Low-grade Epithelial-Myoepithelial Carcinoma of Bartholin Gland: Report of 2 Cases of a Distinctive Neoplasm Arising in the Vulvovaginal Region
Department of Pathology, Royal Group of Hospitals Trust, Belfast, UK.International journal of gynecological pathology: official journal of the International Society of Gynecological Pathologists (Impact Factor: 1.67). 06/2009; 28(3):286-91. DOI: 10.1097/PGP.0b013e31818e1040
We report 2 cases of a distinctive neoplasm arising from Bartholin gland and presenting as a vulval or vaginal mass. The tumors occurred in patients aged 44 and 51 years and were 2 and 3 cm in maximum dimension. In both cases, normal Bartholin gland tissue was identified adjacent to the lesion. The neoplasms were unencapsulated and largely well circumscribed but with a focally infiltrative edge. They were composed of tubular, trabecular, or insular arrangements with a double layer of inner cuboidal cells with round nuclei and outer cells with ovoid nuclei and clear cytoplasm, corresponding to epithelial and myoepithelial cells, respectively. Luminal eosinophilic colloid-like material was present. In both cases, a minor proportion of the neoplasm consisted of cribriform arrangements, creating an appearance reminiscent of adenoid cystic carcinoma, although the overall morphology was not typical of that lesion. Mitotic figures were identified in both cases, the mitotic count being 1 and 5/10 high-power fields. Immunohistochemically, the inner cells were positive with epithelial markers, including broad-spectrum cytokeratins and epithelial membrane antigen, and the outer cell layer was positive with myoepithelial markers p63, calponin, and alpha-smooth muscle actin. Both neoplasms exhibited diffuse strong immunoreactivity of the epithelial cells with c-kit. Activating mutations in KIT exons 9, 11, 13, and 17 and in platelet-derived growth factor receptor alpha exons 12, 14, and 18 were searched for by polymerase chain reaction and direct sequencing but were not identified. We believe this represents a low-grade carcinoma arising from Bartholin gland composed of a dual population of epithelial and myoepithelial cells and closely resembling the salivary gland neoplasm termed epithelial-myoepithelial carcinoma. We propose the term low-grade epithelial-myoepithelial carcinoma of Bartholin gland.
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ABSTRACT: We report 4 myoepitheial neoplasms involving the vulva (2 cases) or vagina (2 cases) in patients aged 40 to 45. Two tumors were composed entirely of ovoid or spindle-shaped cells, one entirely of epithelioid cells, and in the other, there was a mixture of spindled and epithelioid cells. Small foci of ductal differentiation with squamous metaplasia were present in one case and a minor stromal component, which varied from myxoid to hyalinized, in all cases. In all cases, the tumor cells were positive for epithelial markers (cytokeratins and/or epithelial membrane antigen) and the myoid markers alpha smooth muscle actin and calponin. Desmin was positive in 3 cases. S100 and p63 were positive in 1 of the 4 neoplasms. On the basis of the nuclear features and degree of mitotic activity, 2 neoplasms were classified as benign myoepitheliomas and 2 as myoepithelial carcinomas. Judging by the paucity of cases in the literature, myoepithelial neoplasms appear extremely rare in the vulvovaginal region with only 3 previous case reports of primary vulval tumors. As far as we are aware, this is the first description of a primary vaginal myoepithelial neoplasm. At these sites, myoepithelial tumors are liable to be misdiagnosed as a variety of other neoplasms because the pathologist may not think of the diagnosis. In reporting these cases, we discuss the criteria for diagnosis and the differential diagnosis.
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ABSTRACT: Myoepithelial tumors of the vulva are extremely rare, with only 8 cases reported in the literature to date. We report the first case of a high-grade myoepithelial vulvar carcinoma diagnosed in a 35-year-old woman during the 27th week of her pregnancy. The patient initially underwent a wide local excision of the lesion but noted rapid regrowth of the vulvar mass during the next 2 months before her delivery. Shortly thereafter, she underwent a classic radical Taussig-Basset total radical vulvectomy, bilateral superficial and deep inguinal groin node dissection, partial vaginectomy, and reconstruction of the vulva. However, the patient rapidly developed both locoregional and distant mestatatic disease, despite aggressive chemoradiotherapy, and she eventually succumbed to disseminated disease almost 20 months after her initial diagnosis.
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