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Aims To report a previously undescribed phenomenon of incidentally detected microscopic proliferations of sex cord cells, often mimicking adult granulosa cell tumour or sex cord tumour with annular tubules, in extraovarian locations. Methods and results The six cases were in patients aged 23–58 years. The proliferations were located in the fallopian tube in three cases, and in paraovarian connective tissues, the pelvic side wall, and appendiceal serosa (one case each). Microscopically, they were typically composed of well-demarcated nests of regular cells with round/ovoid vesicular nuclei, some containing grooves. Microfollicular and/or cribriform arrangements were present in three cases. In five cases, the sex cord lineage was confirmed by positive staining with inhibin and/or calretinin and other sex cord markers. FOXL2 mutation analysis was performed in one case, but was inconclusive. Bilateral oophorectomies and bilateral cystectomies were performed in three cases and one case respectively; there was no sex cord–stromal neoplasm in the removed ovaries. In the two cases in which the ovaries were not removed, imaging showed no suspicious features. Follow-up in four cases (11 months–6 years) has been uneventful. Conclusions The pathogenesis of these microscopic extraovarian sex cord proliferations is unknown, but they may represent non-neoplastic proliferations of embryonic remnants.

We report 2 cases of classic vulval intraepithelial neoplasia (VIN) with collections of cells throughout the full epithelial thickness and within epithelial downgrowths containing intracytoplasmic mucin. The mucinous cells were bland with eccentrically placed small regular nuclei and were positive with CAM5.2, carcinoembryonic antigen, epithelial membrane antigen, and cytokeratin 7, markers that are typically positive in the neoplastic cells of Paget disease. Both cases were strongly positive with p16 and linear array genotyping demonstrated the presence of high-risk human papillomavirus type 16. As far as we are aware, mucinous differentiation has not been described in VIN, although mucinous metaplasia has rarely been reported in non-neoplastic vulval skin and in cutaneous squamous cell carcinoma in situ at extravulval sites. The main differential diagnosis of the cases we describe is Paget disease or coexistent VIN and Paget disease and the immunophenotype of the mucinous cells may further highlight the potential for misdiagnosis. Other possible differential diagnoses include Pagetoid squamous cell carcinoma in situ (Pagetoid VIN), basal cell carcinoma with sebaceous differentiation, and VIN involving skin appendage structures. The mucinous cells are likely to be metaplastic and a result of aberrant differentiation in a premalignant squamous lesion.

We report 3 cases of a distinctive uterine leiomyoma containing numerous thick-walled vascular channels, which we term angioleiomyoma. The leiomyomas occurred in women aged 33, 44, and 49 and ranged from 1.5 to 6.0 cm. They were composed of bland, spindle-shaped cells with numerous evenly distributed, arteriole-like vessels with thick muscular walls throughout. The spindle cells often swirled around the vessels. In 1 case, fibrin was present within vessel walls. The spindle cells were positive with smooth-muscle markers and negative with HMB45. We believe angioleiomyoma should be recognized as a distinct entity and included in the World Health Organization classification of tumors of the female genital tract as a benign leiomyoma variant, similar to cellular and atypical leiomyoma. Uterine angioleiomyoma has a close morphological resemblance to the similarly named skin tumor. We review uterine smooth-muscle and other mesenchymal neoplasms in which prominent vascular channels are a feature.

This report describes an ovarian serous cystadenofibroma, in a postmenopausal woman, which exhibited extensive sex cord differentiation, in the form of solid and hollow tubules resembling Sertoli cell elements, within the stroma. The sex cord elements, which were located just beneath the epithelium, were positive with both alpha inhibin and calretinin and negative with epithelial membrane antigen. They were also CD56 positive but negative with other neuroendocrine markers. True sex cord structures or sex cord-like elements have been described in ovarian adenosarcomas and pure stromal tumors, especially fibromas. However, as far as we are aware, these elements have not been reported in a serous cystadenofibroma. The endometrium exhibited simple hyperplasia, perhaps secondary to estrogenic activity of the sex cord elements. We discuss the significance of CD56 positivity of the sex cord elements.