Foci Of Yolk Sac Tumor Research Articles

Immature teratoma within an omphalocele: A unique finding

Teratomas are the only true umbilical cord neoplasms. Antenatal diagnosis of an umbilical cord tumor in the presence of an exomphalos is difficult because of the rarity and pleomorphic presentation. We report a case of omphalocele containing an immature umbilical cord teratoma. A 3-day old girl with an antenatally diagnosed omphalocele was found to have, other than intestinal loops and a part of the liver, two solid masses at the caudal aspect near the top of the omphalocele sac. On exploration, a cystic structure with solid components was seen within the sac and excised. Microscopic examination revealed a teratoma with immature neural tissue represented by primitive neural tubes and rosettes. Seen in 4000–5000 live births, 70–80% of omphaloceles are associated with morphologic abnormalities of other systems. Prenatal diagnosis of umbilical cord teratomas is challenging when associated with an exomphalos but should be suspected when a mixed solid and cystic mass is documented attached to the umbilical cord in the midline. Till date, there are 19 reported cases of umbilical teratomas of which only five (26.3%) contained immature tissue. Surgical excision with close follow-up is the recommended treatment. Outcome depends on the presence of associated anomalies, extent of tumor resection, occurrence of surgical complications and the presence of foci of yolk sac tumor in the neoplasm. Immature teratoma within an omphalocele is a rarity. However, the malformation is surgically correctable and prognosis is largely determined by the presence of associated abnormalities.

ZBTB16: a novel sensitive and specific biomarker for yolk sac tumor

Although the function of zinc finger and BTB domain containing 16 (ZBTB16) in spermatogenesis is well documented, expression of ZBTB16 in germ cell tumors has not yet been studied. The aim of this study was to investigate the immunohistochemical expression and diagnostic utility of ZBTB16 in germ cell tumors. A total of 67 adult germ cell tumors were studied (62 testicular germ cell tumors, 2 ovarian yolk sac tumors, 1 mediastinal yolk sac tumor, and 2 retroperitoneal metastatic yolk sac tumors). The 62 testicular primary germ cell tumors are as follows: 34 pure germ cell tumors (20 seminomas, 8 embryonal carcinomas, 2 teratomas, 1 choriocarcinoma, 1 carcinoid, and 2 spermatocytic tumors) and 28 mixed germ cell tumors (composed of 13 embryonal carcinomas, 15 yolk sac tumors, 15 teratomas, 7 seminomas, and 3 choriocarcinomas in various combinations). Thirty-five cases contained germ cell neoplasia in situ. Yolk sac tumor was consistently reactive for ZBTB16. Among the 15 testicular yolk sac tumors in mixed germ cell tumors, all displayed moderate to diffuse ZBTB16 staining. ZBTB16 reactivity was present regardless of the histologic patterns of yolk sac tumor and ZBTB16 was able to pick up small foci of yolk sac tumor intermixed/embedded in other germ cell tumor subtype elements. Diffuse ZBTB16 immunoreactivity was also observed in 2/2 metastatic yolk sac tumors, 1/1 mediastinal yolk sac tumor, 2/2 ovarian yolk sac tumors, 2/2 spermatocytic tumors, 1/1 carcinoid, and the spermatogonial cells. All the other non-yolk sac germ cell tumors were nonreactive, including seminoma (n=27), embryonal carcinoma (n=21), teratoma (n=17), choriocarcinoma (n=4), and germ cell neoplasia in situ (n=35). The sensitivity and specificity of ZBTB16 in detecting yolk sac tumor among the germ cell tumors was 100% (20/20) and 96% (66/69), respectively. In conclusion, ZBTB16 is a highly sensitive and specific marker for yolk sac tumor.

Protocol for the Examination of Specimens From Pediatric and Adult Patients With Extragonadal Germ Cell Tumors

The College of American Pathologists offers these protocols to assist pathologists in providing clinically useful and relevant information when reporting results of surgical specimen examinations. The College regards the reporting elements in the ‘‘Surgical Pathology Cancer Case Summary (Checklist)’’ portion of the protocols as essential elements of the pathology report. However, the manner in which these elements are reported is at the discretion of each specific pathologist, taking into account clinician preferences, institutional policies, and individual practice. The College developed these protocols as an educational tool to assist pathologists in the useful reporting of relevant information. It did not issue the protocols for use in litigation, reimbursement, or other contexts. Nevertheless, the College recognizes that the protocols might be used by hospitals, attorneys, payers, and others. Indeed, effective January 1, 2004, the Commission on Cancer of the American College of Surgeons mandated the use of the checklist elements of the protocols as part of its Cancer Program Standards for Approved Cancer Programs. Therefore, it becomes even more important for pathologists to familiarize themselves with these documents. At the same time, the College cautions that use of the protocols other than for their intended educational purpose may involve additional considerations that are beyond the scope of these documents.

Krukenberg Tumor With Yolk Sac Tumor Differentiation

An unusual case of bilateral Krukenberg tumor with foci of yolk sac tumor (YST) differentiation occurring in a 50-year-old patient is reported. The primary tumor was in the gastric antrum, and it showed morphology of poorly differentiated adenocarcinoma with diffuse and solid growth pattern. A component of typical YST was not found in the gastric primary and lymph node metastases, although some cells in these locations were positive for alpha-fetoprotein. In the ovarian metastases, YST element showed microcystic/reticular and solid patterns, whereas the adenocarcinoma component was of diffuse type with signet ring cells and with some undifferentiated areas. The case represents further example of the somatic cell-derived tumor with focal germ cell-type differentiation and the first report of YST differentiation in Krukenberg tumor.

Clinicopathologic and prognostic study of pediatric immature teratoma

To study the clinicopathologic features and biologic behavior of pediatric immature teratoma. The clinical data, pathologic features, immunohistochemical findings (for cyclin D1, P27 and Ki-67) and follow-up information of 39 cases of pediatric immature teratoma were analyzed. Amongst the 39 cases studied, 12 arose in the sacrococcygeal region, 12 in testis, 5 in retroperitoneum, 4 in ovary, 4 in abdomen and 2 in mediastinum. Histologically, 16 cases were of grade 1, 8 cases of grade 2 and 15 cases of grade 3. Seven of the cases contained foci of yolk sac tumor. Immature neuroepithelial features used in histologic grading included the presence of primitive neural tubules, immature rosettes, undifferentiated neuroblastoma cells and primitive neuroectodermal structures. Immunohistochemical study showed that cyclin D1 was positive in 3 cases of grade 1 tumors, 4 cases of grade 2 tumors and 9 cases of grade 3 tumors. The positivity rates for p27 were 8, 3 and 6 cases respectively, while those for Ki-67 were 3, 4 and 13 cases respectively. Follow-up data were available in 30 cases. Three of them, including 2 cases with histologic grade 3 (with or without yolk sac tumor component), recurred after operation. The expression of cyclin D1 and Ki-67 is a useful adjunct in histologic grading. On the other hand, p27 overexpression shows little correlation with tumor grade. The prognosis of immature teratoma in children is different from that in adults. Sacrococcygeal immature teratoma occurring in patients younger than 1 year old and with low histologic grade do not require postoperative chemotherapy if the tumor is completely excised. Similarly, for testicular immature teratoma occurring in patients below 1 year of age, regardless of tumor grading, need no adjunctive therapy. On the other hand, ovarian immature teratoma with high histologic grade requires postoperative chemotherapy, regardless of age of the patients. The presence of microscopic foci of yolk sac tumor is a useful predictor of recurrence in pediatric immature teratoma.

Perinatal (fetal and neonatal) germ cell tumors

Background/Purpose Germ cell tumors are relatively common in the fetus and neonate and are the leading neoplasms in some perinatal reviews. The purpose of this study is to focus on the fetus and neonate in an attempt to determine the various ways germ cell tumors differ clinically and morphologically from those occurring in the older child and adult and to show that certain types of tumors have a better prognosis than others. Methods The author conducted a retrospective review of perinatal teratomas and other germ cell tumors reported in the literature and of patients treated and followed up at Children’s Hospital San Diego and Children’s Hospital Los Angeles. Only fetuses and infants less than 2 months of age with adequate clinical and pathologic data were accepted for review. Results Five hundred thirty-four fetuses and neonates presented with teratomas diagnosed prenatally (n = 226) and at birth (n = 309). The most common initial finding was a mass, noted either by antenatal sonography or by physical examination during the neonatal period, with signs and symtoms referable to the site of origin. Overall polyhydramnios was next followed by respiratory distress and stillbirth. The number of mature and immature teratomas was approximately the same. The incidence of teratoma with yolk sac tumor either at presentation or at recurrence was 5.8%, and the survival rate was 39%. Sacroccoccygeal teratomas had the highest incidence of yolk sac tumor at 10%. Recurrent disease in the form of either teratoma or yolk sac tumor developed in 5% of patients. All individuals with teratomas who survived received surgical resection. Conclusions Some germ cell tumors of the fetus and neonate have a better prognosis than others. Neonates with gastric teratomas have the best survival rates, and those with intracranial germ cell tumors the worst. Fetuses with teratomas detected antenatally have 3 times the mortality rate compared with postnatally diagnosed neonates. Although perinatal teratomas have a relatively low recurrence rate of 5%, close follow-up with imaging studies and serum α-fetoprotein determinationsis is strongly recommended. Surgical resection alone may be adequate therapy for teratomas with nonmetastatic, microscopic foci of yolk sac tumor. In the nonteratoma group, patients with pure yolk sac tumor and gonadoblastoma have a much better outcome than those with choriocarcinoma, which has a very low survival of rate of 12%. Currently, the use of platinum-based combination chemotherapy has significantly improved the survival rate of infants with advanced malignant germ cell tumor disease.

Surgery for ovarian masses in infants, children, and adolescents: 102 consecutive patients treated in a 15-year period

Background/Purpose: Ovarian pathology, although rare in children, must be included in the differential diagnosis of all girls who present with abdominal pain, an abdominal mass, or precocious puberty. Methods: To improve clinical appreciation of these lesions, the authors reviewed the presentation, evaluation, and outcome of all patients with ovarian pathology surgically treated at their institution since 1985. Results: One hundred two girls (aged 9.8 ± 5.5 years; range, 2 days to 20 years) underwent 106 separate ovarian operations (43 salpingo-oophorectomies, 21 oophorectomies, 33 ovarian cystectomies, and 9 ovarian biopsies). Of those presenting with acute abdominal pain (n = 59), 25 (42%) had ovarian torsion (14 associated with a mature teratoma), and only 1 (2%) had a malignant tumor. In contrast, of those presenting with an abdominal mass (n = 23), 6 (26%) had malignancies. There was no age difference between those with benign disease (9.9 ± 5.6 years; n = 96) and those with malignant tumors (8.6 ± 3.9 years, n = 10). Nine children had 10 operations for presumed malignant tumors (3 dysgerminomas, 2 immature teratomas with foci of yolk sac tumor, 2 juvenile granulosa cell tumors, 1 yolk sac tumor, and 1 Sertoli-Leydig cell tumor). These patients all had unilateral salpingo-oophorectomy, 4 had chemotherapy, and all are now disease free at 8.4 ± 4.1 years follow-up. Conclusion: Ovarian pathology remains a rare indication for surgery in girls less than 20 years of age. Because most of these lesions are benign, ovarian-preserving operations should be performed whenever feasible. J Pediatr Surg 36:693-699. Copyright © 2001 by W.B. Saunders Company.

Surgical resection alone is effective treatment for ovarian immature teratoma in children and adolescents: A report of the Pediatric Oncology Group and the Children’s Cancer Group

Objective: In both adult women and children the potential for malignant recurrence from ovarian immature teratoma has prompted the standard use of chemotherapy after complete resection of the primary tumor. The efficacy of postoperative chemotherapy in children and adolescents with ovarian immature teratoma, however, has not been established. A pediatric intergroup trial (INT 0106) was designed to determine the need for postoperative chemotherapy in patients with ovarian immature teratoma after management with surgical resection only. Study Design: Between 1990 and 1995, 44 patients with completely resected ovarian immature tumor and without postoperative chemotherapy, who were able to undergo assessment, were accrued. Tumor tissue was evaluated by central pathology review to confirm diagnosis and determine tumor grading of immature neural elements. Patients were followed carefully for recurrence of disease with appropriate diagnostic imaging and serum marker studies. Results: Thirty-one patients had pure ovarian immature teratoma with a tumor grade of 1 (n = 17), 2 (n = 12), or 3 (n = 2). Age at diagnosis ranged between 1.5 and 15 years (median, 10). Of the 29 patients studied, the serum α-fetoprotein level was elevated in 10 (34%); the median level was 25 ng/ml. Thirteen patients had ovarian immature teratoma plus microscopic foci of yolk sac tumor. Tumor grade was 1, 2, or 3 in 1, 6, and 6 patients, respectively. Age ranged between 6 and 20 years (median, 12). In the 12 patients evaluated for serum α-fetoprotein, 10 (83%) had elevated levels; the median level was 262 ng/ml. The 4-year event-free and overall survival for the ovarian immature teratoma group and for the ovarian immature teratoma plus yolk sac tumor group was 97.7% (95% confidence interval, 84.9%-99.7%) and 100%, respectively. The only yolk sac tumor relapse occurred in a child with ovarian immature teratoma and yolk sac tumor who was then treated with chemotherapy and is alive and free of disease 57 months after recurrence. Conclusion: The results of this study suggest that surgery alone is curative for most children and adolescents with resected ovarian immature teratoma of any grade, even when elevated levels of serum α-fetoprotein or microscopic foci of yolk sac tumor are present. This experience strongly supports avoiding the long-term effects of chemotherapy in most children with ovarian immature teratoma by reserving postoperative therapy for cases with relapse. (Am J Obstet Gynecol 1999;181:353-8.)

Urothelial Carcinoma of the Bladder with Foci of Yolk Sac Tumor

Urothelial Carcinoma of the Bladder with Foci of Yolk Sac Tumor

Immature teratomas in children: pathologic considerations: a report from the combined Pediatric Oncology Group/Children's Cancer Group.

Pediatric germ cell tumors (n = 135) with a major component of immature teratoma (IT) registered on Pediatric Oncology Group/Children's Cancer Group treatment protocols from 1990 to 1995 were reviewed. Sixty cases were pure IT with no malignant component and 75 were mixed tumors with a major component of IT. Foci of yolk sac tumor (YST) were present in all 75 mixed tumors; additional malignant components were present in 15. The IT component was as follows: 47% grade 3, 29% grade 2, 24% grade 1. There were no significant correlations between tumor grade and patient age by specific subsets or overall (all p > 0.10). Significant correlations were detected between stage and the presence of foci of YST (p = 0.0145) and grade and the presence of foci of YST (p < 0.001). Serum alpha-fetoprotein concentrations were elevated at diagnosis in 96% of ovarian tumors with foci of YST and were mildly elevated (< 60 ng/dL) in only 16% of tumors without YST. Overall 2- to 6-year survival rate was 96% and was related to the presence of YST. Central pathologic review revealed aspects of morphologic diagnosis that were most frequently misinterpreted by contributing pathologists. These included the classification of differentiating tissues as immature and the failure to recognize two well-differentiated patterns of YST (the hepatoid pattern resembling fetal liver and the well-differentiated glandular pattern resembling fetal lung or intestine). Such foci were often overlooked. The authors conclude that the presence of microscopic foci of YST, rather than the grade of IT, per se, is the only valid predictor of recurrence in pediatric IT at any site.