Periurethral aggressive angiomyxoma: a case report

Abstract

Aggressive angiomyxoma is a rare soft tissue tumor of the pelvis. Notorious for its locally infiltrative behavior, this tumor is identified by pathological appearance. Grossly gelatinous, spindle cells widely separated by collagen fibrils with vascular components can be microscopically visualized. Wide local excision is the treatment of choice. A 19-year-old woman presented with a periurethral mass that extended beyond the hymen with Valsalva. With imaging, the differential was narrowed to a soft tissue mass. Surgical excision was performed and histopathological findings were consistent with the diagnosis of aggressive angiomyxoma. Twenty-four months later the patient remains recurrence free. The typical findings of aggressive angiomyxoma are highlighted as well as the novel presentation as a periurethral mass. Histological and radiological findings are reviewed as well as current treatment options.

Aggressive angiomyxoma of vagina: a rare entity

“…7 The tumor usually involves pelvis, often located in the vagina, vulva, perineum or buttocks. 3,4 It is a slow growing tumour invading paravaginal and pararectal spaces occupying whole pelvis with displacement of adjacent structures. Women may take two months to seventeen years in reporting to hospital with a painless mass after initial discovery.…”

“…2 It usually occurs in reproductive age with peak incidence between the third and fourth decades of life, with higher (95%) preponderance in women. [3][4][5][6][7] AA is a rare, locally aggressive myxoid mesenchymal tumor, arising in the deep soft tissues of the vulva, vagina, perineum and pelvis of young adult women.…”

“…MRI remains diagnostic modality of choice as swirled pattern is more specific than seen on CT. High signal intensity on MRI and attenuation on CT is due to loose myxoid matrix and high water content of angiomyxoma. 3,4,10 Preoperative imaging (CT or MRI) is of significant importance as it defines extent of tumour as well as involvement of surrounding structures thus helps to decide the operative route to be followed. 4,7 In our patient preoperative imaging with CT or MRI was not done as the clinical appearance was suggestive of vaginal cyst.…”

“…3,4,10 Preoperative imaging (CT or MRI) is of significant importance as it defines extent of tumour as well as involvement of surrounding structures thus helps to decide the operative route to be followed. 4,7 In our patient preoperative imaging with CT or MRI was not done as the clinical appearance was suggestive of vaginal cyst. Complete surgical resection with long term follow up and careful monitoring with imaging is essential in view of high risk of local recurrence.…”

A rare case of aggressive angiomyxoma of vulva

“…1 It mainly involves the pelvis, often located in the vagina, vulva, perineum or buttocks. 2 Female to male ratio is 6.6/1, usually of reproductive age, with a peak incidence between the third and fourth decades of life. [3][4][5][6] It's uniqueness is in being benign structurally although it is locally malignant behaviourally.…”