A Constellation of Rare Gynecological Malignancies and Familial Adenomatous Polyposis Gastrointestinal Adenocarcinoma: A Case Report.

Ovarian microcystic stromal tumors (MST) are a rare subtype of sex-cord stromal tumors. We are presenting a case of a MST arising in a patient with familial adenomatous polyposis (FAP) and concurrent colonic adenocarcinoma. During the patient's workup of an ampullary adenoma associated with her FAP, she was found to have an enlarged uterus with a thickened endometrium and an incidental pelvic mass on the fundus of the uterus. Subsequent imaging identified heterogenous bulky ovaries. This patient underwent surgical resection including a total abdominal hysterectomy, bilateral salpingo-oophorectomy, omentectomy, bilateral pelvic sentinel lymph node biopsy during her planned total proctocolectomy and transduodenal ampullectomy. Extensive histologic and immunohistochemical investigations were completed and the final pathology report revealed a unique compilation of International Federation of Gynecology and Obstetrics Stage II, grade 1 endometrioid endometrial adenocarcinoma, bilateral ovarian MST, a sperate pedunculated mass favoring a diagnosis of uterine tumor resembling ovarian sex cord tumor (UTROSCT), 2 distinct adenocarcinomas of the colon (T2N0 and T1N0) and a tubular adenoma of the ampulla. The pathology showed the endometroid adenocarcinoma was ß-catenin negative while the MST and UTROSCT both showed nuclear positivity with ß-catenin. To our knowledge this is the first reported case of a UTROSCT with concurrent endometrial adenocarcinoma presenting with bilateral ovarian MST's and adenomatous polyposis coli gene positive FAP colon adenocarcinoma.

Canadian Consensus-based and Evidence-based Guidelines for Benign Endometrial Pathology Reporting in Biopsy Material.

Standardized terminology has proven benefits in cancer reporting; in contrast, reporting of benign diagnoses in endometrial biopsy currently lacks such standardization. Unification and update on the lexicon can provide the structure and consistency needed for optimal patient care and quality assurance purposes. The Special Interest Group in Gynecologic Pathology of the Canadian Association of Pathologists-Association Canadienne des Pathologistes (CAP-ACP) embarked in an initiative to address the current need for consensus terminology in benign endometrial biopsy pathology reporting. Nine members of the Special Interest Group developed a guideline for structured diagnosis of benign endometrial pathology through critical appraisal of the available peer-reviewed literature and joint discussions. The first version of the document was circulated for feedback to a group of professionals in akin fields, the CAP-ACP Executive Committee and the CAP-ACP general membership. The final 1-page document included 17 diagnostic terms comprising the most common benign endometrial entities, as well as explanatory notes for pathologists. The proposed terminology was implemented in the practice of 5 pathologists from the group, who applied the guideline to all benign endometrial biopsies over a 2-wk period. A total of 212 benign endometrial biopsies were evaluated in this implementation step; the recommended terminology adequately covered the diagnosis in 203 cases (95.8%). A list of terminology for benign endometrial biopsy reporting, based on expert consensus and critical appraisal of the available literature, is presented. On the basis of our results of implementation at multiple centers, the proposed guideline can successfully cover the large majority of diagnostic scenarios. The document has the potential to positively impact patient care, promote quality assurance, and facilitate research initiatives aimed at improving histopathologic assessment of benign endometrium.

Is adrenal ovarian thecal metaplasia a misnomer? Report of three cases of radial scar-like spindle cell myofibroblastic nodule of the adrenal gland.

Ovarian thecal metaplasia is a rare tumor-like mesenchymal lesion of the adrenal gland that has been reported mainly in postmenopausal women and rarely in men. It was originally described as a wedge-shaped lesion composed of ovarian-like stroma resembling theca that shows continuity with the capsule of the adrenal gland. We report three cases and identify that these lesions can resemble a radial scar. While a single case has been reported to contain true ovarian stromal elements, there was no evidence of steroidogenic differentiation in our cases. These findings justify the need for a better terminology since the entity falls within the spectrum of fibroblastic-myofibroblastic proliferations. We propose the terminology of "radial scar-like spindle cell myofibroblastic nodule of the adrenal gland" as a more accurate nomenclature to designate these lesions. Although rare, calcifying fibrous tumor of the adrenal gland, hypertrophic nerve bundles, and peripheral nerve sheath tumors may mimic these lesions.