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 Loaf Tin Liner 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 Fan Shop - Collectibles - Frame Packs 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 exclude secondary disease and to recommend an adequate treatment.