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      Rare paratesticular aggressive angiomyxoma mimicking an epididymal tumor in an 82-year-old man: Case report

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          Abstract

          Aggressive angiomyxoma (AAM) is a rare mesenchymal myxoid tumor, and most cases occur in the pelvic region or perineum of adult females. AAM is very rare in males. Most of these cases have been diagnosed in patients aged 30–60 years, and the tumors involved the pelvic cavity, scrotum, or spermatic cord. AAM can mimic inguinal hernia, hydrocele, or paratesticular neoplasm. Four male cases have been reported with paratesticular AAM mimicking a testicular/epididymal tumor, and to the best of our knowledge, this is the oldest patient in the literature. Because of its rarity, making an exact diagnosis before surgery is difficult. Herein, we present a case of AAM in an 82-year-old man and review the literature.

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          Aggressive angiomyxoma of the female pelvis and perineum. Report of nine cases of a distinctive type of gynecologic soft-tissue neoplasm.

          Nine case of a distinctive soft tissue tumor of the female pelvis and perineum are described. They were characterized by their occurrence in young women (ages 21-38), large size (up to 60 X 20 cm), locally infiltrative nature, and grossly gelatinous appearance. The initial clinical impression was usually that of a Bartholin gland cyst. The microscopic appearance was that of spindle or stellate cells widely separated by a loose myxoid stroma focally rich in collagen fibrils, a prominent vascular component, including many large thick-walled vessels without an arborizing pattern, and foci of proliferating glandular elements in two cases. Mitotic activity was exceedingly low. Ultrastructural study of the spindle cells showed features consistent with myofibroblastic differentiation. Four patients developed large local recurrences; one tumor recurred twice, 14 and 15 years after initial excision. No distant metastases have been documented to date, and all patients are alive and well. The differential diagnosis of this unusual tumor includes myxoma, myxoid liposarcoma, sarcoma botryoides, myxoid variant of malignant fibrous histiocytoma, nerve sheath myxoma, and other soft tissue tumors with secondary myxoid changes. We have chosen the term "aggressive angiomyxoma" for this neoplasm to emphasize the neoplastic nature of the blood vessels and its locally infiltrative and recurrent nature.
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            Aggressive angiomyxoma.

            Aggressive angiomyxoma is a rare mesenchymal tumor that most commonly arises in the vulvovaginal region, perineum, and pelvis of women. The term aggressive emphasizes the often infiltrative nature of the tumor and its frequent association with local recurrence. Patients often present with nonspecific symptoms which are frequently misdiagnosed with more common entities, such as a Bartholin cyst, lipoma, or hernia. Histologic examination reveals a hypocellular and highly vascular tumor with a myxoid stroma containing cytologically bland stellate or spindled cells. The tumor cells are characteristically positive for estrogen and progesterone receptors, suggesting a hormonal role in the development of the tumor. Chromosomal translocation of the 12q13-15 band involving the HMGA2 gene has been described. Surgical excision is the treatment of choice, although treatment with gonadotropin-releasing hormone agonists is an emerging therapy. Metastases are exceedingly rare, and overall, the prognosis is good.
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              Aggressive angiomyxoma in the scrotum expressing androgen and progesterone receptors.

              Aggressive angiomyxoma is a rare benign mesenchymal myxoid tumor that arises from the pelvic soft tissues and perineum in relatively young females. This tumor has the ability to infiltrate locally and has a high risk of local recurrence after extirpation, but no potential to metastasize. We report here a rare case of aggressive angiomyxoma that developed in the scrotum of a 47-year-old male. Immunostaining of the resected specimen revealed that the tumor cell nuclei stained strongly and diffusely for androgen receptors (80% of the tumor cells), and moderately and partly for progesterone receptors (20% of the tumor cells). However, staining was negative for estrogen receptors. It is highly suggested that the growth of aggressive angiomyxoma in males may depend on androgen manipulation, contrary to its frequent and close association with estrogen receptor expression, which has been reported in females.
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                Author and article information

                Contributors
                Journal
                Open Med (Wars)
                Open Med (Wars)
                med
                Open Medicine
                De Gruyter
                2391-5463
                30 June 2021
                2021
                : 16
                : 1
                : 973-977
                Affiliations
                Department of Urology, MacKay Memorial Hospital , Taipei City, Taiwan (R.O.C.)
                Department of Pathology, Tamsui Branch, MacKay Memorial Hospital , New Taipei City, Taiwan (R.O.C.)
                School of Medicine, MacKay Medical College , New Taipei City, Taiwan (R.O.C.)
                Department of Urology, Hsinchu Branch, MacKay Memorial Hospital , No. 690, Sec. 2, Guangfu Rd., East Dist., Hsinchu City, Taiwan (R.O.C.)
                Article
                med-2021-0317
                10.1515/med-2021-0317
                8247787
                a36444b9-dc36-44c7-befb-f297f5f611bf
                © 2021 Chi-Fang Chen et al., published by De Gruyter

                This work is licensed under the Creative Commons Attribution 4.0 International License.

                History
                : 12 March 2021
                : 16 May 2021
                : 07 June 2021
                Page count
                Pages: 5
                Categories
                Case Report

                aam,deep angiomyxoma,mesenchymal tumor,paratesticular mass,older

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