Introduction: We present the case of a primary spleen mucinous cystadenocarcinoma. Several cases of this primary tumor of the spleen have been described worldwide so far. These tumors are classified as mucinous cystic neoplasms (MCN) and occur mainly in the ovaries and pancreas. Case report: The case concerns a 45-year-old female patient with an accidentally diagnosed splenic tumor with approximately 20 cm in size. Histopathological examinations, following a splenectomy, confirmed the presence of mucinous cystadenocarcinoma.
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