Malignant fibrous histiocytoma (MFH), a frequent sarcoma in retroperitoneal mesenchimal tissues, has also been reported, although in a few cases, 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 pancreatectomy, plus external and intraoperative radiotherapy over the tumoral bed, and polychemotherapy. Response to treatment was poor, with early local recurrence, liver and bone metastases and a survival of 7 months. Only 4 other cases of MFH of the pancreas have been reported.
We analyze these 5 cases: they have been usually diagnosed in young adults, with vague symptoms and large tumoral masses without jaundice. Local recurrence seems to be frequent; they develop liver metastases rather than lung metastases. Even when they are resectable, the anatomical relations of the pancreas with vital vascular structures make broad resection difficult, and local recurrences frequent. Analysis of these five reported cases of MFH of the pancreas, allows to expect a local recurrence 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 adjuvant treatment after surgery for pancreas and other retroperitoneal sarcoma as it significantly increases survival.
CITATION Rev Esp Enferm Dig. 1993 Jun;83(6):475-80
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