Extraovarian pelvic yolk sac tumors

Abstract

The clinical and pathologic features of four extraovarian pelvic yolk sac tumors (YST) are described. The women, 17 to 39 years of age, were found to have a pelvic mass on physical examination, or in one case, at cesarean section. The tumors were bulky and arose within, or in close proximity to, the uterus. One tumor involved the endometrial cavity and myometrium extensively, another was attached to the anterior uterine serosa, another was in the cul-de-sac, and one lay between the lower uterine segment and urinary bladder. The ovaries were grossly unremarkable in all cases. Peritoneal metastases were present at the time of operation in two patients and were documented on histologic examination in a third. The serum alpha fetoprotein (AFP) level was elevated postoperatively in the three patients in whom it was determined. Three of the YST were pure and had a typical histologic appearance, whereas the fourth had an endometrioid-like glandular pattern and was associated with a teratoma. Immunohistochemical stains performed in three cases all showed AFP and alpha-1-antitrypsin (AAT). All patients received postoperative combination chemotherapy. Two of them died of tumor 14 and 24 months, respectively, postoperatively and two were disease-free 6 and 8.5 years, respectively, postoperatively.

RNA-binding protein LIN28 is a marker for primary extragonadal germ cell tumors: an immunohistochemical study of 131 cases

“…Primary extragonadal germ cell tumors are uncommon tumors that most often occur in the midline structures and mediastinum, but they can also occur in many other organs, not only in children but also in adults. [10][11][12][13][14][15][16][17][18][19][20][21][22][23][24][25][26] Morphologically, primary extragonadal germ cell tumors can mimic many types of non-germ cell tumors. For example, mediastinal seminomas may mimic type B thymomas and embryonal carcinoma may mimic other types of carcinomas.…”

“…The yolk sac tumor is the extragonadal germ cell tumor that poses the greatest diagnostic challenges because of its multiple histological patterns and wide organ distribution. [10][11][12][13][14][15][16][17][18][19][20][21][22][23][24][25][26] Previous markers for diagnosing extragonadal yolk sac tumors included placental-like alkaline phosphatase, a-fetoprotein, and glypican-3, but they lack adequate sensitivity and specificity. Recent studies have shown that SALL4 is a more sensitive marker for primary extragonadal yolk sac tumors.…”

Unusual Presentations of Gynecologic Tumors: Extragonadal Yolk Sac Tumor of the Vulva

“…6,[18][19][20][21][22][23][24][25][26][27][28][29][30] There are several theories regarding the histogenesis of extragonadal yolk sac tumor, including (1) arrested migration of or misplaced germ cells during embryogenesis, (2) reverse migration of germ cells, (3) abnormal differentiation of somatic cells, (4) derivation from pluripotential stem cells within a somatic tumor, (5) origination from residual fetal tissue following incomplete abortion (for primary endometrial yolk sac tumor), and (6) metastasis from an occult gonadal primary. 1,4,5,21,25 The association with somatic tumors such as endometrioid adenocarcinoma or carcinosarcoma, seen in some cases arising in the gynecologic tract of older patients, supports a non-germ cell origin (points 3, 4) for a subset of extragonadal yolk sac tumors. 1 However, in the case of yolk sac tumor primary in the vulva, misplaced/aberrant germ cell migration along the gubernaculum is the leading hypothesis.…”

“…3 It is estimated that 10% to 15% of yolk sac tumors will be of extragonadal origin, with the most common reported sites including sacrococcygeal, thorax (predominantly mediastinum), vagina, brain (predominantly pineal), and retroperitoneum. [3][4][5] At some sites there is a proclivity for certain ages: vaginal yolk sac tumor is seen almost exclusively in young ( 2 years old) girls, whereas mediastinal tumors are typically seen in young adult males. 3,6,7 In addition to classic midline extragonadal locations, isolated case reports and small series of yolk sac tumors arising in the head/neck region (thyroid, maxillary sinus, nasopharynx, mandible, orbit), omentum, pancreas and hepatobiliary system, stomach, bladder, uterus (endometrium, cervix), and vulva have been published.…”

Primary yolk sac tumor of the endometrium

“…A review of the 8 cases reported in the literature [1][2][3][4][5][6][7][8] was undertaken via PubMed search. All of the women in these cases underwent bilateral salpingo-oophorectomies, and endocrine functions were lost in all cases (Table 1).…”