MALIGNANT FIBROUS HISTIOCYTOMA OF THE PAN CREAS

Malignant fibrous histiocytoma (MFH), a frequent sarcoma in retroperit oneal mesenchimal tissues, has also been reported, although in a few c ases, in digestive viscera. We present a case of a primary MFH of the pancreas in a 37-year-old male, treated with a 90% distal Pancreatecto my, plus external and intraoperative radiotherapy over the tumoral bed , and polychemotherapy. Response to treatment was poor, with early loc al recurrence, liver and bone metastases and a survival of 7 months. O nly 4 other cases of MFH of the pancreas have been reported. We analyz e these 5 cases: they have been usually diagnosed in young adults, wit h vague symptoms and large tumoral masses without jaundice. Local recu rrence seems to be frequent; they develop liver metastases rather than lung metastases. Even when they are resectable, the anatomical relati ons of the pancreas with vital vascular structures make broad resectio n difficult, and local recurrences frequent. Analysis of these five re ported cases of MFH of the pancreas, allows to expect a local recurren ce rate higher than 50% and a 5-year-survival rate lower than 30% due to distant metastases. Only radiotherapy seems to play a role as an ad juvant treatment after surgery for pancreas and other retroperitoneal sarcoma as it significantly increases survival.