Mucinous adenocarcinoma of the endometrium with metastasis to the clitoral glans after pelvic exenteration for radiation resistant vaginal cuff recurrence

Abstract

Mucinous adenocarcinoma of the endometrium (MACE) is a rare subtype of endometrial adenocarcinoma that often presents a significant diagnostic challenge due to its variation from the conventional morphologic appearance of endometrioid epithelium. This case report is of a woman who has survived 4 years after pelvic exenteration and subsequent vulvectomy for recurrent MACE.

Endometrial Carcinomas with Intestinal-Type Metaplasia/Differentiation: Does Mismatch Repair System Defects Matter? Case Report and Systematic Review of the Literature

“…However, the extent of IM/diff areas required to increase the aggressiveness of an EC is unknown; in the majority of papers, it was unclear if IM/diff features were focal or diffuse. With the exception of case described by Mogor in which the recurrent tumor exhibited intestinal differentiation, in the remaining cases, including our one, histological evaluation of the metastatic/recurrent tumor was not performed, and for this reason, it is not clear if the intestinal component is more aggressive in comparison to the endometrioid one [13].…”

“…AEH was detected in the background endometrium of two cases: one a mixed form, while it was unclear if an endometrioid component was present in the second case. In both patients, no IM/diff was found in benign glands [11,13]. Alternative pathogenic mechanisms and precursors beside AEH may be considered for pure forms: the tumor reported by Rubio arose after previous removal of an endometrial polyp with IM/diff, and AEH was not found in the background endometrium [14].…”

“…Table 2 summarizes the clinical and pathological features, treatment modalities, and outcomes of all published cases of primary ECs with IM/diff [ 9 , 10 , 11 , 13 , 14 , 15 , 16 ]. Mean and median age were respectively 64 and 62 years (range 49–81 years).…”

“…We performed a systematic review of the English Literature in multiple databases and identified only eight previous cases of EC with IM/diff, arising in patients of 49 to 81 years of age ( Table 2 ) [ 9 , 10 , 11 , 13 , 14 , 15 , 16 ]. IM/diff can manifest in ECs with a wide spectrum of morphological features, ranging from the presence of sole goblet cells to the association of goblet cells with additional gastrointestinal-type cells, such as enterocyte-like cells, neuroendocrine cells, and Paneth-like cells.…”

“…Finally, seven studies were included in the review, for a total of nine patients diagnosed with EC with IM/diff including the case reported by us. Table 2 summarizes the clinical and pathological features, treatment modalities, and outcomes of all published cases of primary ECs with IM/diff [9][10][11][13][14][15][16]. Mean and median age were respectively 64 and 62 years (range 49-81 years).…”