Intestinal type adenocarcinoma of the endometrium with signet ring cells, a rare aggressive variant.

Objective: Intestinal type mucinous adenocarcinoma (iMACE) is a rare and unusual variant of mucinous carcinoma of the endometrium that can show focal features of poorly differentiated adenocarcinomas of gastric, pancreatic or intestinal origin by producing signet ring cells. To date, only two reported cases of signet ring cells as a morphological feature of iMACE have been reported. Alterations in E-cadherin expression have been linked to increased metastatic potential, tumor dedifferentiation, and deep myometrial invasion in endometrial carcinomas. The presence or absence of E-cadherin in iMACE with signet-ring cells has not been studied. Thus, we sought to analyze E-cadherin expression in this aggressive variant of endometrial carcinoma. Cases: Diagnosis of iMACE with signet ring cells was rendered with the aid of immunohistochemical staining and histological analysis. Average age of diagnosis was 72 with the presenting complaint of postmenopausal bleeding in all three women. Focal loss or weakly positive E-cadherin expression was seen in areas of signet-rings cell morphology in all three cases. Conclusion: This case report adds to the body of literature that demonstrates the aggressive nature of intestinal differentiation in the endometrium. In addition, this report suggests that the presence of signet-rings cells and loss of E-cadherin expression may render a poorer prognosis as seen in other parts of the body.

Clinical Implications of Genomic Loss of Heterozygosity in Endometrial Carcinoma.

PURPOSE: Homologous recombination deficiency, identified by homologous recombination deficiency gene alterations or high percentage of genome-wide loss of heterozygosity (gLOH), is associated with improved prognosis, platinum sensitivity (PS), and poly (ADP-ribose) polymerase inhibitor response in high-grade ovarian cancer. Since the copy number-high (CN-H) endometrial cancer molecular subtype (EC-MS) shares molecular features with high-grade ovarian cancer, our aim was to assign EC-MS on the basis of comprehensive genomic profiling (CGP) results and evaluate the gLOH status with clinical behavior of EC. METHODS: Eighty-two epithelial EC tumor tissues were sequenced by hybrid capture-based CGP, and results were used to assign EC-MS (ultramutated, microsatellite instability-high, CN-low; CN-high). Retrospective chart review established clinical characteristics, including PS. Relationships of PS, EC-MS, gene alterations, and gLOH were assessed statistically. RESULTS: PS and EC-MS of CN-H showed statistically significant difference in overall survival (OS). Most notably, when the CN-H EC-MS was subcategorized by gLOH status, there was a significant difference in OS with gLOH-H being associated with longer survival. Cox semi-proportional hazard modeling showed that gLOH, stage, and race were significant in modeling OS. CONCLUSION: The method of assigning EC-MS by CGP demonstrates similar clinical features to previous reports of EC-MS assigned by other methods. CGP can also assess gLOH status with gLOH-H most commonly seen in CN-H tumors. CN-H, gLOH-H patients showed significantly improved OS (hazard ratio, 0.100 [0.02-0.51 95% CI]). Thus, gLOH status may be a meaningful prognostic biomarker within the CN-H tumors and possibly across EC-MS.

Yolk sac tumor of the endometrium in a post-menopausal woman: Case report and review of the literature.

•Seven of eighteen postmenopausal female endometrial YST cases were pure YST.•IHC supports somatic tumor cell retro-differentiation yielding extra-gonadal YST.•Studying genetic alterations in endometrial YST may elucidate its histiogenesis.

Malignant Female Adnexal Tumor of Probable Wolffian Origin (FATWO): A case report and review for the literature.

•Of the fewer than 100 FATWO case reports published, at least 25 reports have metastatic quality.•Very little information regarding optimal management of FATWO is known; immunohistochemical stains may help guide therapy.•FATWO may be more of a low malignant potential entity, and patients with the diagnosis should be followed closely.

Mesonephric-like adenocarcinoma of the ovary with co-existent endometriosis: A case report and review of the literature.

INTRODUCTION: Mesonephric-like adenocarcinoma (MLA) is a rare malignant gynecologic neoplasm occurring in the uterine corpus and ovary. The morphological and immunohistochemical characteristics of MLA closely resemble that of cervical mesonephric adenocarcinomas, but whether they share a common histogenesis remains unclear. Two main theories for histogenesis of MLAs include the origination of these neoplasms from mesonephric remnants, as is the case for cervical mesonephric adenocarcinoma, versus the differentiation along a mesonephric pathway from Mullerian lesions. CASE: A 67-year-old presented after a right salpingo-oophorectomy for a complex ovarian mass revealed a mesonephric-like adenocarcinoma of the ovary and endometriosis. She underwent a total abdominal hysterectomy, pelvic lymphadenectomy, and infra-colic omentectomy, and diagnosed with Stage IA mesonephric-like adenocarcinoma of the ovary. At 18 months post-operatively, the patient developed flank and abdominal pain and was found to have multiple sites of recurrent disease. She was referred to medical oncology for chemotherapy as she was not a candidate for surgical cytoreduction. DISCUSSION: This case demonstrates the aggressive nature of ovarian MLA and the need for a multidisciplinary approach when determining the treatment. In addition, this case provides further evidence to support the theory that at least a subset of MLAs arises from a Mullerian lesion which then differentiates down a mesonephric pathway.

A case of non-HPV related primary endometrioid adenocarcinoma of the cervix.

OBJECTIVE: Primary endometrioid adenocarcinoma of the cervix is a rare subtype of adenocarcinoma that has often been misclassified in the literature due to the lack of clear-cut diagnostic criteria. A new classification system has recently been developed that aims to provide clarity and reproducibility when diagnosing subtypes of endocervical adenocarcinoma. This case report demonstrates the difficulty in diagnosing primary endometroid adenocarcinoma, application of the new diagnostic guidelines, and a review of the literature of this rare non-HPV subtype. CASE: A 76 year-old women presented with postmenopausal bleeding and was found to have an exophytic cervical mass. Biopsies showed an adenocarcinoma of probable endometrial origin. She underwent a robotic-assisted simple hysterectomy with bilateral pelvic lymph node sampling and omental biopsy. Final pathology report demonstrated a primary endometrioid adenocarcinoma of the cervix, measuring 2.4 cm in size, diagnosed using the recently developed International Endocervical Adenocarcinoma Criteria and Classification (IECC) system. Patient was then treated with external beam radiation therapy and concurrent chemotherapy, followed by vaginal brachytherapy. She had no evidence of disease at her 15-month follow-up visit. CONCLUSION: Primary endometrioid adenocarcinoma of the cervix is a rare and diagnostically challenging tumor of the cervix. This case illustrates the challenges associated with diagnosis of this endocervical carcinoma subtype and the need for a multi-disciplinary approach when determining treatment.

Overall Survival and Adjuvant Therapy in Women with Ovarian Carcinosarcoma: A Single-Institution Experience.

BACKGROUND: Carcinosarcoma of the ovary (CSO) is a rare and aggressive variant of ovarian cancer. Due to the rare nature of the disease there is insufficient evidence to make recommendations regarding standard management and overall prognosis. METHODS: An Institutional Review Board-approved study identified all our patients with CSO between January 2011 and May 2018. Demographic and outcome measures were abstracted from the medical records and tumor board files. Cox proportional hazard models, log rank tests, and comparisons of means were used to calculate significance (p < 0.05). RESULTS: 27 women with CSO were identified. The median age at diagnosis was 65 years (range 48-91). Five women (18%) presented with early stage disease (Stage I or II) and 22 patients (82%) presented with late stage III or IV disease. Twenty patients (74%) received intravenous platinum-based combination chemotherapy. Seven patients did not receive chemotherapy during their treatment course. The median overall survival was 23 months (range 2-68 months). Overall survival was not significantly worsened by the stage of disease at diagnosis. There was no difference in survival based on the age at diagnosis, tobacco status or ethnicity (p > 0.05). CONCLUSION: This is one of the largest single institution experiences with CSO. The majority of our patients presented with advanced stage disease and received adjuvant platinum-based chemotherapy after cytoreductive surgery. The median overall survival of 23 months was not affected by the stage of the disease. The optimal management of this rare disease needs further study with collaborative, prospective multi-institutional trials.