Rationale:
Aggressive angiomyxoma (AAM) is a rare and benign invasive mesenchymal stromal tumor predominantly in women at reproductive age. AAM tends to relapse locally and should be differentially diagnosed from other mesenchymal tumors.
Patient concerns:
We report here a rare case of massive vulvar AAM in a 22-year-old Chinese woman with left labia majora mass with ulcer.
Diagnoses:
The diagnosis "aggressive angiomyxoma of vulva" was based on clinicopathologic and immunohistochemical features.
Interventions:
A surgery with local excision of the mass was performed.
Outcomes:
The patient was discharged 12 days after the surgery. There was no AAM recurrence or metastasis in a period of 12-month follow-up.
Lessons:
The vulvar AAM is a benign and aggressive mesenchymal tumor. In this case, we present the diagnosis, treatment, and prognosis for vulvar AAM. The tumor was removed completely by the surgery, but a long-term follow-up is requisite for surveilling on recurrence.