Cases reported "Angiofibroma"

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1/10. 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.
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2/10. Cellular angiofibroma of the vulva with dna ploidy analysis.

    Cellular angiofibroma (CAF) is a recently described rare soft tissue neoplasm of the vulva (with only four reported cases) that typically occurs as a well-circumscribed solid rubbery vulvar mass in middle-aged women. The distinct histologic features of bland spindle cells admixed with numerous hyalinized medium to small blood vessels, and a vimentin-positive desmin-negative immunoprofile differentiates this neoplasm from other vulvar tumors such as angiomyofibroblastoma and aggressive angiomyxoma. In this report an additional case of CAF is presented with dna ploidy analysis and CD99 immunohistochemistry.
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3/10. Cellular angiofibroma of the vulva.

    BACKGROUND: The cellular angiofibroma is a benign mesenchymal neoplasm that clinically and histologically must be distinguished from biologically more aggressive lesions. It typically arises in women of late reproductive age and lends itself to cure by complete local excision. A report of an unusual case in a postmenopausal patient is presented. CASE: A 77-year-old woman presented with a painless vulvar mass that slowly enlarged over 3 years. Past history included a hysterectomy and bilateral salpingo-oophorectomy followed by estrogen replacement therapy. Surgical excision of the mass was performed and there is no evidence of recurrence 1 year postoperatively. Histopathologic examination revealed an admixture of hyalinized blood vessels and loose cellular stroma characteristic of a cellular angiofibroma. Immunohistochemical studies revealed stromal cell immunoreactivity for vimentin and CD34 and nonreactivity for desmin, actin, and S100 protein. The nuclei of the stromal cells demonstrated strong reactivity for estrogen and progesterone receptors. CONCLUSION: Mesenchymal lesions of the vulva and perineum include both benign and malignant neoplasms. The cellular angiofibroma is benign; however, other lesions including the aggressive angiomyxoma must be excluded when arriving at that diagnosis. The role of long-term estrogen therapy in the genesis of this tumor awaits further analysis.
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4/10. Angiomyofibroblastoma of the vulva: report of a case.

    A 45-year-old woman was first seen by us 2 years after becoming aware of a slightly painful subcutaneous mass in her left vulva. The mass was 7.5 x 3.0 cm in size, well circumscribed, mobile, and rubbery. It was at first clinically considered to be a benign tumor. Microscopically, the resected mass was composed of spindle or polygonal tumor cells which were cellularly or hypocellularly arranged with perivascular accentuation in a mucoid or fibrocollagenous background. Immunohistochemically, myxoid tumor cells were positive for vimentin but not for alpha-smooth muscle actin, CD34, CD31, desmin, or S-100 protein. The tumor was diagnosed as an angiomyofibroblastoma (AMBF), based on the typical findings of histology and immunohistochemistry. There are many histological types of vulvar tumors, and establishing a preoperative diagnosis is difficult in many patients. Rapid intraoperative pathological diagnosis should be performed if possible, considering the possibility of diseases such as AMFB and aggressive angiomyxoma (AAM). When AAM is suspected, the peripheral tissues should also be resected to prevent recurrence.
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5/10. Cellular angiofibroma of the vulva: case report with clinicopathological and immunohistochemistry study.

    CONTEXT: Cellular angiofibroma of the vulva is a rare tumor that was first described in 1997. It occurs in middle-aged women (average age: 47 years), has small size (< 3 cm) and well-circumscribed margins. CASE REPORT: We describe a case in a 51-year-old woman whose preoperative diagnosis was confounded with Bartholin's glandular cyst. The neoplasia was well delimited and made up of three characteristic components: fusiform cells forming small fascicles, numerous blood vessels and adipose tissue interspersed between the fusiform cells. The stroma cells were positive for vimentin and negative for CD34, protein s-100, actin and desmin. The differential diagnoses for this tumor include aggressive angiomyxoma, angiomyofibroblastoma, lipoma of fusiform cells, solitary fibrous tumor, perineurioma and leiomyoma.
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6/10. Angiomyofibroblastoma-like tumors (cellular angiofibroma).

    Angiomyofibroblastoma-like tumor (cellular angiofibroma) is a rare, circumscribed, slow-growing mesenchymal tumor that occurs predominantly in the vulva, perineum, and pelvis of women. We report two cases of this tumor in men arising as paratesticular masses of the scrotum, summarize the history of this tumor, and discuss why efforts should be made to differentiate it from aggressive angiomyxoma. Recommended treatment is complete surgical excision with long-term follow up exams, as local recurrence may occur many years after resection of the lesion.
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7/10. Perinodular hydropic degeneration of a uterine leiomyoma: a diagnostic challenge.

    Perinodular hydropic degeneration of a uterine leiomyoma is a rare form of the more common hydropic change observed in leiomyomas. With minimal discussion in the surgical pathology literature, appropriate evaluation may be challenging because the differential diagnosis includes other uncommon uterine disorders such as intravenous leiomyomatosis, diffuse leiomyomatosis, myxoid leiomyosarcoma, endometrial stromal sarcoma, angiofibroma, and angiomyxoma. We describe such a diagnostic challenge in a 42-year-old woman with a left adnexal mass discovered during an annual examination. With only three cases of perinodular hydropic degeneration previously reported, this case is the first with extrauterine extension and was initially concerning for a more aggressive process.
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keywords = angiomyxoma
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8/10. Comparison of angiomyofibroblastoma and aggressive angiomyxoma in both sexes: four cases composed of bimodal CD34 and factor xiiia positive dendritic cell subsets.

    Aggressive angiomyxoma (AA) is a distinctive, locally aggressive, fibromyxoid tumor of the pelvic and genital soft tissues. AA is of unknown histogenesis but the cytologically bland spindled tumor cells, which surround characteristic variegated blood vessels, show fibroblastic or myofibroblastic features. AA may be related to angiomyofibroblastoma (AMF), another cytologically bland fibromyxoid genital spindle cell tumor with variable myoid differentiation that does not, as a rule, recur. Recently, CD34 primitive fibroblasts and factor xiiia dendritic histiocytes have been found in varying combination in many fibrovascular, fibrohistiocytic, and myxoid soft tissue tumors. Both cells belong to the microvascular unit, a tissue responsible for stromal repair and remodeling and angiogenesis. To determine if these ubiquitous stromal cells participate in the histogenesis of AA and AMF, we examined two scrotal tumors, one AA with multiple recurrences and one AMF, for the presence of CD34 and FXIIIa dendritic cell subsets. For comparison, a vaginal AMF and a pararectal AA in a woman were included. We also studied actins and desmin to detect myofibroblastic differentiation, and, through double labeling studies, assessed hormone receptors and the cell cycle marker Ki 67 in the different cell subsets. The AA showed unusual cytologic atypia and was initially diagnosed as liposarcoma. It massively recurred four times over 12 years, the first time after seven years. The histologic appearance was fairly constant over the years. The scrotal AMF was a circumscribed 6 cm mass in a 37 year old man. In both cases, most tumor cells were wavy and fibrillar, spindled, stellate, or polygonal fibroblast-like CD34 dendritic cells. Depending on the area examined, a 20-50% subset of dendritic cells showed both nuclear and cytoplasmic staining for FXIIIa. Actin cells were rare but vessels had actin myopericytes, although a small focus of the initial male AA was desmin positive. The recurring AA expressed androgen receptors and had Ki 67 index of 10-20% in "hot spots" of the primary and up to 30% in recurrent tumors. The scrotal AMF widely expressed androgen and progesterone receptors with focal estrogen receptor positivity and the Ki 67 index was 10%. Both CD34 fibroblasts and FXIIIa histiocytes were present in the Ki 67 cycling fraction in both the male AA and AMF and both cell types expressed androgen receptors. The female pararectal AA had more focal CD34 reactivity, particularly in perivascular fibroblasts and these cells were admixed with small FXIIIa cells. The vaginal AMF was strongly desmin and variably to weakly CD34 with 20% FXIIIa dendritic cells and Ki 67 index of 2%. The vaginal AMF strongly expressed estrogen, progesterone, and androgen receptors. In conclusion our data suggest that at least some AA and AMF are myxoid fibrohistiocytic tumors composed of CD34 fibroblasts and FXIIIa dendritic histiocytes. In our tumors, neoplastic CD34 dendritic fibroblasts showed predominantly myxo-collagenous differentiation with prominent myofibroblastic differentiation in only one desmin vaginal AMF. Our results support the notion that AMF and AA are part of a morphologic and histogenetic continuum of myxofibrous and myoid tumors that may arise due to interactions between microvascular CD34 fibroblasts and FXIIIa histiocytes. CD34 and FXIIIa reactivity may be underappreciated in these tumors and is more important when considered histogenetically and biologically rather than in classifying individual neoplasms. Hormonal stimulation of proliferating pelvico-gential microvascular dendritic cells appears to play a role in the morphogenesis of both tumors.
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keywords = angiomyxoma
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9/10. Extravulvar subcutaneous cellular angiofibroma.

    Cellular angiofibroma is a rare distinctive mesenchymal neoplasm of the vulva or perineal region. We report here one unique extravulvar case. A 43-year-old woman presented with an asymptomatic tumor, 7 cm in diameter, located in the subcutaneous tissue of the chest below the left submammary sulcus. Histologically, the lesion was composed of uniform spindled stroma cells, numerous thick-walled vessels, and scarce mature adipocytes. An additional feature was the presence of prominent perivascular lymphoid aggregates. The stromal cells were positive for vimentin and negative for CD34 and muscle, epithelial, myoepithelial, or neural markers. Although nasopharyngeal angiofibroma or the group of acral angiofibromas have a concurrent heading, cellular angiofibroma should not be mistaken with them. Differential diagnosis of this distinctive tumor especially includes aggressive angiomyxoma, angiomyofibroblastoma, superficial angiomyxoma, vascular myxolipoma, and other tumors with spindle cells reminiscent of those in angiofibroma.
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10/10. Angiomyofibroblastoma of the vulva: case report.

    Angiomyxomas are a group of relatively rare mesenchymal myxoid tumors. Three types of angiomyxomas have been identified: superficial angiomyxoma, aggressive angiomyxoma and angiomyofibroblastoma. These tumors have a predilection for occurring in the perineum of middle-aged females and are often clinically diagnosed as Bartholin's cyst. A case of a 42-year-old female with a perineal angiomyofibroblastoma mimicking a Bartholin's cyst is presented. The pathological and clinical characteristics of this tumor are discussed.
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