Abstract
Background: Adenoma–borderline tumor–carcinoma sequence, which was substantiated in the pathogenesis of ovarian mucinous carcinoma, could be extrapolated to that of primary retroperitoneal mucinous cystadenocarcinoma. Nevertheless, direct evidence is still insufficient due to its rarity. Case: A 34-year-old Japanese woman with huge unilocular retroperitoneal cyst (16×8 cm) refused surgery. The cyst was gradually shrunken, measuring 10×4 cm at the age of 40. One year later, however, remarkable enlargement of the cyst (17×14 cm) with the emergence of three mural nodules was noted. The cyst was surgically removed intact. Histologically, a benign single layer of mucin-producing epithelial cells lined most of the cystic wall with focal microinvasive adenocarcinoma localized to the bases of mural nodules. Conclusion: Primary retroperitoneal mucinous cystadenocarcinoma could arise from the pre-existing benign mucinous cystadenoma.
Highlights
The retroperitoneum is an area where various soft tissue tumors could arise from the inherent structures, including muscle, nerve, and vascular tissue
Primary retroperitoneal mucinous cystadenocarcinoma could arise from the pre-existing benign mucinous cystadenoma
Majority of primary retroperitoneal mucinous tumors grow as a unilocular cyst [7] in contrast to ovarian mucinous tumors that usually give a multilocular appearance with numerous septations, retroperitoneal and ovarian mucinous tumors could share similar pathogenesis
Summary
Primary retroperitoneal mucinous cystadenocarcinoma could arise from the pre-existing benign mucinous cystadenoma.
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