1/7. Importance of accurate preoperative diagnosis in the management of aggressive angiomyxoma: report of three cases and review of the literature.BACKGROUND: Aggressive angiomyxoma is a benign but locally aggressive tumor that occurs mostly in young women. Because excision is often incomplete, the risk of local recurrence is high. This report describes differences in presentation and the importance of accurate preoperative diagnosis of this rare neoplasm. methods AND RESULTS: We describe three cases with different presentations. Two were initially misdiagnosed, and local recurrence necessitated reoperation. Accurate diagnosis in the third case was followed by complete excision, with no recurrence. CONCLUSION: Aggressive angiomyxoma should be considered in the differential diagnosis of young women who present with a well-defined mass in the pelvic tissue. Accurate preoperative diagnosis should alert the surgeon to the need for wide excision, which is essential for prevention of local recurrence.- - - - - - - - - - ranking = 1keywords = angiomyxoma (Clic here for more details about this article) |
2/7. Deregulation of HMGA2 in an aggressive angiomyxoma with t(11;12)(q23;q15).Aggressive angiomyxoma is a soft-tissue neoplasm with a predilection for the pelvic and perineal regions and a tendency to recur locally. Cytogenetic data on this tumor type are limited to five cases, three of which showed rearrangement of chromosomal bands 12q13-15. Molecular investigation of two of the tumors identified the HMGA2 gene as the target of the 12q rearrangements. However, the two previously analyzed tumors were different at the molecular level: in one, the rearrangement of 12q13-15 resulted in a fusion product, whereas, in the second case, the breakpoint was telomeric (3') to the HMGA2, leaving the gene intact although expressed in its entire length. To shed more light on the pathobiology of aggressive angiomyxoma and to investigate the molecular mechanisms behind the involvement of the HMGA2 gene in this tumor type (fusion transcript vs deregulated expression), we investigated, cytogenetically and with molecular techniques, one such tumor which presented a t(11;12)(q23;q15) as the sole karyotypic aberration. FISH analyses demonstrated no structural alteration of HMGA2 at the cytogenetic level; however, expression of the full-length gene was detected molecularly.- - - - - - - - - - ranking = 1keywords = angiomyxoma (Clic here for more details about this article) |
3/7. 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.- - - - - - - - - - ranking = 0.16666666666667keywords = angiomyxoma (Clic here for more details about this article) |
4/7. Aggressive angiomyxoma of the female pelvis and perineum. Report of nine cases of a distinctive type of gynecologic soft-tissue neoplasm.Nine case of a distinctive soft tissue tumor of the female pelvis and perineum are described. They were characterized by their occurrence in young women (ages 21-38), large size (up to 60 X 20 cm), locally infiltrative nature, and grossly gelatinous appearance. The initial clinical impression was usually that of a Bartholin gland cyst. The microscopic appearance was that of spindle or stellate cells widely separated by a loose myxoid stroma focally rich in collagen fibrils, a prominent vascular component, including many large thick-walled vessels without an arborizing pattern, and foci of proliferating glandular elements in two cases. Mitotic activity was exceedingly low. Ultrastructural study of the spindle cells showed features consistent with myofibroblastic differentiation. Four patients developed large local recurrences; one tumor recurred twice, 14 and 15 years after initial excision. No distant metastases have been documented to date, and all patients are alive and well. The differential diagnosis of this unusual tumor includes myxoma, myxoid liposarcoma, sarcoma botryoides, myxoid variant of malignant fibrous histiocytoma, nerve sheath myxoma, and other soft tissue tumors with secondary myxoid changes. We have chosen the term "aggressive angiomyxoma" for this neoplasm to emphasize the neoplastic nature of the blood vessels and its locally infiltrative and recurrent nature.- - - - - - - - - - ranking = 0.83333333333333keywords = angiomyxoma (Clic here for more details about this article) |
5/7. Aggressive angiomyxoma in males. A report of four cases.Aggressive angiomyxoma (AAM) is a rare, locally infiltrative but nonmetastasizing tumor of the pelvic and perineal soft tissues that occurs almost exclusively in adult females. The authors describe four cases of AAM in adult males that arose in the scrotum. There was some histologic variation among the tumors. One case was focally hypercellular around the blood vessels, two were more densely cellular throughout, and one had cystic degeneration. Three of the tumors were widely infiltrative, and one of the four tumors recurred locally. The clinicopathologic features of these cases are similar to those of AAM occurring in females. In either sex, AAM should be distinguished from benign myxoid tumors with a low risk of local recurrence and fully malignant myxoid tumors with distant metastatic potential.- - - - - - - - - - ranking = 0.83333333333333keywords = angiomyxoma (Clic here for more details about this article) |
6/7. Aggressive angiomyxoma of the female pelvis and perineum: case report and literature review.A rare neoplasm was described in young white women in 1983. Its gelatinous appearance, histologic characteristics, locally infiltrative nature, and local recurrence are discussed. Twenty-five cases have been previously reported, our case being the first one in the United Kingdom. The value of a preoperative computed tomographic scan and a systematic anatomic, microscopic, and pathologic identification of all perineal tumors are described.- - - - - - - - - - ranking = 0.66666666666667keywords = angiomyxoma (Clic here for more details about this article) |
7/7. Resection of a giant aggressive angiomyxoma in the philippines.INTRODUCTION: medicine for Humanity is a multidisciplinary team of health care professionals, travelling to developing areas throughout the world on medical missions. The following is one of many technically challenging and unique cases presented for treatment. CASE: A 38-year-old woman gradually developed a giant mass arising from the right labium majus, extending into the retroperitoneum. Surgical resection was performed by abdominal and perineal teams. Intraoperative blood loss was approximately 10,000 ml, requiring 18 units of whole blood transfusion-including 6 units acutely donated by members of the surgical team. A "pack and go back" technique was used for hemostasis. The tumor weighed 19.8 kg. Final histology confirmed an aggressive angiomyxoma. CONCLUSION: This patient had the largest, histologically confirmed, aggressive angiomyxoma described to date. The surgical management of this case followed the principles in treating hemorrhagic shock, but required modifications based on availability of resources in Cebu City, philippines.- - - - - - - - - - ranking = 1keywords = angiomyxoma (Clic here for more details about this article) |