The case of a female patient with an alpha-fetoprotein (AFP)-producing acinar cell carcinoma of the pancreas is reported, and 28 cases in the literature are reviewed. In our case, the serum AFP level declined drastically after removal of the tumor, but increased when widespread metastases appeared. AFP was detected in the cytoplasm of the cancer cells by immunohistochemical staining. Immunoelectron microscopic studies revealed AFP on the endoplasmic reticulum of the cancer cells. Of the 28 cases with AFP-producing pancreatic cancer, liver metastases were identified in 21 cases (76% overall). There was no correlation between the serum AFP level and liver metastases. Immunohistochemical studies revealed localization of AFP at the primary lesion in 6 out of eight cases tested. In cases of AFP-producing pancreatic cancer, serum AFP levels are useful for the diagnosis and as a marker for evaluating recurrent disease and therapeutic response, and for the management of gastrointestinal disease it should be remembered that some pancreatic cancers produce AFP.

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