Primary intestinal-type adenocarcinoma of the vulva. A case report and review of the literature

Primary intestinal-type adenocarcinoma of the vulva. A case report and review of the literature

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Background: Primary non-squamous cell carcinomas of the vulva are rare entities including various brown paper napkin tumor types.Among these, primary vulvar intestinal-type adenocarcinoma (vPITA) is extremely rare.Until 2021, less than twenty-five cases have been reported in the literature.Case presentation: We report a case of vPITA in a 63 years old woman with a histopathological diagnosis of signet-ring cell intestinal type adenocarcinoma at vulvar biopsy.

Accurate clinical and pathological work-up excluded secondary metastatic localization, and vPITA was diagnosed.The patient was treated with radical vulvectomy and bilateral inguinofemoral dissection.Adjuvant chemo-radiotherapy was performed because of a positive lymph node.At 20 months follow-up the patient was alive and free of disease.

Conclusion: The prognosis of this very rare disease is unclear and optimal treatment is not well established.About 40% of clinical early-stage diseases reported in literature had positive inguinal nodes, more than in vulvar squamous cell carcinomas.A proper histopathologic and clinical diagnosis is mandatory to harley himber instagram exclude secondary disease and to recommend an adequate treatment.