Cases reported "Vulvar Neoplasms"

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1/23. Human papillomavirus types 16 and 39 in a vulval carcinoma occurring in a woman with Hailey-Hailey disease.

    A woman with Hailey-Hailey disease, suffering from carcinoma of the vulva, was examined by histology and for the presence of human papillomavirus (HPV) DNA by polymerase chain reaction (PCR) and in situ hybridization. Our diagnosis by histological examination revealed the vulval carcinoma to be a squamous cell carcinoma (SCC), adjacent to lesions of Hailey-Hailey disease and severe dysplasia/carcinoma in situ [vulval intraepithelial neoplasia (VIN) III]. The PCR with consensus primers for the L1 region (L1-PCR) successfully amplified HPV DNA using total DNA extracted from formalin-fixed and paraffin-embedded tissue specimens. Restriction fragment length polymorphism analysis and sequencing of L1-PCR products revealed HPV types 16 and 39. HPV 16-specific primers for the E6 region identified HPV 16 DNA. in situ hybridization analysis with biotinylated HPV 16 and 39 dna probes revealed the presence of the HPV 39 genome in the nuclei of the tumour cells in the SCC. These results indicate that HPV 16 and 39 are associated with lesions in vulval carcinoma. Regarding the patient's susceptibility to infection in the case of Hailey-Hailey disease, there is a possibility that HPV was inoculated into the lesions of Hailey-Hailey disease and induced those of VIN III and SCC.
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2/23. Cervical adenoid cystic carcinoma coexisting with multiple human papillomavirus-associated genital lesions. A common etiology?

    Adenoid cystic carcinoma of the uterine cervix is a rare tumor with unknown etiology. We report a case of adenoid cystic carcinoma occurring in a young woman, associated with multiple human papillomavirus (HPV)-related lesions including condyloma acuminata, vulvar intraepithelial neoplasm, cervical intraepithelial neoplasm and invasive basaloid squamous cell carcinoma. While adenoid cystic carcinoma has previously been found to coexist with squamous cell carcinoma or cervical intraepithelial neoplasia, its association with such a variety of HPV-related lesions in our case has not been previously reported, and raises the speculation that HPV may also be the causative factor for adenoid cystic carcinoma. However, in situ DNA hybridization and polymerase chain reaction in our current study failed to demonstrate the existence of HPV DNA in adenoid cystic carcinoma.
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3/23. Vulvar carcinoma in young patients and its relationship with genital warts.

    We report the occurrence of aggressive vulvar carcinoma associated with condyloma acuminata in three patients under 33 years old. Discussion of the role of the human papilloma virus (HPV) in the development of vulvar cancer is also presented. Three patients with condyloma associated with aggressive vulvar squamous cell carcinoma, in situ (1 case) and invasive (2 cases), documented by biopsy and/or vulvectomy are presented. in situ hybridization (ISH) was used to characterize the subtypes of HPV. One patient with erythematous systemic lupus developed in situ carcinoma after 5 years. The other two cases also developed aggressive multicentric, invasive squamous cell carcinoma after 10 years of diagnosis of condyloma. In all cases HPV cytological abnormalities were seen throughout the pathological examination. HPV 16 and 18 were present in cells of invasive squamous cell carcinoma in cases 2 and 3. HPV 6 and 11 were detected only in the condyloma area in case 2. HPV 30 was seen only in the condyloma area in case 3. This report emphasizes the need for biopsies of all unusually persistent or treatment-resistant condylomas, particularly in young and/or immunosuppressed patients.
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4/23. Sebaceous carcinoma of the vulva.

    Extraocular sebaceous carcinoma is an uncommon neoplasm usually localized on the head and neck. sebaceous glands are abundant on the vulva, but vulvar sebaceous carcinoma is an uncommon neoplasm. To our knowledge, there are only five previously reported cases of sebaceous carcinoma on this location. We report an additional case of vulvar sebaceous carcinoma associated with bowen's disease in the overlying epidermis. The patient also had bowenoid papulosis involving the skin of labia majora. We analyzed by immunohistochemistry, Southern blot hybridization, and polymerase chain reaction (PCR) techniques for the presence of DNA of human papilloma viruses (HPVs) in the specimen of sebaceous carcinoma and in lesions of bowenoid papulosis. immunohistochemistry, Southern blot hybridization, and PCR studies in specimens of bowenoid papulosis lesions and sebaceous carcinoma did not detect DNA of HPVs. A significant increase in intranuclear p53 staining was demonstrated in several areas of neoplastic aggregations of sebaceous carcinoma.
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5/23. Chromosomal translocation t(8;12) induces aberrant HMGIC expression in aggressive angiomyxoma of the vulva.

    Benign mesenchymal neoplasms associated with rearrangements of the DNA architectural factor gene HMGIC on chromosome 12 include lipomas, uterine leiomyomata, pulmonary chondroid hamartomas, endometrial polyps, salivary gland pleomorphic adenomas, and breast fibroadenomas. Although HMGIC also has been implicated in the pathobiology of aggressive angiomyxoma of the vulva, the molecular mechanisms pertaining to this neoplasm are unclear. Tissue from a recurrent aggressive angiomyxoma was investigated by cytogenetic and expression analysis for HMGIC and HMGIY. The trypsin-Giemsa-banded karyotype showed a clonal translocation between chromosomes 8 and 12 [46,XX,t(8;12)(p12;q15)]. fluorescence in situ hybridization (FISH) analysis with whole chromosome paint probes for chromosomes 8 and 12 excluded cryptic involvement of other chromosomes. The chromosome 12 breakpoint was mapped with two-color FISH analysis using cosmid probes at the 5' and 3' termini of HMGIC. Both cosmid probes showed hybridization to the normal chromosome 12 and the der(12) chromosome, indicating that the breakpoint was 3' (telomeric) to the gene. Reverse transcriptase-polymerase chain reaction (RT-PCR) analysis revealed HMGIC expression in the tumor, and immunohistochemistry localized HMGIC expression to the tumor's spindle cells. Like numerous benign mesenchymal tumors, this locally aggressive tumor is associated with rearrangements near or within HMGIC, but chimeric gene formation was not required for tumorigenesis. Inappropriate expression of this DNA binding protein, however, may be important in the pathobiology of this tumor. Understanding the pathogenetic mechanism may also be helpful in developing new diagnostic tools for identifying residual disease.
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6/23. Multiple HPV 16-related squamous cell carcinomas of the vulva, vagina, anus, skin and cervix in a 31-year-old woman.

    A 31-year-old woman is reported with in the genital region multiple squamous carcinomas of the skin (buttock), vulva, vagina, anus and cervix uteri. All these carcinomas were HPV 16 positive as tested by DNA in situ hybridization. The existence of areas with normal epithelium between all tumor localisations and the absence of distant metastases indicate multicentric development of these multiple carcinomas. The presence of HPV 16 DNA in all carcinoma cells, as detected by DNA in situ hybridisation, argues for an etiological role of HPV 16 in the development of these multiple tumors.
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7/23. Detection of human papillomavirus type 58 in polydactylous bowen's disease on the fingers and toes of a woman - concurrent occurrence of invasive vulval and cervical carcinomas.

    The group related to human papillomavirus (HPV) type 16 (HPV-16, -31, -33, -35, -52, -58 and -67) is dominantly identified in cervical intraepithelial neoplasia and cervical carcinomas. HPV-16 has also been frequently detected in bowen's disease on the hands and feet. We describe herein a case of polydactylous bowen's disease on the fingers and toes of a woman who had had radical vulvectomy and hysterectomy for concomitant invasive vulval and cervical carcinomas. All the lesions, except for the lesions on the periungual side of her left index, middle and ring fingers, harbored HPV-58 DNA with more than 100 entire viral genome copies per cell detected by Southern blot hybridization. The histological localization of the viral DNA was confirmed in all the lesions by in situ hybridization. We could also retrospectively demonstrate HPV-58 DNA in her invasive vulval and cervical carcinoma tissues.
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8/23. Multinucleated atypia of the vulva.

    Multinucleated atypia of the vulva (MAV) is an entity with a distinctive histologic pattern of multinucleation in the basal and middle layers of the squamous epithelium that may mimic human papillomavirus (HPV)-related squamous atypias. MAV is rarely reported in the literature, and we believe it should be considered in the differential diagnosis of flesh-colored vulvar papules and vulvar epidermal atypias with multinucleated squamous cells. We describe the case of a 49-year-old patient with the diagnosis of MAV. Results of histopathologic examination revealed a focal area of multinucleation in the basal to middle epithelial layers of the vulvar squamous epithelium, accompanied by mild hyperkeratosis and chronic inflammation. HPV was not identified in the lesion by in situ hybridization techniques.
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9/23. Metastasizing vulvar carcinosarcoma with squamous carcinomatous and leiomyosarcomatous differentiation: genetic evidence of clonal origin.

    In this report, we present the first case of a vulvar carcinosarcoma with squamous carcinomatous and leiomyosarcomatous differentiation. comparative genomic hybridization was used to analyze clonality of the two tumor components. A widely identical pattern of genetic imbalances in the comparative genomic hybridization analysis in both the carcinomatous and the sarcomatous tumor component strongly supported the concept of a bidirectionally differentiated neoplasm. In both tumor components and two lymph node metastases, an amplicon was detected on chromosome 11q12-q13, homing the cyclin d1 gene locus. In contrast, exclusively in the sarcomatoid component, a characteristic amplicon on 12q13-q14 was found. The cytogenetic profile of the lymph node metastases revealed an increase in imbalances compared with the primary tumor. In summary, we found strong indications for a clonal origin of the two tumor components in a vulvar carcinosarcoma and a good correlation of the histological morphology with the pattern of genetic imbalances.
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10/23. Squamous cell carcinoma arising in a case of vulvitis granulomatosa or vulval variant of melkersson-rosenthal syndrome.

    A case of vulvitis granulomatosa or the vulval variant of melkersson-rosenthal syndrome (MRS) in a young adult patient with systemic lupus erythematosus is presented. She also had evidence of cervicovulvovaginal human papilloma virus (HPV) infection. Nine years later she developed a squamous cell carcinoma (SCC) of the vulva. HPV 6/11 was found by DNA in situ hybridization within a vulval condyloma as well as within the carcinoma. This case is unusual in that SCC associated with HPV 6/11 developed in her vulva affected by MRS rather than in the cervix, where SCC was more likely to occur considering this patient's age.
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