Cases reported "Angiomyoma"

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1/6. prolapse of the fallopian tube after hysterectomy associated with exuberant angiomyofibroblastic stroma response: a diagnostic pitfall.

    We report two cases of prolapse of the fallopian tube associated with an exuberant angiomyofibroblastic stroma response, which occurred after hysterectomy and which is a hitherto unreported feature of this lesion. The tumors were composed of richly vascularized stroma arranged in a retiform pattern and mildly atypical glandular inclusions, which had the morphology of tubal epithelium. The stroma of the lesion was composed of either thin bipolar cells with tapered nuclei and stellate-shaped cells with minimal amount of cytoplasm or small epithelioid-looking cells with eosinophilic cytoplasm. The tubal glandular inclusions displayed mildly atypical nuclear features. If the tubal glandular component was overlooked, these tumors might be erroneously diagnosed as mesenchymal lesions of the vagina, such as vaginal fibroepithelial polyp, angiomyofibroblastoma, aggressive angiomyxoma, or superficial myofibroblastoma.
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keywords = angiomyxoma
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2/6. 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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keywords = angiomyxoma
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3/6. Giant perianal angiomyofibroblastoma--a case report.

    A 45-year old female had a long history of slow growing perianal tumor at the right side of her anus. Encapsulated tumour was found intraoperatively and completely excised using the Harmonic Scalpel. Tumour was well-circumscribed and relatively firm; measuring 12x6x4 cm. Histologically it was composed of oval to spindle cells with minimal nuclear atypia, set in mucous matrix with numerous thin-walled blood vessels. Immunohistochemically, expression of smooth-muscle actin and desmin, as well as estrogen and progesterone receptor were found in the tumour cells. The diagnosis of angiomyofibroblastoma was established. This rare benign tumour typically involves vulvovaginal, pelvic and perinal region. It is important to separate this neoplasm from locally invasive aggressive angiomyxoma and low grade fibromyxoid sarcoma, which can arise in the the same localisation. The patient was discharged on the third postoperative day and no recurrence was noted in 18 months follow-up.
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ranking = 1
keywords = angiomyxoma
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4/6. Vulvar angiomyxoma, aggressive angiomyxoma, and angiomyofibroblastoma: an immunohistochemical and ultrastructural study.

    To investigate the histogenetical unifying theory of a single, pluripotential primitive cell for vulvar angiomyxoma, aggresive angiomyxoma, and angiomyofibroblastoma, an optical, immunohistochemical and ultrastructural study of a superficial angiomyxoma, aggressive angiomyxoma, and angiomyofibroblastoma was performed. These three tumors showed immunohistochemical and ultrastructural overlapping features. The results of the study suggest that these three tumor entities probably arise on a common pluripotential primitive cell located around the vessels of connective tissue, which could show the capacity for modulating its penotype toward similar but distinct mature cell types.
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ranking = 12
keywords = angiomyxoma
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5/6. 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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ranking = 5
keywords = angiomyxoma
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6/6. 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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ranking = 3
keywords = angiomyxoma
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