Güncel Patoloji Dergisi
2018, Cilt 2, Ek Sayı
EPS408(33) - Agressive angiomyxoma ;case report
Kemik ve Yumuşak Doku Patolojisi
1 Sbü Sultan Abdülhamid Han Eğitim Ve Araştırma Hastanesi
Introduction
Aggressive angiomyxoma (AAM) 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. Under 250 cases of AAM were reported in the literature since the first description of AAM in 1983.
Case Report
A 39-year-old woman who visited our hospital presented with a 3-month history of perineal mass. Mass has grown over time. Characteristic appearances on magnetic resonance imaging include hypointensity on T1-weighted images and hyperintensity on T2-weighted images. The patient underwent surgery under anesthesia for removal of the perianal mass. 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 immunohistochemical CD31, CD34 and Desmin.
Conclusion
AAM is an uncommon mesenchymal neoplasm, occurring predominantly in the pelvi-perineal region of adults, first described in 1983, by Steeper and Rosai. Often reported from the vulvovaginal region, simultaneous abdominal swelling and gluteal region involvement has also been reported.Though 90% of patients are women, involvement in men has also been observed. AAMs are usually solitary.
AAM has not often been reported in Dermatological Journals. This could probably be because patients with AAM often register with the gynaecologist or a surgeon and rarely, if ever, report to a dermatologist. The diagnosis is often suggested by the pathologist, as with our case. Anahtar Kelimeler : Agressive angiomyxoma, histopathologic examination, immunohistochemistry
Introduction
Aggressive angiomyxoma (AAM) 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. Under 250 cases of AAM were reported in the literature since the first description of AAM in 1983.
Case Report
A 39-year-old woman who visited our hospital presented with a 3-month history of perineal mass. Mass has grown over time. Characteristic appearances on magnetic resonance imaging include hypointensity on T1-weighted images and hyperintensity on T2-weighted images. The patient underwent surgery under anesthesia for removal of the perianal mass. 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 immunohistochemical CD31, CD34 and Desmin.
Conclusion
AAM is an uncommon mesenchymal neoplasm, occurring predominantly in the pelvi-perineal region of adults, first described in 1983, by Steeper and Rosai. Often reported from the vulvovaginal region, simultaneous abdominal swelling and gluteal region involvement has also been reported.Though 90% of patients are women, involvement in men has also been observed. AAMs are usually solitary.
AAM has not often been reported in Dermatological Journals. This could probably be because patients with AAM often register with the gynaecologist or a surgeon and rarely, if ever, report to a dermatologist. The diagnosis is often suggested by the pathologist, as with our case. Anahtar Kelimeler : Agressive angiomyxoma, histopathologic examination, immunohistochemistry