Background: Intestinal-type vulvar adenocarcinoma (VAIt) is an exceptionally rare form of primary vulvar cancer, characterized by histological features resembling mucinous colonic carcinomas, including villo-glandular structures composed of goblet and Paneth cells with intracytoplasmic mucin. Objective: To provide a comprehensive synthesis of the existing literature on VAIt and to also report a case from our institution in order to define its clinical, pathological, and immunohistochemical characteristics and its management and prognosis. Materials and Methods: A systematic review of the literature according to PRISMA guidelines was performed through searching five electronic databases (MEDLINE, EMBASE, Web of Science, SCOPUS and Cochrane Library), considering studies from 1998 to May 2025. In our research, we included all peer-reviewed studies which reported cases of VAIt. Data about VAIt were extracted by included studies and compared. Results: All in all, 32 studies with a total of 40 cases (including our case) of VAIt were assessed. The median age at diagnosis was 58 years. Most tumors arose in the labia or perineal structures, often mimicking benign lesions. Immunohistochemistry consistently showed CK20 and CDX2 positivity, with variable CK7 and p16 expression. FIGO stage IA was the most frequent stage at diagnosis. Surgical excision was the mainstay of treatment, while adjuvant therapy was less commonly reported. Lymph node metastases were present in about 31.5% of cases. Despite aggressive histology, most patients were disease-free at follow-up. Mortality due to disease occurred in 10% of cases. Conclusions: VAIt is a very rare histotype of vulvar cancer. Compared to vulvar squamous cell carcinomas, approximately 40% of early-stage clinical diseases reported in the literature presented positive inguinal lymph nodes with recurrence even after many years. The optimal treatment is not well defined and should be based on the individual clinical history of the patient, as there are no established guidelines. Further studies and longer follow-up periods are needed to clarify the best therapeutic management and its long-term prognosis.
背景：肠型外阴腺癌（VAIt）是一种极为罕见的原发性外阴癌，其组织学特征类似于黏液性结肠癌，包括由杯状细胞和潘氏细胞构成的绒毛腺管结构，并伴有胞浆内黏液。目的：系统综述现有关于VAIt的文献，并结合本机构的一例病例报告，以明确其临床、病理及免疫组化特征、治疗方式及预后。材料与方法：根据PRISMA指南，通过检索五个电子数据库（MEDLINE、EMBASE、Web of Science、SCOPUS和Cochrane Library），对1998年至2025年5月期间的研究进行系统综述。我们纳入了所有报告VAIt病例的同行评议研究。从纳入的研究中提取VAIt相关数据并进行比较分析。结果：共评估了32项研究，总计40例VAIt病例（包括本机构病例）。诊断时的中位年龄为58岁。大多数肿瘤发生于阴唇或会阴结构，常与良性病变相似。免疫组化结果一致显示CK20和CDX2阳性，CK7和p16表达情况不一。诊断时FIGO分期以IA期最为常见。手术切除是主要治疗方式，辅助治疗报道较少。约31.5%的病例存在淋巴结转移。尽管组织学表现具有侵袭性，但大多数患者在随访期间无病生存。10%的病例因该疾病死亡。结论：VAIt是一种非常罕见的外阴癌组织学亚型。与外阴鳞状细胞癌相比，文献中报道的早期临床病例约40%存在腹股沟淋巴结阳性，且即使在多年后仍可能复发。目前尚无明确的治疗指南，最佳治疗方案尚未明确，应根据患者的个体临床情况制定。需要进一步研究和更长的随访时间来明确最佳治疗策略及其长期预后。
肿瘤(癌症)患者之家