We report the case of a 53-year-old man who presented a mass involving the head of the pancreas resulting in isolated jaundice. Histologically, the tumor was an undifferentiated carcinoma with osteoclast-like giant cells of the pancreas. This very rare neoplasm resembles giant cell tumor of bone. An epithelial origin is now established and this tumor has been recently considered as a variant of ductal adenocarcinoma of the pancreas in the last WHO histological classification. The diagnosis requires both morphology and immunohistochemistry. Although the prognosis of these tumors is reported to be poor, our case is unusual because of a favorable outcome without relapse after 2 years.
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