■原发性外阴非鳞状细胞癌是罕见的实体,包括各种肿瘤类型。其中,原发性外阴肠型腺癌(vPITA)极为罕见。直到2021年,文献中报告的病例不到25例。
■我们报告一例63岁女性vPITA,外阴活检时组织病理学诊断为印戒细胞肠型腺癌。准确的临床和病理检查排除了继发性转移定位,vPITA被诊断出来了.患者接受了根治性外阴切除术和双侧腹股沟股骨颈淋巴结清扫术。由于淋巴结阳性,进行了辅助化学放射疗法。在20个月的随访中,患者还活着并且没有疾病。
■这种非常罕见的疾病的预后尚不清楚,最佳治疗方法尚不明确。文献中报道的临床早期疾病中约有40%的腹股沟淋巴结阳性,超过外阴鳞状细胞癌。必须进行适当的组织病理学和临床诊断,以排除继发性疾病并建议适当的治疗。
UNASSIGNED: 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.
UNASSIGNED: 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.
UNASSIGNED: 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.