Uterine Adenosarcoma Originating in Adenomyosis: Report of an Extremely Rare Phenomenon and Review of Published Literature

Abstract

Müllerian adenosarcoma is an uncommon biphasic malignant tumor most often occurring in the uterine corpus and derived from native surface endometrium. We report a case of intramural uterine adenosarcoma arising in association with adenomyosis, in the absence of tumor involving the surface endometrium. This is an extremely rare phenomenon, with only 8 other published cases of uterine corpus adenosarcoma in the absence of surface endometrial involvement, 5 originating in adenomyosis and 3 in adenomyomas. We review these cases. The current FIGO staging system for uterine adenosarcoma assumes origin from the surface endometrium and does not address the rare occurrence of intramural tumors without a surface endometrial component. Such tumors are problematic to stage and could potentially be overtreated, particularly if there is deep myometrial involvement.

Mucinous carcinoma originating from uterine adenomyosis: a case report

“…Demarcated nodules were not observed on preoperative MRI or included in macroscopic findings of the resected uterus. Many cases of malignant tumors originating from adenomyosis exhibited demarcated lesions in the myometrium [ 6 , 7 , 15 , 17 , 20 ], and abnormal proliferation, as observed in our case, has not been reported to our knowledge. Moreover, the preoperative serum CA19-9 level in our case was markedly high at 115,950 U/mL.…”

“…The most frequent histological subtype of malignant lesions is endometrioid carcinoma [ 3 ]. In our literature review, we found 23 well-documented cases of other histological subtypes as follows: 4 clear cell carcinomas [ 3 – 6 ], 4 serous carcinomas [ 4 , 7 , 8 ], 6 serous endometrial intraepithelial carcinomas [ 8 , 9 ], 6 adenosarcomas [ 10 – 15 ], and 1 case each of adenosquamous carcinoma [ 16 ], carcinosarcoma [ 17 ], and mucinous carcinoma (minimal deviation adenocarcinoma; MDA) [ 18 ]. Therefore, the current case is the second case of mucinous carcinoma originating from uterine adenomyosis.…”

Endometrial Cancer Arising in Adenomyosis (EC-AIA): A Systematic Review

“…For the "unbiased study endpoints" domain, the risk of bias was low in 14 studies [13,14,17,18,20,24,[29][30][31][32][33]37,39,40,42], unclear in 11 studies [15,16,19,22,23,[26][27][28]34,35], and high in six studies [12,21,25,36,38,41]; in particular, in the studies at high risk of bias, Colman and Rosenthal's criteria were only partially fulfilled.…”

“…All included studies were case reports [36][37][38][39][40][41], except for four case series [12,34,35,42] (Table 1).…”

“…For the "patient selection" domain (when applicable), two studies were considered at low risk of bias [12,42], while two studies were judged as having unclear risk of bias [34,35]. For the "study endpoints" domain, the risk of bias was considered low in 15 studies [12][13][14]17,18,20,21,24,[29][30][31][32][33]37,41,42], while it was unclear in 16 studies [15,16,19,22,23,[25][26][27][28][34][35][36][38][39][40].…”