The AAM predominantly occurs in the perineal and pelvic regions, which leads to a possible misdiagnosis as Bartholin gland cyst or hernia. Moreover, AAM is also difficult to distinguish from angiomyofibroblastoma due to similar morphology.[14,15] Therefore, the diagnosis of AAM should be based on both clinical features and histologic pathologies.
The tumor maker CEA, CA125, or CA199 is normal in the complete blood count, which is consistent with the previous finding. In this case, the Hsp90 level was elevated, consistent with the role of Hsp90 playing an essential role in cancer and being also correlated with poor prognosis in cancer.[17–19] To some extent, it seems that AAM possesses potentially malignant or a tendency of regrowth. At gene expression level, AAM has a positive expression of vimentin, SMA, MSA, desmin, CD34, F8, ER, PR, and negative expression for S-100, CK, and CD68.[21,22] This suggests that AAM is characterized by differentiation into fibroblasts and muscle fibroblasts.
The surgical removal of the tumor is the main treatment for AAM. Drug therapy, such as GnRH agonists, possesses therapeutic effect such as shrinking AAM or retarding its recurrence as an adjunct therapy in some cases. Since ERs or PRs are commonly positive in AAM, targeting ERs or PRs may be used as a potential therapeutic target.[23–25] Other treatment method such as vascular embolism (ER and PR positive) may be used as adjuvant therapy, while radiotherapy and chemotherapy have an undefined and limited role. Given that its characteristics of aggression and relapse, appropriate management and long-term follow-up are necessary.
In conclusion, in this case, AAM is a locally benign and aggressive mesenchymal entity and the surgical removal of the solid mass cures the AMM without a sign of recurrence during 12-month follow-up. A long-term surveillance is further required for this treated patient with AAM.
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