Surgical treatment of adult primary hepatic sarcoma

Background: Primary sarcomas of the liver are extremely rare in adults, Opt imal therapeutic approaches remain unclear. Methods: Twenty consecutive adult patients who had surgical treatment for p rimary hepatic sarcomas were reviewed. Patient age ranged from 23 to 80 yea rs, Other than one patient with primary hepatic angiosarcoma who had a hist ory of thorium dioxide colloid (Thorotrast) exposure 23 years before diagno sis, no predisposing causes were apparent, Nineteen patients had hepatic re section and one patient had an orthotopic Liver transplant. No patient rece ived neoadjuvant chemotherapy or radiotherapy but radiotherapy was delivere d intraoperatively in one: patient. Results: Leiomyosarcoma was the most common histological type of sarcoma di agnosed (five of 20 patients), followed by malignant solitary fibrous tumou r (four) and epithelioid haemangioendothelioma (three), Fourteen tumours we re high-grade sarcomas and six were low grade malignancies. Thirteen patien ts developed a recurrence. Distant metastases (ten patients) and intrahepat ic recurrence (six) were the predominant sites of initial treatment failure . Six patients received salvage chemotherapy. Histological grade was the on ly factor significantly associated with overall patient survival (P=0.03). With complete resection, patients with high-grade: tumours had a 5-year sur vival rate of 18 (95 per cent confidence interval 5-62) per cent compared w ith 80 (52-100) per cent for patients with low-grade tumours. The 5-year su rvival rate for all 20 patients was 37 (20-60) per cent. Conclusion: Surgical resection is the only effective therapy for primary he patic sarcomas at present. Better adjuvant therapy is necessary, especially for high-grade malignancies, owing to the high failure rate with operation alone.