Abstract
Background: Superficial myofibroblastoma (SMF) is a very rare benign mesenchymal tumor in the female lower genital tract. Only 46 cases have been reported in the English language literature, among which only 7 cases arose in the vulva. Sometimes SMF histologically mimics aggressive angiomyxoma (AA) in which massive myxoid change in stroma is characteristic. We herein report a case of vulvar SMF with prominent myxoid stroma and review the literature with the emphasis on the differential diagnosis of SMF and AA. Case presentation: a 37-year-old woman presented with a painless mass in the vulva. Magnetic resonance imaging (MRI) showed a well-circumscribed 7 cm mass in the subcutis of the vulva. The tumor was resected. Histopathologically, the tumor was characterized by sparsely populated spindle-shaped cells in the fibromyxoid stroma. Thin-walled blood vessels were detected. Mitoses or pleomorphism was not found. Tumor cells were positive for vimentin, ER, PgR, and desmin. Some cells were positive for alpha-SMA and CD34. All cells were negative for S100 protein. Conclusions: because SMF and AA show different clinical prognoses, distinguishing SMF from AA is important. However, SMF may share many common histological features with AA: superficial localization (above fascia), sharp borderline from adjacent tissue, expansive growth pattern; a specific vascular pattern will lead to an accurate diagnosis of SMF. Familiarization with the histological characteristics of the two entities will help to make a prognostic prediction.
Highlights
Superficial myofibroblastoma (SMF) is a very rare benign mesenchymal tumor in the female lower genital tract
Only 46 cases have been reported in the English language literature [1,2,3,4,5,6]
SMF was known as superficial cervicovaginal myofibroblastoma because it was believed to occur exclusively in the cervix and vagina
Summary
Superficial myofibroblastoma (SMF) is a very rare benign mesenchymal tumor of the female genital region. SMF was known as superficial cervicovaginal myofibroblastoma because it was believed to occur exclusively in the cervix and vagina. In 2005, Ganesan R et al [2] proposed the term “superficial myofibroblastoma of the lower female genital tract” for this type of tumor because they found that some tumors with the same histological and immunohistochemical features could occur in the vulva. Many gynecologists and pathologists might not be familiar with this type of tumor. SMF is characterized by myofibroblast proliferation in collagenous and myxoid stroma. The collagen and myxoid stroma proportions may vary among cases. SMF may be associated with extensive edema in the stroma, which may make it difficult to distinguish it from aggressive angiomyxoma (AA). Familiarization with the characteristics of SMA and AA is important
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