Background: Primary retroperitoneal mucinous adenocarcinomas are very rare tumors that resemble ovarian mucinous cystadenocarcinomas.
Case: A 39-year-old woman presented with a 20-cm cystic lesion. Following surgery final pathology revealed mucinous adenocarcinoma consistent with a primary retroperitoneal process. The patient responded favorably to 6 cycles of paclitaxel and carboplatin chemotherapy and currently has no evidence of disease.
Conclusion: During surgical resection of a primary retroperitoneal mucinous adenocarcinoma, ensuring that the lesion remains intact has clinically significant implications.
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