Cases reported "Vulvar Neoplasms"

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1/14. Atypical solitary fibrous tumor of the vulva.

    An atypical solitary fibrous tumor (SFT) was encountered as a slow-growing, 15-cm, well-demarcated, vulvar tumor in a 70-year-old woman. The tumor was highly cellular and composed predominantly of hemangiopericytomatous and capillary hemangioma-like proliferations and short fascicular arrangements of spindled cells. Multinucleated giant cells and tumor necrosis also were present. The tumor cells were positive for vimentin, CD34, progesterone receptors, and bcl-2 and were diploid by flow cytometry. The patient was well without disease 9 months after surgery. awareness of the occurrence of atypical SFT in the vulva is important so that confusion with other neoplasms can be avoided.
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2/14. A case of dermatofibrosarcoma protuberans of the vulva with a COL1A1/PDGFB fusion identical to a case of giant cell fibroblastoma.

    dermatofibrosarcoma protuberans (DFSP) is a highly recurrent low-grade soft tissue sarcoma, which is usually located on the trunk. Presentation in the vulva is rare, with only 13 cases being reported to date, none of which have been investigated at the cytogenetic or molecular level. Specific cytogenetic abnormalities, involving chromosomes 17 and 22, are characteristic features of DFSP and giant cell fibroblastoma (GCF), a tumor closely related to DFSP. These chromosomal rearrangements result in the fusion of the COL1A1 and PDGFB genes in both lesions and show wide variation in the position of the fusion point in COL1A1. Here, we describe a case of DFSP of the vulva with a typical monotonous storiform pattern, with no foci of multinucleated giant cells. cytogenetic analysis showed a 47,XX, r karyotype in 50% of the cells, and molecular investigation disclosed the presence of a transcript fusing COL1A1 exon 37 to PDGFB exon 2. This is the first case of DFSP showing such a fusion point, which is intriguingly identical to that found in a GCF case, indicating that the COL1A1/PDGFB fusion point position does not seem to affect tumor morphology. This finding further underlines the very close relationship between these two morphologically distinct entities.
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3/14. Intradermal spindle cell/pleomorphic lipoma of the vulva: case report and review of the literature.

    BACKGROUND: Spindle cell/pleomorphic lipoma (SC/PL) is a benign adipose tissue tumor that usually affects the subcutaneous tissues of shoulders, backs, and neck region of middle-aged male patients. Histologically, it is characterized by the presence of primitive CD34-positive spindle cells arranged in short fascicles, bizarre floret-like multinucleated giant cells, mature adipocytes, and a small number of lipoblasts. Recently, an intradermal subset has been described, which mainly affects female patients and presents a wider antomical distribution when compared to the classical variant of SC/PL. methods: We report a case of intradermal SC/PL affecting the labium majus of a 56-year-old female patient. RESULTS: The histological examination disclosed the typical histological features, however the lesion showed poorly demarcated and infiltrative borders, as well as involvement of dermal nerves. The immunohistochemical analysis according to streptovidin-biotin-peroxidase technique showed immunoreactivity for CD34 and vimentin in the spindle cells, as well as S100 protein and vimentin in the adipocytic cells. CONCLUSIONS: To the best of our knowledge, this is the first case of intradermal SC/PL affecting the vulvar region. Care must be taken not to misdiagnosis this rare tumor as well-differentiated liposarcoma, cellular angiofibroma, solitary fibrous tumor, and cutaneous neurofibroma.
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4/14. Recurrent giant fibroepithelial stromal polyp of the vulva associated with congenital lymphedema.

    BACKGROUND: The relatively site-specific mesenchymal lesions of the vulvovaginal region can exhibit superficially overlapping histological features and can be diagnostically challenging. Fibroepithelial stromal polyp is generally an easily recognizable entity, but certain cases cause differential diagnostic problems. CASE: We present a case of a 16-year-old girl with a 10-cm polypoid lesion localized to the left labium. The patient has therapy resistant congenital lymphedema localized to the left arm and leg. The labial lesion was resected and recurred after 12 months and 6 years following the initial treatment. Histologically, it exhibited characteristics of a fibroepithelial stromal polyp with scattered bizarre multinucleated giant cells and ectatic tortuous lymphatic spaces. CONCLUSION: This vulvar lesion represents a unique example of giant fibroepithelial stromal polyp developed in association with Nonne-Milroy-Meiges syndrome.
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5/14. carcinoma of the vulva with sarcomatoid features: a case report with immunohistochemical study.

    A tumor of the vulva with sarcomatoid features was studied by immunocytochemistry to characterize the phenotype of the spindle-shaped and giant cells. Sarcomatoid-looking cells were positive for intermediate filament keratin polypeptides of stratified epithelium. These results favor histogenesis of the sarcomatoid-looking cells from a metaplastic alteration of the malignant squamous component.
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6/14. Proliferating giant pigmented nevus: a report of an unusual tumor occurring in association with a congenital giant pigmented nevus.

    A 32-year-old Japanese woman with a giant pigmented congenital nevus of the torso presented with a massive pigmented tumor mass of the vulva which grew over an 8-year period. Histologically, the tumor was composed of benign appearing nevus-like cells with focal areas of extensive fibrous response. The tumor cells were positive for S-100 protein and with an antihuman melanoma antibody (MoAb 225, 28S) stain. Electron microscopy confirmed the nevomelanocytic nature of the tumor cells and demonstrated peculiar cytoplasmic crystalline tubular structures similar to those seen in cells infected with herpes virus type II. We propose the term "proliferating giant pigmented nevus" for this previous undescribed tumor.
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7/14. Giant condyloma acuminatum of the vulva and anal canal.

    This publication describes the second known reported case of benign giant condyloma acuminatum of the vulva and anal canal (Buschke-Loewenstein tumour). The diagnosis of squamous cell carcinoma was made initially on clinical examination and could not be excluded by punch biopsy. A full pathological study of the tumour established the diagnosis. A defunctioning colostomy and a perineo-ano-vulvectomy with groin gland dissection was performed and the patient is free of disease 36 months later. The biology of this type of tumour is discussed.
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8/14. Two distinct pathologic types of giant cell tumor of the vulva. A report of two cases.

    Giant cell carcinoma of the vulva has been described as a distinctive primary tumor of the vulva associated with multinucleated tumor giant cells and nuclear pleomorphism. These tumors have been reported to have a poorer prognosis than does squamous cell carcinoma, to which they are thought to be related. Two women were treated for primary vulvar malignancies possessing the morphologic features of giant cell tumor. Electron microscopy was not beneficial in distinguishing the tumors. A panel of immunoperoxidase procedures, including AE 1/3, 35 beta H-11, carcinoembryonic antigen, epithelial membrane antigen, HMB-45, S-100, leukocyte common antigen, placentalike alkaline phosphatase, alpha-1-antichymotrypsin and vimentin made it possible to distinguish the two tumors and characterized one as a nodular amelanotic melanoma with multinucleate tumor giant cells and the second as a squamous cell carcinoma with tumor giant cells. The latter term should replace the term giant cell carcinoma. Histologic criteria can help define this tumor.
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9/14. Semisurgical treatment of giant condylomata.

    A semisurgical method to treat giant condylomata acuminata is described. Several silk ligatures are placed on the tumourous mass. Complete recovery was found to occur even in very extensive lesions. The procedure proved to be safe, neither bleeding, late scarring, nor local recurrence were observed.
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10/14. Giant basal cell carcinoma of the vulva.

    A giant basal cell carcinoma of the vulva is reported. The lesion was atypical in its large size and gross morphology, therefore making clinical diagnosis difficult. diagnosis of this lesion is often delayed because of its varied clinical appearance. The importance of adequate biopsy and careful pathological evaluation of vulvar lesions is stressed. Differentiation from metatypical or basosquamous cell carcinoma is discussed, and proper therapy is reviewed.
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