cells (may be multinucleated) - cytoplasmic eosinophilic globules of variable size - PAS positive, diastase resistant - express alpha-1-antitrypsin - entrapped bile ducts and hepatocellular elements Clinical manifestations: - abdominal enlargement - fever - weight loss - nonspecific gastrointestinal complaints

General

embryonal sarcoma liver neoplasm

References

WHO Classification - Tumours of the Digestive System. Hamilton & Aaltonen, eds. IARC Press 2000