Primary Retroperitoneal Müllerian Adenocarcinoma: Report of Two Cases Treated With Radical Surgery

Primary retroperitoneal müllerian adenocarcinoma: a case report

“…A variety of different histological subtypes of PRMA, including serous ( 7 ), endometrioid ( 1 ), clear cell ( 8 ), mucinous ( 4 ), papillary ( 2 ), mixed (serous, mucinous, and endometrioid), and even malignant mixed müllerian tumor ( 2 , 9 ), has been reported in the literature. The serous, endometrioid, and clear cell histologies were the common types ( 3 ). In this report, our PRMA case had poor cell differentiation, and the histopathology was clear cell adenocarcinoma.…”

“…Primary retroperitoneal müllerian adenocarcinoma (PRMA) is an extremely rare entity, which often presents in elderly menopausal women and is highly aggressive. Several mechanisms of this clinical entity have been suggested, including retroperitoneal ectopic supernumerary ovarian tissue, retroperitoneal mono-dermal teratoma, coelomic metaplasia, the secondary müllerian system, and retroperitoneal endometriosis ( 3 ). The purpose of this study is to report a patient of PRMA and analyze the characteristics of imaging and histomorphology of PRMA to improve the understanding of this tumor.…”

Primary Retroperitoneal Carcinomas: New Insights into Pathogenesis and Clinical Management in Comparison with Ovarian Carcinomas and Carcinoma of Unknown Primary

“…Histological subtypes of retroperitoneal epithelial carcinomas include mucinous [ 8 , 9 , 10 , 11 , 12 , 13 ], serous [ 14 , 15 , 16 , 17 , 18 , 19 , 20 , 21 , 22 , 23 , 24 , 25 , 26 , 27 , 28 , 29 ], endometrioid [ 30 , 31 , 32 , 33 , 34 ], clear cell [ 35 , 36 , 37 , 38 ], and carcinosarcoma [ 39 , 40 , 41 ]. Morphologically, mucinous, serous, and endometrioid tumors resemble the phenotype of the colon, fallopian tube, and endometrium, respectively [ 42 ].…”

“…Of note, a few cases reported as primary retroperitoneal serous carcinoma might not be primary but lymph node metastasis from adnexal or peritoneal carcinoma [ 26 ], although there is a possibility that they may be double synchronous primaries. Retroperitoneal endometrioid and clear cell carcinomas are even rarer, as only five and four cases have been reported, respectively ( Table 3 ) [ 30 , 31 , 32 , 33 , 34 , 35 , 36 , 37 , 38 ]. Whereas endometrioid carcinomas developed only in women, two of the four clear cell carcinomas developed in men [ 36 , 38 ].…”

“…These retroperitoneal carcinomas may be diagnosed as carcinomas of unknown primary [38]. Mixed epithelial carcinomas (Table 3) [37,44] and carcinosarcoma [39][40][41] also develop in the retroperitoneal space.…”