Cases reported "Vulvar Neoplasms"

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1/39. 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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keywords = angiomyxoma
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2/39. Multifocal aggressive angiomyxoma: a case report.

    A case of aggressive angiomyxoma in a 25 year old woman is presented. The patient was admitted to hospital with a history of hesitancy of micturation and pain in the right iliac fossa. She was found to have a left labial mass, which was clinically diagnosed to be a Bartholin gland cyst. A pelvic ultrasound revealed an additional mass in the right paravesical region. At surgery, two distinct masses were removed, one from the right perivesical space and the other from the left labium. Both masses were rubbery, white, and gelatinous and showed similar histopathology findings of thick and thin walled vascular channels set in a loose myxoid stroma. A diagnosis of multifocal aggressive angiomyxoma was made. This is the first reported case of aggressive angiomyxoma occurring as two distinct masses in one patient.
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ranking = 1.4
keywords = angiomyxoma
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3/39. Vulvar hypertrophy with lymphedema. A mimicker of aggressive angiomyxoma.

    We report the case of a 43-year-old quadriplegic woman with bilateral vulvar enlargement. The clinical impression was labial hypertrophy, but the microscopic features mimicked aggressive angiomyxoma because of the location, hypocellular proliferation of fibroblastic cells in an edematous-myxoid stroma, and vessels with perivascular collagen deposition, which simulated the thick-walled vessels of aggressive angiomyxoma. Since the lesion lacked true thick-walled vessels and contained ectatic tortuous lymphatics, the pathologic interpretation was lymphedema. This vulvar lesion should be recognized to prevent the misdiagnosis of aggressive angiomyxoma.
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keywords = angiomyxoma
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4/39. Aggressive angiomyxoma of the pelvis and perineum: case study.

    Aggressive angiomyxoma (AAM) is a rare locally aggressive tumor affecting the pelvis and perineum of young females. Histopathologically, it is characterized by fibreoblasts in a myxoid background with vascular proliferation, scanty mitotic figure and no real capsule. AAM needs to be considered in the differential diagnosis of vulval mass in a reproductive age woman. We describe the first Ethiopian case of huge, recurrent, AAM of the pelvis and perineum in a 35 year old para II lady and stress that early diagnosis offers a better option for treatment by wide local excision and low recurrence rate.
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keywords = angiomyxoma
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5/39. Aggressive angiomyxoma of the pelvis. A case report.

    Aggressive angiomyxoma is a locally mesenchymal, benign, and rare neoplasm. The vulva, perineum, and pelvis are the most common sites of involvement. The preoperative diagnosis is postulated by CT, sonography, MR image and angiography. The immunohistochemical study reveals the definitive diagnosis. The therapy is only surgical, and because of its tendency to recur locally, the excision has to be as complete as possible.
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keywords = angiomyxoma
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6/39. Primary medical management of recurrent aggressive angiomyxoma of the vulva with a gonadotropin-releasing hormone agonist.

    BACKGROUND: An aggressive angiomyxoma of the pelvis is a locally infiltrative lesion treated with wide local excision. recurrence is common. A potential medical treatment alternative is reported. CASE: A 34-year-old woman presented with her second recurrence of a vulvar angiomyxoma following two prior surgical excisions. Analysis of the recurrent tumor for estrogen and progesterone receptors was strongly positive. The patient was treated with 3 months of a gonadotropin-releasing hormone (GnRH) agonist. Comparison of pre- and posttreatment magnetic resonance imaging scans showed complete radiographic resolution of the tumor. physical examination confirmed these findings. CONCLUSION: Medical management with a GnRH agonist may obviate the need for radical exenterative surgery for a recurrent aggressive angiomyxoma of the vulva.
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ranking = 1.4
keywords = angiomyxoma
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7/39. 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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ranking = 0.2
keywords = angiomyxoma
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8/39. 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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ranking = 0.2
keywords = angiomyxoma
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9/39. 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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ranking = 0.2
keywords = angiomyxoma
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10/39. Perineal excision of a large angiomyxoma in a young woman following magnetic resonance and angiographic imaging.

    BACKGROUND: Aggressive angiomyxomas are rare, arise from connective tissue of the perineum or the lower pelvis, and affect predominantly young women. CASE: We describe an unusual case of aggressive angiomyxoma in which the perineal approach was possible owing to MRI scanning and selective angiography indications. CONCLUSION: In cases of large aggressive angiomyxomas these diagnostic procedures should make it possible to decide which operative route might be best for the patient.
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ranking = 1.4
keywords = angiomyxoma
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