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

Biliary adenofibroma is a rare tumor with a bile duct origin characterized by a complex tubulocystic non-mucin secreting biliary epithelium with abundant fibrous stroma. The MRI features of biliary adenofibroma are not well established. The authors encountered two patients with biliary adenofibroma and reviewed the literature focusing on the MRI findings. A well-circumscribed multicystic tumor with septal enhancement and no intrahepatic bile duct communication may be the characteristic MRI findings of biliary adenofibroma.
Adenofibroma ; Bile Ducts ; Bile Ducts, Intrahepatic ; Epithelium ; Humans ; Magnetic Resonance Imaging

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*

OBJECTIVETo study the clinicopathologic features and differential diagnosis of recurrent Müllerian adenofibroma (MAF) of the uterus.

METHODSClinicopathologic information of 7 cases of recurrent MAF of uterus was retrieved from January 1992 to April 2006 and compared with 12 cases of MAF without recurrence and 14 cases of low-grade Müllerian adenosarcoma (MAS). EnVision immunohistochemistry of estrogen receptor (ER), progesterone receptor (PR), smooth muscle actin (SMA), CD10, Ki-67 and p53 were performed in all cases.

RESULTSAll cases of recurrent MAF of the uterus were polypoid, lobulated, and broad based mass arising from the corpus or cervix. Microscopically, the tumor consisted of benign epithelial and mesenchymal components with low mitotic activity ( ≤ 1/10 HPF). The clinical and pathologic features of 3 recurrent tumors were similar to their primary tumors, while 4 cases of recurrent tumor presented with focally higher cellularity and mitotic activity, meeting the diagnostic criteria of adenosarcoma. The stromal expression patterns of ER, PR, SMA and p53 in recurrent MAF were similar to those of clinically benign MAF and low-grade MAS. Negative or focally positive stromal cell expression of CD10 was seen infrequently in recurrent MAF (1/7) and clinically benign MAF (1/12). In contrast, a moderate to strong CD10 staining was frequently seen in MAS (9/14, P < 0.05). The difference of Ki-67-labeling index between MAF and MAS did not reach a statistical significance (P > 0.05). Ki-67-labeling index increased in areas of periglandular stromal cuffing as compared with interglandular areas in all MAS cases, but it was not observed in either recurrent MAF or clinically benign MAF cases.

CONCLUSIONSRecurrent MAF may be associated with aggressive behavior. It is difficult to distinguish MAF from low-grade MAS. CD10 and Ki-67 staining pattern in stromal cells may be helpful for the differential diagnosis.

Adenofibroma ; metabolism ; pathology ; surgery ; Adenosarcoma ; metabolism ; pathology ; Adolescent ; Adult ; Aged ; Aged, 80 and over ; Biomarkers, Tumor ; metabolism ; Diagnosis, Differential ; Female ; Humans ; Hysterectomy ; methods ; Ki-67 Antigen ; metabolism ; Middle Aged ; Neoplasm Grading ; Neoplasm Recurrence, Local ; Neprilysin ; metabolism ; Survival Rate ; Uterine Neoplasms ; metabolism ; pathology ; surgery ; Young Adult

Adenofibroma ; pathology ; Adenosarcoma ; metabolism ; pathology ; surgery ; Aged ; Diagnosis, Differential ; Endometrial Neoplasms ; pathology ; Female ; Follow-Up Studies ; Humans ; Hysterectomy ; Keratin-7 ; metabolism ; Middle Aged ; Mucin-1 ; metabolism ; Polyps ; pathology ; Postoperative Period ; Preoperative Period ; Uterine Neoplasms ; metabolism ; pathology ; surgery

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

Adenofibroma is an extremely rare benign mullerian mixed tumor composed of epithelium and mesenchymal cells. Most uterine adenofibromas occur in the endometrium, but they rarely protrude into the vagina. To date, only a few such cases with the imaging findings have been reported. Therefore, we report here on the sonographic and magnetic resonance (MR) imaging findings of a case of endometrial adenofibroma protruding into the vaginal cavity in a 28-year-old woman. The uterine adenofibroma appeared as a large intracavitary echogenic mass containing multiple small internal cysts, and it was distending the vaginal cavity on transrectal sonography. T2- weighted MR images showed a large intracavitary mass with heterogeneous high signal intensity protruding into the vaginal cavity. On gadolinium-enhanced T1-weighted MR images, heterogeneous septa-like enhancement was noted in the mass. Although uterine adenofibroma is extremely rare, adenofibroma can be suggested as a possible diagnosis when an intracavitary uterine mass, with multiple internal small cystic components and enhancing septa-like structures, is protruding into the vaginal cavity on imaging.
Adenofibroma ; Adult ; Endometrium ; Epithelium ; Female ; Humans ; Magnetic Resonance Imaging ; Magnetic Resonance Spectroscopy ; Mixed Tumor, Mullerian ; Uterus ; Vagina

Toremifene is an anti-estrogen which has been shown to be effective in the treatment of breast cancer, and is thought to be a less uterotrophic agent than tamoxifen. The risk assessment concerning endometrial cancer has been inconclusive because of its rare use up to the mid-1990s. We report a case of an adenosarcoma, which is a very rare type of uterine malignancy, after toremifene treatment for 5 years in a breast cancer patient. After 1 year of toremifene use, the patient had a benign Mullerian adenofibroma. After an additional 4 years of toremifene treatment, the endometrial polypoid lesion was transformed into a Mullerian adenosarcoma. Although toremifene is a promising anti-estrogenic agent in the treatment of breast cancer patients, clinicians should not neglect the possibility of a uterine malignancy.
Adenofibroma ; Adenosarcoma ; Breast ; Breast Neoplasms ; Endometrial Neoplasms ; Female ; Humans ; Risk Assessment ; Tamoxifen ; Toremifene

Adenofibroma ; metabolism ; pathology ; Antigens, CD34 ; metabolism ; Child, Preschool ; Diagnosis, Differential ; Female ; Humans ; Kidney Neoplasms ; metabolism ; pathology ; surgery ; Neoplasms, Germ Cell and Embryonal ; metabolism ; pathology ; surgery ; Nephroma, Mesoblastic ; metabolism ; pathology ; Sarcoma, Clear Cell ; metabolism ; pathology ; Stromal Cells ; metabolism ; pathology ; Vimentin ; metabolism

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