Introduction/Background Angiomyxoma aggressivum is an extremaly rare perineal tumor that expansivelly grows compressing the locally adjacent organs.
Methodology This is a case report of a 47-year-old patient with huge tumor of perineum localised on the left side of perineum, along the vaginal and rectal wall stretching out from the left obturator fossa till the left buttock. The tumor was previously partially debulked in other hospital giving the histopathological diagnosis of aggressive angiomyxoma with high estrogen receptors expression. The adjuvant hormotherapy of GNRH analogues was then introduced with initial good clinical outcome. After almost one year of clinical remission the flabby tumor grew back to the vast dimensions causing many ailments. After thorough imaging diagnostics excluding local pelvic muscles infiltration and any distant metastases as well the patient was qualified for radical debulking surgery from the perineal access. The resection was completely performed when the proper dissecting plane of this locally aggressive tumor had been found with the anatomic respect of vital vessels and nerves. The perineal plastic surgery followed then the surgical complete excision simultaneously. The postoperative course was uneventful. The patologic report confirmed the previous diagnosis of aggressive angiomyxoma with clear surgical margins.
Results The patient is being strictly followed-up for one year so far with no signs of the relapse both in clinical and imaging examinations.
Conclusion Perineal aggressive angiomyxoma is a very rare tumor with unequivocal tendency of local recurrence. The scant publications suggest that complete surgical resection is the best option to prevent of the disease relapse. Systematic treatment or radiotherapy have not been proved to be effective, however antiestrogenic hormontherapy is recommended as this kind of perineal tumor usually has pronounced hormonal receptors expression.
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