Cases reported "Pelvic Neoplasms"

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1/21. 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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2/21. 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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3/21. 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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4/21. Aggressive angiomyxoma of the pelvis: case report and review.

    OBJECTIVE: To report a case of recurrent aggressive angiomyxoma managed by a team composed of a radiologist, general surgeon, and reproductive endocrinologist, with a literature review which focuses on histologic differences between various types of myxomas. STUDY DESIGN: Case report and literature review. RESULTS AND CONCLUSIONS: The proband patient has an apparent cure, but this particular type of myxoma shows a proclivity for recurrence, sometimes years after resection. Therefore, long-term follow-up with MRI or CT scans is necessary. Preoperative management with vessel embolization and creation of gonadal suppression facilitates the surgical approach, which usually can be via a perineal, extraperitoneal route.
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keywords = angiomyxoma
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5/21. Aggressive angiomyxoma. A report of four cases occurring in men.

    Aggressive angiomyxoma is a distinctive, locally aggressive but nonmetastasizing soft tissue tumor of the pelvic soft tissues and perineum. This rare tumor occurs almost exclusively in adult women. We report four cases occurring in adult men, one each located in the scrotum, inguinal region, spermatic cord, and pelvis. The tumors were infiltrative, and were composed of fibromyxoid matrix sparsely populated by bland-looking spindled and stellate cells with delicate cytoplasmic processes. There were haphazardly scattered small and large blood vessels, some of which exhibited hypertrophy or hyalinization of the wall. Immunohistochemically, the stromal cells stained consistently for vimentin and variably for muscle-specific actin, but not alpha-smooth muscle actin, desmin, and S-100 protein. None of the four tumors recurred in follow-up times from 11 months to 6 years, although two previously reported cases in men recurred. This uncommon tumor occurring around the genital region in men merits wider recognition because of its potential for recurrence. It should be distinguished from benign tumors with low risk of recurrence on one hand, and from malignant myxoid tumors with metastatic potential on the other.
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ranking = 1
keywords = angiomyxoma
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6/21. Medical management of recurrent aggressive angiomyxoma with gonadotropin-releasing hormone agonist.

    patients with aggressive angiomyxoma may experience local recurrences. We report a case of recurrent aggressive angiomyxoma medically treated successfully with a gonadotropin-releasing hormone agonist. A 34-year-old woman with a huge perineal tumor underwent an extensive resection of the abdominoperineal tumor combined with total pelvic exenteration. histology showed aggressive angiomyxoma and the tumor cells were immunoreactive for estrogen and progesterone receptors. Although the patient had experienced no local recurrence for 12 months under adjuvant therapy with a gonadotropin-releasing hormone agonist, a recurrence occurred 3 months after the completion of adjuvant therapy. The patient underwent medical treatment with a gonadotropin-releasing hormone agonist and had a complete resolution of the recurrent tumor again. Hormonal treatment with a gonadotropin-releasing hormone agonist can be applied for small primary aggressive angiomyxomas in addition to adjuvant therapy for residual tumors.
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ranking = 1.6
keywords = angiomyxoma
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7/21. Aggressive angiomyxoma of the vulva extending into the pelvis: report of two cases.

    Aggressive angiomyxoma is a rare soft-tissue neoplasm found mainly in the female pelvis. Approximately 130 cases have been reported in the literature to date. In most cases treatment consists of surgical resection, but local recurrence rates remain high (36-72%). Therefore, long-term follow up is necessary and magnetic resonance imaging seems to be the preferred method for detecting recurrence. We report our experience of a primary and a recurrent aggressive angiomyxoma.
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ranking = 1.2
keywords = angiomyxoma
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8/21. Pelvic excision of large aggressive angiomyxoma in a woman: irradiation for recurrent disease.

    Aggressive angiomyxoma (AAM) is a rare tumor that preferentially involves the pelvis and perineal regions and arises from the connective tissue. Its cause and pathogenesis are unknown at present. Treatment typically involves surgery, and despite apparently complete resection, local recurrences are common. We describe a case of a large angiomyxoma of the left pelvis in a 59-year-old woman who underwent two surgical excisions. The first had been done in May 1998. She developed a local recurrence in December 1998. A palliative resection with macroscopic residuals was performed in February 2001, followed by radiation therapy with a total dose of 60 Gy. The diagnosis was revised at the time of the second operation. Initially, the tumor was diagnosed as angiomyofibroblastoma. Follow-up 3 years after the radiation treatment revealed no recurrence. The time of the local control achieved as yet is already longer than the former time to progression between the first two surgical procedures. This is, to our knowledge, the second description of a therapeutic irradiation of a recurrent AAM. radiation therapy is able to control a recurrent AAM for at least 3 years.
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ranking = 1.2
keywords = angiomyxoma
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9/21. A solitary encapsulated pelvic aggressive angiomyxoma.

    Aggressive angiomyxoma (AAM) was first reported in 1983 as a distinct, slow growing, benign but locally infiltrative, soft tissue tumour. It usually arises in the pelvic and perineal organs, mostly in women. A 47-year-old woman was found to have a large encapsulated retroperitoneal aggressive angiomyxoma. The mass was completely excised via abdomino-perineal approach, and no recurrence noted on MRI at 19 months' follow-up. The encapsulation of this tumour together with other reported rare presentations, suggest an isolated mesenchymal cell origin. A review of the literature is provided.
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ranking = 1.2
keywords = angiomyxoma
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10/21. Clinicopathological features of unusual vascular lesions of the pelvis, retroperitoneum and colon in females: a report of five cases and review of the literature.

    Vascular lesions comprise benign and malignant neoplasms as well as non-neoplastic conditions that may be located in various sites but only rarely in the pelvis or the retroperitoneum. In our study we describe five diverse and unusual cases of vascular lesions of the pelvis, retroperitoneum and colon in female patients: a case of retroperitoneal angiosarcoma, pelvic hemangioendothelioma, pelvic angiomyxoma, retroperitoneal lymphangioleiomyomatosis and a case of diffuse cavernous hemangiomatosis of the colon, with emphasis on their clinicopathological features and differential diagnosis. The recent literature on the subject is also briefly reviewed.
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ranking = 0.2
keywords = angiomyxoma
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