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Diagnostics and treatment of aggressive angiomyxoma

Aleksandra Maciejczyk12, Karol Bartecki12, Anna M. Czarnecka1, Anna Szumera-Ciećkiewicz3, Mateusz Spałek14, Michał Wągrodzki3, Piotr Rutkowski1, Tomasz Świtaj1

Abstract

Aggressive angiomyxoma (AAM) is a rare benign soft tissue tumor that occurs predominantly in premenopausal women. Due to its nonspecific symptoms, AAM diagnosismay be challenging. The clinical diagnosis of AMM is challenging due to its rarity and nonspecific physical and radiological characteristics. Aggressive angiomyxoma can present manifestations similar to entities such as Bartolin cysts or angiomyofibroma. Histological examination shows a hypocellular and highly vascular tumor with a myxoid stroma containing cytologically bland stellate or spindle cells. The current gold standard of AAM treatment is surgical resection, with no difference in the recurrence rate observed for radical incisions. Aggressive angiomyxoma often stains positively for estrogen and progesterone receptors, leading to the exploration of gonadotropin-releasing hormone agonists as adjuvant or neoadjuvant therapy. The presence of chromosomal abnormalities, including translocation at the 12q13-15 locus involving the HMGA2 gene has been identified in AAM. Although AAM is benign and does not show potential for metastasis, its infiltrative nature in surrounding tissues and the high rate of postoperative recurrences warrant the use of the term ‘aggressive.’ Surgical resection remains the current gold standard of treatment, but the possibility of therapy using gonadotropin-releasing hormone agonists has emerged. Ongoing and future studies of AAM can potentially reveal the role of hormones in tumor growth and genetic mutations responsible for its development. 

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