Aggressive angiomyxoma of the vulva: A case report
A 35-year-old female presented with a slow-growing mass on the right labium majora. She visited the clinic because she felt uncomfortable as the size of mass had grown. The patient was referred to gynecological services for further evaluation with considerations of a fibroepithelial polyp, a vulvar fibroma or a giant acrochordon. Local examination showed a well-circumscribed pedunculated polypoidal mass measuring about 10 × 7 cm. The mass was non-tender, soft and spongy in consistency. The inguinal lymph nodes were not enlarged. At first, we supposed the mass to be vulvar hemangioma and performed a pelvis magnetic resonance imaging (MRI). The pelvis MRI revealed findings that were compatible with those of AA in the subcutaneous layer of the right labium majora. A well-defined mass-like lesion of about 10 × 7 cm was seen in the subcutaneous layer of the right labium majora. The mass showed bright signal intensity on T2WI and demonstrated delayed enhancement after contrast administration (). The patient underwent local excision of the tumor with ligation of the stalk. The tumor mass was about 10 × 7 cm and it was soft and pink-tan colored (). Microscopically, the tumor was composed of spindle and stellate-shaped cells embedded in a loose myxoid matrix. These cells showed low to moderated cellularity and had eosinophilic cytoplasm with no significant nuclear pleomorphism and mitosis. Variable-sized thin-walled capillaries and thick-walled vascular channels were haphazardly arranged in the stroma. Some of these vessels showed perivascular hyalinization in the vascular walls (). Immunohistochemical staining of the tumor was positive for estrogen receptor (ER), progesterone receptor (PR), vimentin () and negative for S-100. Based on these pathologic features, aggressive angiomyxoma was diagnosed. She was continuously followed-up in our hospital for 7 years after resection, with no clinical or radiologic evidence of recurrence.