AGGRESSIVE ANGIOMYXOMA A CASE SERIES AND LITERATURE REVIEW

MRIs, CAT scans or pelvic ultrasound examinations are recommended for initial imaging of any pelvic-perineal-inguinal mass. The immunohistochemical findings of the present tumor confirmed positivity for both estrogen and progesterone receptors. Findings on CT and MR imaging. Complete resection is considered to be the treatment of choice for primary tumors and for recurrences , 6, 7, AA is considered as an invariably benign tumor; however, in a few recent reports, its metastasis to lungs resulting in death has been revealed. Nevertheless, angiomyxoma, unlike angiomyofibroblastoma, tests positive for factor VIII and negative for smooth muscle actin 3, 4, 7.

Physical examination often underestimates the total extent of angiomyxoma except for cases of small vaginal or vulvar lesions 6. Management requires complete removal of the lesion and the clinical and imaging follow-up given their high rate of recurrence. AA commonly manifests a painless swelling located around the genitofemoral region. Aggressive angiomyxoma was first described in by Steeper and Rosai as a locally infiltrative benign mesenchymal neoplasm. Unusual Abdominal and Pelvic Tumors. Incomplete or partial resection may lead to high recurrence rates.

It is notable that preoperative imaging has a crucial importance in the diagnosis of AA.

Case Reports in Surgery

Cosmetic results are satisfactory as a consequence of the approach selected, and there are no alterations in anorectal continence. Moreover, the optimal duration of therapy is unknown. AA is distinguished from the other lesions by its immunohistological findings. Introduction Aggressive angiomyxoma AA is an uncommon mesenchymal tumor which is predominantly encountered among adult females in reproductive age [ 1 ].

However, there is still a debate about the treatment because of high recurrence rates in spite of wide surgical excision. It is locally aggressive and metastasizes quickly and easily. Most are diagnosed in the first two to three years after initial surgery with average recurrence time between 9 and 14 months following surgery 2,9, 11, 12, CAT scans show tumors with well defined margins and lower attenuation than muscles while MRIs characteristically show high signal intensity.

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Case Reports in Medicine. Aggressive angiomyxoma AA is a rare pelvic soft-tissue tumor often found in the perineum which originates from myocardial fibroblasts. She was initially diagnosed as a femoral hernia and underwent surgery one month ago at another state hospital. Zuur and The Language Workshop Received: Also, due to the high rate of local recurrence, follow-up examinations with MRI and ultrasound alternating at intervals of one to two years should be sufficient.

Accordingly, she was referred to our clinic because of surgical failure.

Aggressive angiomyxoma: A case series and literature review

Aggressive Angiomyxoma in men. They described it lkterature having a predilection for the soft tissues of the pelvis and perineum of young women and of being aggressive in the sense of having high frequencies of local recurrence even after adequate initial treatment9.

Digital examination of the rectum was normal. The principle treatment should be complete surgical excision with tumor-free margins.

aggressive angiomyxoma a case series and literature review

Consequently MRIs are the method of choice for angiojyxoma and controlling possible recurrences 2, 3, 6, 7, piterature, 10, Cancer Genet Cytogenet ; 2: Results depend on the size of the lesion and on whether it is a primary or secondary tumor.

The evolution of the disease in one of these patients took 10 years from diagnosis while in the other it took 27 years. An immobile, painless mass which filled lower quadrants of the abdomen was palpated.

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The most common clinical symptom is painless swelling at vulva or groin area. Mitosis was not observed.

aggressive angiomyxoma a case series and literature review

They are most commonly found in pelvic locations in women. Although this finding initially suggested the use of the Refiew gene as a marker for this malignancy, the idea was rejected because the translocation is often shared by other mesenchymal tumors such as uterine leiomyomas, lipomas, pulmonary hamartomas, liposarcomas, and hemangiopericytomas.

The pathogenesis of AA is not well understood. This is key to determining the choice of surgical approach either perineal, abdominal, or both in order not angiomyxom leave residual tumor 2, 6, 9.

Male patients may test positive for estrogen and progesterone receptors Also, the tumor size is not correlated with recurrence.

aggressive angiomyxoma a case series and literature review

agrgessive Macroscopically, AA has a gelatinous appearance, and it is microscopically characterized by a myxoid stroma and abundant thin-thick walled vascular channels [ 16 ]. The external iliac artery was intact. Moreover, long-term treatment with GnRH agonists may induce adverse effects such as osteoporosis and depression even among young women. The tumor is distinguished from other lesions by these histopathologic features.