1/7. Mucinous adenocarcinoma of Bartholin gland treated with radiation therapy: a case report.adenocarcinoma of Bartholin gland is a very rare disease and its molecular pathological features are poorly delineated. A 92-year-old woman with mucinous adenocarcinoma of Bartholin gland with metastasis to the right inguinal lymph node was treated with radiation therapy alone. Despite intensive radiation therapy, the tumor was locally recurrent and the patient died 10 months after radiation therapy. We searched for the presence of human papillomavirus 16 and 18 dna and the expression of p53 protein, CA19-9, CEA and MIB-1 antigen. Immunohistochemical study showed that mucinous adenocarcinoma of Bartholin gland produced CA19-9 and CEA.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/7. Peripheral primitive neuroectodermal tumor of the vulva: report of a case with imprint cytology.BACKGROUND: Peripheral primitive neuroectodermal tumor (PNET) of the vulva is an extremely rare disease, and, to our knowledge, only two cases have been previously reported. CASE: A 45-year-old woman presented with a mass in the right labium major. Three years after removal of the tumor, she noticed a new lesion in the same place and underwent a partial vulvectomy. The imprint cytology of the recurrent tumor showed a monomorphic appearance, composed of small round cells with scant cytoplasm against a hemorrhagic background. These tumor cells were loosely connective, but rosettelike structures were observed focally. On pathologic examination, the neoplasm was composed of small round tumor cells showing sinusoidal, diffuse or micropapillary growth. Immunohistochemically, the neoplastic cells stained positively for neuron-specific enolase, vimentin and HBA 71 and negatively for cytokeratin, HBA 45 and muscle-specific actin. The morphologic characteristics of the disease were well expressed in the imprint cytology, and this influenced the selection of immunohistochemical studies. CONCLUSION: Cytologic examination for vulvar tumors, even imprint cytology, can be a useful tool in obtaining an accurate pathologic diagnosis of a rare disease, such as peripheral PNET.- - - - - - - - - - ranking = 2keywords = rare disease (Clic here for more details about this article) |
3/7. Malignant melanoma associated with lichen sclerosus in the vulva of a 10-year-old.Malignant melanoma of the vulva in childhood is a rare neoplasm. Lichen sclerosus of the vulva in childhood is also a rare disease. The association of these two rare lesions in the vulva of young girls is extremely rare. We present a 10-year-old white girl with malignant melanoma associated with lichen sclerosus of the vulva. She had dark pigmentation of both the labia minora and posterior fourchette. The inner labia majora and fourchette showed whitish, glistening areas of skin. Histologic examination found mostly an in situ lentiginous/mucosal melanoma with focal invasion to a depth of 0.44 mm in the left upper labium majus. All specimens showed evidence of lichen sclerosus. Partial vulvectomy was performed, and no metastases were detected at the time of treatment. The patient has been disease free for the 12 months after treatment. It is critical for physicians to realize that melanoma can occur in children, and although rare, can occur in the vulva. We feel that lichen sclerosus in this instance may represent a pattern of host immune response to melanoma.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
4/7. Endometrial carcinoma metastatic to the vulva: a case report and review of the literature.In this paper, we report a new case of metastatic endometrial carcinoma to the vulva and describe the clinical and pathological features. We reviewed the literature to determine the frequency and to evaluate the prognostic significance of this rare disease. Moreover, we discuss the criteria for the differential diagnosis of endometrial carcinoma metastatic to the vulva, the primary vulvar adenocarcinomas, and other metastatic adenocarcinomas. The patient, previously diagnosed to have endometrial adenocarcinoma with squamous differentiation at III C stage (according to the FIGO system) and T2N1M0 stage (according to the TNM system), presented with a small plaque on the vulvar mucosa 8 months after endometrial primary carcinoma had been diagnosed. The histological evaluation of excisional vulvar biopsy revealed a neoplasm with pathological features of endometrial carcinoma. Thus, the final diagnosis was metastatic endometrial carcinoma to the vulva. A total body computed tomography scan (CT) and an echotomography with contrast medium revealed a second metastatic lesion at the 8th segment of the liver. No other metastatic lesions developed, nor was a reduction in the size of liver metastasis observed after 3 months of hormonic treatment with progesterone. Fourteen months after the diagnosis of primary endometrial carcinoma, the patient died of disseminated metastatic lesions. In conclusion, metastatic endometrial carcinoma to the vulva, although rare, might develop and could appear within a few months after the diagnosis of primary tumor. Moreover, in the presence of metastatic endometrial carcinoma to the vulva, it is necessary to verify if other visceral metastases are present.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
5/7. Villoglandular adenocarcinoma in situ of intestinal type of the hymen: de novo origin from squamous mucosa?Adenocarcinomas of the lower genital tract are rare diseases, and most of them arise from the Bartholin glands. Villoglandular adenocarcinoma of intestinal type is a very uncommon neoplasm of unknown origin with only few cases described on the vulva and in the vagina. It is characterized by villoglandular architecture, mucinous-type epithelium with intestinal differentiation (goblet cells), and direct apposition of the tumor with the surface epithelium. We report a case that developed on the hymen of a 64-year-old woman and discuss its possible origin as arising de novo from the squamous epithelium.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
6/7. White sponge nevus of the vulva.The white sponge nevus is a hereditary leukokerotosis localized preferably in the oral mucosa, but may simultaneously manifest itself in other regions, e.g. perianally. We report the case of a 18-year-old patient with a primarily and exclusively extraorally located white sponge nevus of the vulva. The differential diagnosis and clinical as well as therapeutical problems of this extremely rare disease are discussed.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
7/7. Endodermal sinus tumour of the vulva: an interesting clinico-pathological problem.Endodermal sinus tumours (EST) of the lower female genital tract are uncommon malignancies. The majority of these involve the vagina and cervix, though there are a few case reports of tumours involving the vulva. These are usually either locally advanced or have metastatic disease present at initial diagnosis, and generally do badly on treatment. This case report discusses primary vulval involvement by EST. It shows that the absence of tumour markers can be misleading, and discusses the role of radiation and chemotherapy in the treatment of this rare disease.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |