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

Autor: Francesco Sopracordevole, Giulia Azzalini, Nicolò Clemente, Anna Del Fabro, Giorgio Giorda, Mariasole Fichera, Marco Gigante, Giorgio Bogani, Vincenzo Canzonieri
Jazyk: angličtina
Rok vydání: 2023
Předmět:
Zdroj: Gynecologic Oncology Reports, Vol 47, Iss , Pp 101193- (2023)
Druh dokumentu: article
ISSN: 2352-5789
DOI: 10.1016/j.gore.2023.101193
Popis: Background: Primary non-squamous cell carcinomas of the vulva are rare entities including various 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 exclude secondary disease and to recommend an adequate treatment.
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