In the English medical literature, 27 cases of primary retroperitoneal mucinous cystadenocarcinoma have been published. We report the second case of a primary retroperitoneal mucinous cystadenocarcinoma in a man. The patient was an 83-year-old man, with a large 26 x 20 x 16-cm retroperitoneal cystic mass causing abdominal discomfort and cachexia, who underwent excision of the mass. Prior reports suggest that this type of tumor has an aggressive clinical course, and surgical excision is the treatment of choice. These rare tumors need to be included in the differential of retroperitoneal cystic tumors.
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