Clinicopathological Features and Treatment Analysis of Rare Aggressive Angiomyxoma of the Female Pelvis and Perineum - a Retrospective Study.

The study was to evaluate the clinicopathological features of aggressive angiomyxoma (AAM) of the female pelvis and perineum and its treatments. This was a retrospective study of female patients with AAM admitted to our hospital. Clinical and pathological data were analyzed, as well as the postsurgical follow-up. Median age at initial presentation was 41 years. Thirteen patients had lesions involving adjacent organs. Eighteen patients underwent complete tumor resection, while one patient underwent partial tumor resection. The tumors were soft in texture, pink in color, and had mucus on the surface. A microscopic examination revealed that the tumors were non-encapsulated, with spindle cells and stellate cells of almost identical size loosely distributed in the myxoid stroma, and vessels of different sizes and wall thicknesses. Immunohistochemistry indicated that AAMs were strongly positive for CD34 and smooth muscle actin, moderately positive for desmin, estrogen receptors and progesterone receptor, and mostly negative for S-100. After a median follow-up of 24 months, the recurrence rate was 33.3 %. Four recurrences were in patients with positive initial margins. AAM is a slow growing, locally invasive, benign tumor. Complete resection could lead to lower recurrence rate compared with incomplete resection. Follow-up is necessary for recurrent cases with repeated surgeries. The overall prognosis could be favorable.