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Background: Superficial myofibroblastoma of the lower female genital tract is a rare, recently described tumor, which has a distinctive clinico- pathological profile. It is a diagnosis that needs to be thinking for appropriate treatment.
Case: We report a 21-year old patient who presented with a pelvic mass, with no significant medical history. The radiological assessment showed a pelvic cystic tumor suppressing up the uterus. A surgical excision was performed with a diagnosis of superficial myofibroblastoma of the lower female genital tract. The patient received no adjuvant therapy after surgery and remains well with no signs of local recurrence at 16-month follow-up
Conclusion: This tumor should be differentiated from other mesenchymal lesions, which may arise in this area. Treatment and prognosis are different according to histological type. The authors discuss this finding through the diagnostic difficulties and the evolution of this entity.