Yolk Sac Tumor of the Ovary

Abstract

One hundred fifty yolk sac tumors (YSTs) of the ovary in patients from 1 to 61 (mean: 21.5) years of age are described; 75% of the patients were in the second and third decades and only 1 was above 50 years of age. The clinical manifestations were typically related to a fast-growing adnexal mass; endocrine manifestations (hirsutism) were present in only 2 cases. The tumors were all unilateral and 70% were ≥15 cm; an associated dermoid cyst was present in 20 cases. The tumors were solid and cystic in 57% of the cases, 25% were multicystic, and 18% uniformly solid. The solid tissue was typically tan to pink or yellow and often friable with hemorrhage and necrosis; smaller solid neoplasms were sometime uniformly yellow. The most common histologic pattern was reticular composed of an irregular meshwork of spaces that was conspicuous in 68% of the neoplasms but present to at least a minor degree in all of them. That appearance almost always merged with small to large cysts that were prominent in about 40% of tumors. In 25% of the tumors, cysts sometimes associated with a cellular stroma (the polyvesicular pattern), were present but conspicuous in only half these cases. One third of the tumors had a labyrinthine pattern, 22% glands, and 6% a festoon pattern. Papillae with a central blood vessel (Schiller-Duval bodies) were seen in one-third of the tumors but were numerous in only 5% of them. Nonspecific appearing papillae were seen in 10% of the tumors. A solid growth of cells with pale cytoplasm was seen in one-third of the tumors but was conspicuous in only half of that subset. The solid appearance was typically reminiscent of that of dysgerminoma, but lacked the septa and lymphocytic infiltrate of that neoplasm. Nine tumors had a component of cells with scant cytoplasm resulting in a blastema-like appearance and 3 had cells with abundant clear cytoplasm. Cords and clusters of cells were common but did not dominate the microscopic appearance. The stroma typically had a nonspecific collagenous to edematous appearance. Stromal luteinization was seen in 12 tumors; in 5 this was likely due to the patient being pregnant. Two tumors had minor foci of cells that resembled hepatocytes. Hyaline bodies were seen in most of the tumors and were often conspicuous. The neoplastic cells typically had modest amounts of lightly staining cytoplasm and only mild nuclear pleomorphism. Cells lining cysts were often flattened sometimes resulting in a deceptively innocuous appearance. Many of the tumors (mostly consultation cases), caused diagnostic difficulty; tumors in the differential diagnosis included clear cell carcinoma, embryonal carcinoma, Sertoli-Leydig cell tumor, and juvenile granulosa cell tumor. The patient age and marked elevation of the serum alpha-fetoprotein level (if measured) is helpful in many of these considerations. The overtly malignant gross appearance of most YSTs contrasts with certain other tumors in the differential and the association of some YSTs with dermoid cyst and many clear cell carcinomas with endometri...

Cytologic features of gynecologic germ cell tumors and carcinomas exhibiting germ cell tumor differentiation

“…For example, primary yolk sac tumors and carcinomas with yolk sac tumor‐like differentiation in our cohort frequently exhibited cytoplasmic vacuolation, a feature that has also been described in cytologic material from nongynecologic germ cell tumors 10–12 . Although hyaline globules and aggregates of basement membrane‐like material are not infrequently seen on histologic sections from gynecologic yolk sac tumors 13 and in cytologic material from yolk sac tumors at extragonadal sites, 14,15 we did not identify these in the cytologic slides in our study. It is not entirely clear why these structures were not readily identified in our cases, but this may have been caused by differences in cytologic specimen types and preparations (compared with the studies cited above).…”

Malignant Gonadal Germ Cell Tumors (Other Than Pure Germinoma) in Patients With Disorders of Sex Development

“…They are typically irregular in shape and surrounded by reticular/ microcystic foci. 2 A prominent cystic glandular pattern, to the best of our knowledge, has not yet been described in YST.…”

“…These figures contrast with the much lower frequencies, 22% and 34%, respectively, of these patterns, in YSTs of non-DSD patients 41 and even smaller figures in a recent report of 150 ovarian YSTs. 2 One study reported hepatic-like cells in 48% of YSTs from various sites 42 ; the latter, however, appeared to be small rather than extensive areas based on a review of the illustrations. pattern, whereas in the remaining 3, hepatoid morphology was described as prominent.…”

“…O ur interest in the occurrence of malignant neoplasms other than pure germinoma in the gonads of patients with disorders of sex development (DSD) came about following the involvement of one of us in 2 studies concerning malignant mixed germ cell tumors and yolk sac tumors (YSTs) of the ovary. 1,2 In identifying those cases from our files, some examples of these tumors were found in patients with abnormal gonads that harbored gonadoblastoma, prompting the realization that there is little information on malignant tumors other than pure germinoma in this setting. In addition, over the same time period, we reviewed a case of hepatoid YST in a patient with Turner syndrome, a provocative circumstance given the rarity of that neoplasm.…”

Refractory Ascites Revealing an Ovarian Yolk Sac Tumor with Intraperitoneal Rupture

“…The tumor is usually round, oval or globular with smooth and glistening surface that may be firm or somewhat lobulated. Cut sections are solid and cystic with gelatinous changes and areas of hemorrhage and necrosis [10].…”