The Yolk Sac Tumor

Abstract

One of the most remarkable of human neoplasms, the yolk sac tumor, is reviewed, emphasizing its histologic diversity and differential diagnosis, occurrence at many sites, and the shared passion for this unique neoplasm of Dr Gunnar Teilum (who deserves almost all credit for delineation of the nature of the tumor and its features) and Dr Aleksander Talerman (who made his own contribution to our knowledge of it) and the friendship it helped forge between these 2 distinguished pathologists. In a unique series of articles, beginning in the early 1940s, Teilum delineated the distinctive features of the neoplasm and recognized that it was 1 of 2 initially included as “mesonephroma ovarii” by Dr Walter Schiller in 1939 (the second we now know as clear cell carcinoma). Teilum named the tumor “endodermal sinus tumor” because it came to his attention that papillary formations common in the yolk sac tumor resembled the endodermal sinuses of the rat placenta. He focused on the histogenesis of the tumor and its morphologic features culminating in a classic paper in Cancer in 1959. Although Teilum and others recognized that yolk sac tumor could be a component of mixed germ cell tumors, Talerman was one of the first to emphasize that, particularly in the testis, it was common to see yolk sac tumor as a component of a mixed germ cell tumor. Teilum, working in Copenhagen, and Talerman, when the former was alive, working in Rotterdam, developed a warm friendship in part due to their great interest in the yolk sac tumor, although it also extended to other areas of gonadal neoplasia and indeed beyond the boundaries of medicine when they shared time together. The typical histologic features of the yolk sac tumor are the reticular-microcystic patterns Teilum described, but various other patterns, including solid and even rarer ones such as glandular and hepatoid, are now well known. There are some interesting variations in the age distribution of this tumor at various sites: for example, vaginal examples are almost restricted to children under 2 years of age; those of the testis that are pure also occur mostly in young boys (average age about 20 months) but are occasionally seen in later years; ovarian examples peak at about 19 years of age; mediastinal forms are mostly restricted to young adult males. Brief consideration is also given to the occurrence of this tumor at well-known extragonadal sites such as retroperitoneum, mediastinum, and pineal as well as more exotic locations. Note is made of the recently emphasized occurrence of the yolk sac tumor on the background of a somatic neoplasm, most often endometrioid carcinoma of the ovary. Given the wide ranging and fascinating clinical and pathologic aspects of the neoplasm, it is no surprise that it continues to be a source of great interest to any pathologist who sees one or more examples, and we are indebted to Dr Teilum for his monumental studies and to Dr Talerman for his own contributions.