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Aggressive angiomyxoma presenting with huge abdominal lump: A case report
© Kumar et al; licensee BioMed Central Ltd. 2008
- Received: 23 July 2008
- Accepted: 28 August 2008
- Published: 28 August 2008
Agressive angiomyxoma is a rare mesenchymal neoplasm. It mainly presents in females. We here present a case of angiomyxoma presenting as huge abdominal lump along with gluteal swelling. Case note is described along with brief review of literature.
- Progesterone Receptor Positivity
- Aggressive Angiomyxoma
- Rare Mesenchymal Neoplasm
- Myxomatous Tissue
- Trucut Needle
Aggressive angiomyxoma is a rare mesenchymal tumor occurring predominantly in the pelvi-perineal region of females. We report such a case presented as abdominal as well as gluteal lump, a very unusual presentation. Patient underwent laparotomy and tumor was successfully excised.
Aggressive angiomyxoma is an uncommon mesenchymal neoplasm occurring predominantly in the pelvi-perineal region of adults, first described in 1983 by Steeper and Rosai . About 90% of patients are women, usually of reproductive age . A few cases have been described in males, usually in scrotum. It presents as a painless, poorly circumscribed gelatinous vulvar mass and clinically simulates a bartholin gland cyst or an inguinal hernia. On gross examination the tumors are lobulated, soft to rubbery, solid masses. The cut surface reveals a glistening, soft homogeneous appearance. Recurrent tumors show more prominent areas of hemorrhage and fibrosis. Histologically angiomyxoma is a mesenchymal tumor, composed of fibroblasts within a strong myxoid background. Vascular proliferation is also prominent, and virtually no mitoses are present . The vast majority of cases demonstrate positivity for desmin in the myxoid bundles and/or stromal cells, while actins and CD34 may be variably positive . The estrogen and progesterone receptor positivity suggests that aggressive angiomyxoma might be hormone dependent as rapid growth has been observed during pregnancy. The tumor grows slowly, and its benign nature is suggested by the histology and by the fact that it shows no tendency to metastasize. However, it is locally aggressive and tends to recur (36–72%) after resection . Imaging of these tumors is important to determine extent and, thus, the optimal surgical approach. Sonography shows a mass that is hypoechoic or appears frankly cystic. Angiography usually shows a generally hypervascular mass. It has a characteristic appearance on CT and MR imaging and these techniques reveal the extent of the tumor as well. On CT, the tumor has a well-defined margin and attenuation less than that of muscle. On T2-weighted MR imaging, the tumor has high signal intensity . Treatment is usually surgery in form of wide local excision. Preoperative angiographic embolization, preoperative external beam irradiation and intraoperative electron beam radiotherapy are useful to decrease the chances of local recurrence . Hormonal treatment with a gonadotropin-releasing hormone agonist can be applied for small primary aggressive angiomyxomas in addition to adjuvant therapy for residual tumors .
Although a rare diagnosis, aggressive angiomyxoma can present with unusual features. Detailed radiological examination is helpful in suspecting the problem, but histology is gold standard for diagnosis. Wide excision is curative and prognosis of such tumor is good.
A fully informed written consent was obtained from the patient for the publication of this case report and accompanying images. A copy of the written consent can be sent to Editor-in-Chief of this journal if article is accepted for publication.
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