Abstract

Aims and Background: Aggressive angiomyxoma is rare tumor of pelvic and perineal organs, occurring usually in women of reproductive age, and carrying a high tendency to local infiltration and relapse. It is non-malignant but can be locally aggressive and frequently recurs. Misdiagnosis is frequent. Wide excision is the preferred method of treatment but the challenge is to perform the degree of extirpation necessary to prevent recurrence. Most literature about Aggressive angiomyxoma consists of isolated case reports.Presentation of Case: We reported a case of 35-year-old Indian woman complaining of a slow and progressive growth of a left vulvar labia major a pedunculated tumor with long a stalk was detected second time after first resection 2yrs back, measuring of 15.0x8.0 cm. Wide local surgical resection of the tumor were performed. Histopathology diagnosed a large aggressive angiomyxoma with uninvolved resection margins. Patient remains without recurrence at 8.0-months follow up.Discussion and Conclusion: Non Aggressive angiomyxomais rare but should be excluded in any large vulval mass. We expect that awareness accompanied with wide free safe margin excision has important role to prevent the recurrence of tumor.

Highlights

  • Aggressive angiomyxoma (AA) is a rare, locally aggressive myxoidmesenchymal neoplasm arising in the pelvis and perineal regions

  • Patient was admitted in August 2016, and clinical examination showed a large skin covered non-tender, pedunculated polypoidal mass involving the middle part of left labia majora

  • The soft doughy tumor mass attached arising from vulva of size 15.0x8.0 cm (Figure 1 and 2)

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Summary

Introduction

Aggressive angiomyxoma (AA) is a rare, locally aggressive myxoidmesenchymal neoplasm arising in the pelvis and perineal regions. The term Aggressive angiomyxoma was not coined until 1983 but similar tumors were described as early as in the 1860s. Patient was admitted in August 2016, and clinical examination showed a large skin covered non-tender, pedunculated polypoidal mass involving the middle part of left labia majora. It usually occurs in women, especially middle-aged; 95% of total cases are found in females.[2] it has later been reported in males. Have been reported about 150 cases in the world medical literature.[4] This benign tumor usually presents in middle aged adults as single nodule or polypoid lesion that may be clinically confused with skin tag or neurofibroma.[5]

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