Distinctive clinicopathological features and disease-specific survival of adenoid cystic carcinoma and adenoid basal carcinoma in the lower female genital tract

摘要

Adenoid cystic carcinomas (ACCs) and adenoid basal carcinomas (ABCs) in the lower female genital tract are very rare. Data on the clinicopathologic features and survival outcomes of ACCs and ABCs in the lower female genital tract are limited to case reports and small case series studies. The present study systemically analyzed 233 cases, including 84 cervical ACCs, 78 cervical ABCs and 71 vulvar ACCs, to identify clinicopathologic features and survival factors in a population-based Surveillance, Epidemiology and End Results (SEER) study. Whereas cervical ACCs and ABCs tend to occur in the elderly (median, 72 and 69 years, respectively), vulvar ACCs commonly occurred in patients a decade younger (median, 59 years). The majority of patients with cervical ABC had localized disease and almost all received surgery. In contrast, cervical and vulvar ACC patients tended to have higher stage disease, and a significant proportion of these patients received radiotherapy, with or without surgery. The 5-year cause-specific survival (CSS) rates for patients with cervical ACC were 69.3%, vulvar ACC 87.7% and cervical ABC 96.6%. The 5-year overall survival (OS) rate for patients with cervical ACC was 59.2%, significantly worse than that of cervical ABC (88.3%; P=0.002) and vulvar ACC (81.2%; P=0.01). Increased age and high stage were significantly associated with a worse prognosis in patients with cervical and vulvar ACCs by univariate and multivariate analysis (P0.05). Tumor stage was the only significant factor associated with 5-year overall survival in patients with cervical ABC (P0.05). The present data demonstrated that the distinctive clinicopathologic features and survival outcomes differed significantly among ACCs and ABCs in the lower female genital tract, thus providing a rationale for location/pathologic type-based treatment modalities.