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. |
Databáze: |
Directory of Open Access Journals |
Externí odkaz: |
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