creators_name: Patil, RN creators_name: Reshme, N creators_name: Kundargi, R creators_id: ravi2varsha@yahoo.co.in editors_name: Kakkilaya, Srinivas editors_id: Kakkilaya BS type: journale datestamp: 2014-02-25 12:41:09 lastmod: 2014-02-25 12:41:09 metadata_visibility: show title: Perineal Aggressive Angiomyxoma: A Rare Presentation ispublished: pub subjects: OJHAS full_text_status: public keywords: Aggressive angiomyxoma; Vulva; Benign; Surgery abstract: Aggressive angiomyxoma(AA) is defined as benign neoplasm but has infiltrative potential into skeletal muscle and fat. Aggressive angiomyxoma is a rare neoplasm with about 150 reported cases so far. It is most often found in women in reproductive age with a peak incidence in the fourth decade of life. Aggressive angiomyxoma is most often found in or in proximity to the lower pelvis, more specifically perineum, vulva, vagina or inguinal regions. Most AA are big, often more than 10 cm in largest diameter. Radical surgery with wide margins is the treatment of choice. The prognosis is very good here. Because of its rarity and unique presentation we are reporting a case of a huge perineal AA in a 42 years female which presented as a tumour coming out of the breach in skin. She underwent surgery, wide local excision and after 7 months follow-up is free of tumor. date: 2013-04-15 date_type: published publication: Online Journal of Health and Allied Sciences volume: 12 number: 1(11) publisher: Kakkilaya BS refereed: TRUE referencetext: 1. Micci F, Brandal P . Soft Tissue Tumors: Aggressive angiomyxoma. Atlas Genet Cytogenet Oncol Haematol. April 2007;11(4)340-343. Available at http://documents.irevues.inist.fr/bitstream/handle/2042/16967/04-2007-AggresAngiomyxomaID5203.pdf. 2. Kempson RL, Teixeira MR, Hendrickson MR. Tumors of the Vulva - Mesenchymal tumours. In: Travasolli F A, Devilee P. (Eds). World Health Organization Classification of Tumors - Pathology and Genetics of Tumours of the Breast and Female Genital Organs. IARC Press; Lyon. 2003. pages 328-329. Available at http://www.iarc.fr/en/publications/pdfs-online/pat-gen/bb4/bb4-chap7.pdf. 3. Coppola S, Desai A, Tzania D et al. Conservative En Bloc Surgery for Aggressive Angiomyxoma Achieves Good Local Control: Analysis of 14 Patients From a Single Institution. Int Journ of Gynecol Cancer. 2013;23:540-545. 4. Behranwala KA, Thomas JM. 'Aggressive' angiomyxoma: a distinct clinical entity. Eur J Surg Oncol. 2003;29(7):559-563. 5. Sadaghiani MM, Nazari F, Atashkhoei S. Aggressive Angiomyxoma of the Vulva: a Case Report. Shiraz E-Medical Journal. 2009;10(4):231-235. 6. Steeper TA, Rosai J. Aggressive angiomyxoma of the pelvis and perineum: report of nine cases of a distinctive type of gynaecologic soft tissue neoplasm. Am J Clin Pathol. 1983;7:463-475. citation: Patil, RN and Reshme, N and Kundargi, R (2013) Perineal Aggressive Angiomyxoma: A Rare Presentation. [Journal (On-line/Unpaginated)] document_url: http://cogprints.org/9145/1/2013-1-11.pdf