1/273. Angiomyofibroblastoma and aggressive angiomyxoma: two benign mesenchymal neoplasms of the female genital tract. An immunohistochemical study.We describe a rare case of angiomyofibroblastoma (AMF) of the vulva and one case of aggressive angiomyxoma (AAM) of the pelvic region and, with the help of an extensive revision of the literature, we attempt to define their histogenesis and peculiar biological behaviour by an immunohistological evaluation. Our results indicate that AAM, which is characterized by the presence of a high content of glycosaminoglycans in the stroma, expresses uniformly vimentin and hyaluronate receptor CD44, and heterogeneously muscle specific actin (MSA) and desmin, while AMF displays a positive reaction for vimentin, desmin and laminin, and only a weak and heterogeneous positivity for CD44. Both AMF and AAM showed no immunohistochemical reactivity for alpha-smooth muscle actin (ASMA), myoglobin, cytokeratin, collagen type iv, CD68 and S-100. The stromal cells of AAM were negative for laminin. These findings support the suggestion of an origin of the two entities by a common myofibroblastic progenitor, which normally occurs in the lower female genital tract and subsequently undergoes a neoplastic transformation. The expression of CD44 by AAM, which has never been reported before, could be responsible for its more aggressive behaviour, because this receptor is able to mediate migration of neoplastic cells on a hyaluronate rich extracellular matrix. It is speculated that the neoplastic cell of the AAM and AMF of the vulva is a specific myofibroblast which probably arises from undifferentiated mesenchymal cells normally occurring in the lower female genital tract.- - - - - - - - - - ranking = 1keywords = neoplasm, muscle (Clic here for more details about this article) |
2/273. Primary breast carcinoma of the vulva: a case report and literature review.BACKGROUND: In 1872, Hartung was the first to describe the case of a fully formed mammary gland arising in the left labium majora of a 30-year-old woman. Since Hartung's initial report, 38 additional cases of ectopic vulvar breast tissue have been described. This case report describes the rare occurrence of primary mammary adenocarcinoma arising within the vulva. CASE: A 64-year-old G4P4 white female presented with a 4-year history of a 2 x 1 cm firm, indurated, raised lesion of the left lateral mons. A wide local excision with ipsilateral inguinofemoral lymphadenectomy was performed. Given histological findings characteristic of both invasive ductal carcinoma and invasive lobular carcinoma, in conjunction with the presence of estrogen and progesterone receptors within the tumor, a diagnosis of infiltrating adenocarcinoma arising within ectopic breast tissue was made. CONCLUSIONS: Thirty-nine reported cases of ectopic breast tissue arising within the vulva have been reported in the world literature. Though the diagnosis of primary breast carcinoma arising within the vulva is based primarily upon histologic pattern, estrogen and progesterone receptor positivity provide supporting evidence. Given the rarity of this condition, guidelines for therapy are unavailable; we therefore suggest looking to the current management of breast cancer in order to establish a sensible approach.- - - - - - - - - - ranking = 0.13935777473348keywords = cancer (Clic here for more details about this article) |
3/273. Epithelioid leiomyoma of the vulva.Smooth muscle tumors are uncommon lesions of the vulva and represent a variety of histologic types. When encountered, surgical treatment is guided by the malignant potential of the tumors. This article presents the case of a 45-year-old woman who underwent conservative excision of a 10-cm vulvar lesion consistent with benign epithelioid leiomyoma. This unusual case provides an opportunity to review the clinical and pathologic features of this uncommon variant of leiomyoma and to describe the recently suggested pathologic criteria for determining the malignant potential of smooth muscle tumors arising in the vulva. knowledge of these criteria can guide the clinician in selecting the appropriate management.- - - - - - - - - - ranking = 0.018343026879699keywords = muscle (Clic here for more details about this article) |
4/273. The 1396del A mutation and a missense mutation or a rare polymorphism of the WRN gene detected in a French Werner family with a severe phenotype and a case of an unusual vulvar cancer. Mutations in brief no. 136. Online.The Werner's syndrome (WS) is a rare recessive disease characterized by an early onset of geriatric disorders. The Werner's syndrome gene (WRN) recently cloned, encodes for an helicase and therefore plays a role in DNA metabolism and dna repair. Here, we report the study of a French family with two affected members and numerous cancers. Using the protein truncation test and sequencing, we identified a homozygous mutation in the WRN gene. This mutation generates a frame shift leading to a very short 391 amino acids truncated protein without the helicase motif. A particularly severe phenotype of the affected patient was associated with an unusual vulvar cancer traditionaly observed in elderly patients and therefore likely to be related to the Werner's syndrome. An additional substitution of G for A at nucleotidic position 1392 was also described. We suggest that a relation between genotype and phenotype could exist in the studied family.- - - - - - - - - - ranking = 0.8361466484009keywords = cancer (Clic here for more details about this article) |
5/273. 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.- - - - - - - - - - ranking = 0.49082848656015keywords = neoplasm (Clic here for more details about this article) |
6/273. Extramammary Paget's disease of the vulva: report of five cases and review of the literature.Five patients with vulvar extramammary Paget's disease (EMPD) are reported. They accounted for 4.4% of all vulvar malignancies diagnosed in the south of israel between 1961-1997. Mean age was 64.4 years and the predominant symptom was vulvar pruritus. Three patients had intra-epidermal lesions (managed by wide local excision or, at most, simple vulvectomy), one had a minimally invasive lesion (managed by simple vulvectomy) and one had EMPD with an underlying apocrine gland adenocarcinoma (managed by radical vulvectomy and bilateral groin dissection followed by pelvic radiotherapy). EMPD recurred in two patients: 1) local recurrence after simple hemivulvectomy for an intra-epidermal lesion was successfully treated by wide local excision; 2) widespread recurrence at distant skin sites after simple vulvectomy for a minimally invasive lesion was unsuccessfully treated with systemic chemotherapy. It is concluded that vulvar EMPD is an uncommon neoplasm that primarily affects postmenopausal women. Its histogenesis is uncertain and most commonly it is an intra-epidermal lesion. The high rate of recurrent disease remains a challenge for optimal management.- - - - - - - - - - ranking = 0.24541424328008keywords = neoplasm (Clic here for more details about this article) |
7/273. Malignant myoepithelioma of the vulva resembling a rhabdoid tumour.AIMS: We report an example of malignant myoepithelioma of the vulva, which has not been hitherto described. We discuss the differential diagnosis and briefly review the literature. methods AND RESULTS: The lesion was found in an 81-year-old woman as an indolent 40 mm tumour. The neoplastic cells showed a myoid, spindled, epithelioid and plasmacytoid phenotype. Hyalinization of extracellular material and myxoid changes were present. There was a partly solid and microcystic pattern and a tight cohesiveness of cells was lacking. The circumscribed multinodular tumour somewhat resembled an extrarenal rhabdoid tumour, having large tumour cells with prominent nucleoli and large amounts of acidophilic cytoplasm. Immunohistochemically, the tumour cells were immunoreactive for cytokeratin, vimentin, muscle-specific actin, alpha-smooth muscle actin, and S100 protein, but not for desmin, epithelial membrane antigen, factor viii-related antigen, CD34 and CD31. CONCLUSIONS: The histological and cytomorphological appearance of the tumour well as the immunohistochemical findings suggest the diagnosis of malignant myoepithelioma, possibly derived from minor vestibulary glands or ectopic breast tissue. Differential diagnoses are, in particular, extrarenal rhabdoid tumour and 'proximal type' epithelioid sarcoma. Differentiation is important, because the tumours show a different behaviour and prognosis.- - - - - - - - - - ranking = 0.018343026879699keywords = muscle (Clic here for more details about this article) |
8/273. A case of clitoral metastasis without skin involvement from rectal cancer.An 84-year-old woman visited a private dermatologist and gynecologist due to pain in the external genitals. However, no abnormality was found. She was referred to a surgeon in our hospital to clarify the etiology of the pain. Rectal cancer and liver metastatic tumor were detected, and the rectal cancer was resected. However, the pain increased after the operation and she was referred to our department. No macroscopic abnormalities of the external genitals were found. However, a vaginal examination could not be performed due to severe pain. By local examination under anesthesia, enlargement of the clitoris was detected. A simple clitoridectomy was performed. Histological examination revealed that the clitoral tumor was metastatic cancer originating from rectal cancer.- - - - - - - - - - ranking = 1.1148621978679keywords = cancer (Clic here for more details about this article) |
9/273. 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.- - - - - - - - - - ranking = 0.13935777473348keywords = cancer (Clic here for more details about this article) |
10/273. Malignant rhabdoid tumor of the vulva. Case report.The poor prognosis of malignant rhabdoid tumor is emphasized and histopathological criteria for distinction from epithelial sarcoma of the vulva are discussed. Immunohistochemical analyses were performed by using nine different antigens including vimentin, cytokeratin, epithelial membrane antigen, carcinoembryonic antigen, desmin, muscle-specific actin, S-100 protein, AP-15, neuron specific enolase. This is the sixth reported case of a malignant rhabdoid tumor of the vulva. The patient died eight months after the initial diagnosis in spite of a combination of surgery, adjuvant chemotherapy and external radiotherapy.- - - - - - - - - - ranking = 0.0091715134398493keywords = muscle (Clic here for more details about this article) |
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