Malignant fibrous histiocytoma is a soft tissue sarcoma of mesenchymal orig
in. It can rarely present as a primary gallbladder tumor with only five cas
es having been reported to date in the English literature. Here we report t
he sixth documented case of malignant fibrous histiocytoma of the gallbladd
er, and we review all other cases reported. The outcome of the visceral sar
comas is poor when compared with tumors arising from the soft tissues. The
treatment of primary malignant fibrous histiocytomas of the gallbladder is
surgery. However, tumor recurrence is the norm even if wide clean margins a
re obtained. In contrast to tumors arising from the extremities the role of
adjuvant radiotherapy and chemotherapy is less clear in the case of retrop
eritoneal and visceral sarcomas. Our patient is still alive and free of dis
ease 46 weeks after surgery. The fact that this is the longest survival rep
orted to date underscores the dismal prognosis of this disease.