Primary hepatic malignant fibrous histiocytoma: diagnostic pitfalls and therapeutic challenge.

Background: Due to the rarity of primary hepatic malignant fibrous histiocytoma (MFH), the natural history, optimal management and prognosis are poorly characterized.

Methodology: Between January 2003 and December 2008, we treated 12 consecutive patients with primary hepatic MFH. The patient demographics, tumor characteristics, type of treatment and actuarial survival were analyzed.

Results: The mean +/- SD tumor size was 8.4 +/- 3.2cm. Four patients had satellite lesions. R0, R1 and R2 resection of the liver tumor were achieved in 5, 2 and 5 patients, respectively. There was no hospital mortality and the complication rate was 8.3%. At a median follow-up of 11.3 months, local recurrence had occurred in 6 patients and local recurrence + distant metastases in 3 patients. Most patients (8/12) died of the tumor within a year after surgery, with a median survival of 6.1 months. For the remaining 4 patients, 2 patients had undergone surgery for less than 1 year previously, one patient who had a R0 liver resection with extrahepatic metastasis survived for 14 months with multiple metastases, and another patient who had a R0 liver resection but without extrahepatic metastasis survived for 60 months and was disease free. The median survival for the R0 liver resection group carried out in patients without extrahepatic metastases was 8.5 months, while the median survival of the debulking group (R0 liver resection with extrahepatic metastasis/ R1 or R2 liver resection) was 7 months. There was no significant difference in survival between the two groups.

Conclusion: Hepatic resection was safe for patients with primary MFH with a poor prognosis. Complete resection offers the only hope of long-term disease free survival.