Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is characterized by failure of embryologic growth of the mullerian ducts resulting to agenesis or hypoplasia of the uterus and upper part of the vagina while both ovaries and fallopian tubes are normal. Various associated malformation with MRKH syndrome are noted in literature, with a renal system anomaly as the most common. However, adnexal tumors in MRKH syndome are rare. To date there have been no reported cases of fallopian tube tumors in patients diagnosed with MRKH. This paper presents a case of an 18 year old nulligravida who presented with primary amenorrhea secondary to MRKH sydnrome, with an associated Papiliary Serous Cystadenofibroma of the right fallopian tube Management of the case as well as review of related literature are presented. 

Cystadenofibroma

Most clear cell tumors of the ovaries are carcinomas; benign and borderline clear cell tumors are uncommon. We describe here a 52-year-old postmenopausal woman with an ovarian clear cell cystadenofibroma that was misdiagnosed before surgery as a borderline malignant cystic mass of the ovary. The ovarian mass had cystic and solid components. Histological examination revealed widely spaced simple glands embedded in a dense fibrous stroma. The glands were lined with one to two layers of hobnail cells, flattened cells, or cells with abundant clear cytoplasm. The patient successfully underwent a left oophoro-salpingectomy.
Cystadenofibroma ; Cytoplasm ; Female ; Humans ; Middle Aged ; Ovary

Ovarian clear cell adenocarcinomas (CCACs) are frequently associated with endometriosis and, less often with clear cell adenofibromas (CCAFs). We encountered a case of ovarian CCAC arising from benign and borderline adenofibromas of the clear cell and endometrioid types with endometriosis in a 53-year-old woman. Regions of the adenofibromas showed transformation to CCAC and regions of the endometriosis showed atypical endometriotic cysts. This case demonstrates that CCAC can arise from CCAF or endometriosis.
Adenocarcinoma, Clear Cell* ; Adenofibroma* ; Cystadenofibroma ; Endometriosis* ; Female ; Humans ; Middle Aged ; Ovary*

Familial adenomatous polyposis (FAP) is a hereditary disease characterized by the appearance of numerous polyps in the large bowel with a high potential for malignant transformation unless untreated. A variety of extracolonic manifestations were reported such as osteoma, epidermoid cyst, desmoid tumor, gastroduodenal polyps, small bowel tumor, congenital hypertrophy of the retinal pigment epithelium, hepatobiliary tumor, thyroid tumor, and tumor of the central nervous system. However, the ovarian involvement of FAP as an extracolonic manifestation was very rare and there have been only few reports. We experienced a rare case of ovarian cystadenofibroma in a patient with FAP as an extracolonic manifestation. We also found colon cancer with multiple hepatic metastasis initially manifested as intestinal obstruction in the same patient. Surgical treatment and subsequent chemotherapy for colon cancer and intraoperative radiofrequency ablation of hepatic metastasis were performed.
Adenomatous Polyposis Coli* ; Catheter Ablation ; Central Nervous System ; Colonic Neoplasms ; Cystadenofibroma* ; Drug Therapy ; Epidermal Cyst ; Fibromatosis, Aggressive ; Genetic Diseases, Inborn ; Humans ; Hypertrophy ; Intestinal Obstruction ; Neoplasm Metastasis ; Osteoma ; Polyps ; Retinal Pigment Epithelium ; Thyroid Gland

OBJECTIVETo investigate the expression and promoter methylation status of p73 gene in ovarian epithelial tumors and their clinicopathological correlations.

METHODSTissue microarrays (TMA) consisting of 68 ovarian cancers, 37 ovarian borderline tumors and 21 ovarian benign tumors were constructed. p73 expression was detected by immunohistochemistry (EnVision method). Fresh-frozen tissue samples from 13 cases of ovarian carcinomas and 5 cases of borderline tumors were evaluated for the presence of p73 promoter methylation using bisulfite sequencing.

RESULTSOverall, 92.6% (63/68) ovarian carcinomas expressed p73, with a mean value of 32% (percentage of p73 positive cells in the tumor). The mean value of p73 expression rate (40%) in serous carcinoma (26/26) was higher than those of other cancer types (P = 0.006). The mean value of p73 expression rate (40%) in type II ovarian carcinoma was significantly higher than that in type I ovarian carcinoma (24%, P = 0.010). The expression of p73 was not associated with FIGO stage and histological grade (both P > 0.05). The mean values of p73 expression in ovarian borderline tumor (30/37) and benign tumor (12/21) were 16% and 15%, respectively. Of the two groups, the mean value of p73 expression rate in serous type was higher than that in mucous type (P = 0.003, P = 0.026). Ovarian carcinomas had a higher level of p73 expression than borderline tumors and benign tumors (both P < 0.05), while that between ovarian borderline tumors and benign tumors had no statistical difference (P > 0.05). Among serous tumors (49/53), the mean value of p73 expression in the carcinoma group (26/26) was significantly higher than those in the borderline tumor group (12/14) and benign tumor group (11/13; P = 0.024 and P = 0.002, respectively), while that between borderline tumor group and benign tumor group had no statistical difference (P = 0.428). Among mucous tumors (15/27), the mean value of p73 expression in carcinoma group (6/7) was higher than that in benign tumor group (1/8; P = 0.032). No statistical difference of p73 expression was seen between the carcinoma group and ovarian borderline tumor group (8/12) and between the borderline tumor group and benign tumor group (P = 0.234, P = 0.201, respectively). p73 promotor methylation was found in 8 of 13 cases of carcinomas but at different methylation levels with a mean value of 8.0%. Two of 5 ovarian borderline tumors showed detectable p73 promotor methylation with a mean value of 9.0%. Compared with the borderline tumors, ovarian carcinomas showed a similar p73 methylation level (P > 0.05). The p73 methylation level in ovarian carcinomas was not associated with histological type, pathogenetic type, histological grade and FIGO stage (all P > 0.05).

CONCLUSIONSMost of ovarian epithelial tumors express p73 protein with mean values higher in ovarian carcinomas than those in the borderline and benign tumors. Ovarian serous carcinomas have the highest expression level of p73. A simple linear correlation does not exist between the promoter methylation and protein expression of p73.

Adult ; Aged ; Cystadenocarcinoma, Mucinous ; metabolism ; pathology ; Cystadenocarcinoma, Serous ; metabolism ; pathology ; Cystadenofibroma ; metabolism ; pathology ; Cystadenoma, Mucinous ; metabolism ; pathology ; Cystadenoma, Serous ; metabolism ; pathology ; DNA Methylation ; DNA-Binding Proteins ; metabolism ; Female ; Gene Expression Regulation, Neoplastic ; Humans ; Immunohistochemistry ; Middle Aged ; Neoplasm Grading ; Neoplasm Staging ; Neoplasms, Glandular and Epithelial ; metabolism ; pathology ; Nuclear Proteins ; metabolism ; Oligonucleotide Array Sequence Analysis ; Ovarian Neoplasms ; metabolism ; pathology ; Promoter Regions, Genetic ; Tumor Protein p73 ; Tumor Suppressor Proteins ; metabolism ; Young Adult