Vulva Glandular and Other Neoplasms
摘要
Primary vulvar adenocarcinomas are rare, and approximately half of adenocarcinomas are secondary. Glandular neoplasms are classified based on the presumed tissue of origin, the tumoral phenotype, or both. In this chapter, the authors summarize the clinicopathologic features of benign and malignant glandular tumors that originate from the vulva and related structures. Adenocarcinomas of this region encompass lesions that are defined by their primary site (such as adenocarcinomas of the Bartholin gland) and histomorphology and line of differentiation (such as adenocarcinoma of intestinal type). Other types, such as mammary-type adenocarcinomas, are presumed to originate from the mammary-like glands of the vulva and display a spectrum of pathologic features that are similar to their mammary counterparts. Similarly, vulvar carcinomas of sweat gland origin are pathologically like their counterparts in the nonvulvar skin and include a variety of cutaneous adnexal-type malignancies such as apocrine adenocarcinoma and eccrine adenocarcinoma. Invasive carcinomas of various types have been reported in 7–13% of extramammary Paget disease, and these are likely the most common primary glandular malignancy of the vulva.