| Summary: | Abstract Background Primary retroperitoneal mucinous cystadenocarcinoma (PRMC) is extremely rare, and its biological behavior, pathogenesis, optimum treatments, and prognosis remain to be elucidated. We herein report a case of PRMC with an 80-month follow-up. Case presentation A 29-year-old woman was diagnosed with unknown retroperitoneal tumor with benign right ovarian cyst and uterine fibroids, and she underwent laparotomy. The tumor was completely resected with a subsequent histopathological diagnosis of primary retroperitoneal mucinous cystadenocarcinoma (PRMC). Eighty months after surgery, she remains recurrence-free. Conclusion PRMC is an extremely rare tumor. Only around 60 cases have so far been published in the literature. The preoperative diagnosis of PRMC is difficult, and a definitive diagnosis can usually only be made based on the findings of histopathological examinations after surgery. Presently, only radical resection is useful for both diagnostic and therapeutic purposes. The optimal long-term management after surgery is still not well established. Further studies on PRMC are therefore needed to elucidate the etiology and establish effective treatments.
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