scholarly journals Pathogenesis of intracranial germ cell tumors reconsidered

1998 ◽  
Vol 5 (1) ◽  
pp. E3
Author(s):  
Keiji Sano

The author studied 153 cases of intracranial germ cell tumors (GCTs) through 1994, 62.7% of which showed monotypic histological patterns and 37.3% of which were shown to be mixed tumors. All of these cases, except for six patients who died soon after admission and underwent autopsy, underwent surgery followed by radio- and/or chemotherapy. All patients with choriocarcinoma died within 2 years. Patients with yolk sac tumor (endodermal sinus tumor) and embryonal carcinoma also had poor outcomes. Patients with mature teratoma had 5- and 10-year survival rates of 92.9% each. Patients with immature teratoma and malignant teratoma had a 5- and 10-year survival rate of 70.7% each. Patients with germinoma had a 5-year survival rate of 95.4% and a 10-year survival rate of 92.7%. These results may bring into question the validity of the germ cell theory, because germinoma, which should be the most undifferentiated according to the theory, was the most benign and choriocarcinoma and yolk sac tumor (endodermal sinus tumor), which should be the most differentiated, were the most malignant according to results obtained during follow-up study. Therefore, GCTs other than germinoma may not originate from one single type of cell (primordial germ cells). The embryonic cells of various stages of embryogenesis may perhaps be misplaced in the bilaminar embryonic disc at the time of the primitive streak formation, becoming involved in the stream of lateral mesoderm and carried to the future cranial area to become incorrectly enfolded into the brain at the time of the neural tube formation. The authors propound the following law: tumors composed of cells resembling the cells that appear in the earlier stages of embryogenesis (ontogenesis) are more malignant than those composed of cells resembling the cells that appear in the later stages of embryogenesis.

1999 ◽  
Vol 90 (2) ◽  
pp. 258-264 ◽  
Author(s):  
Keiji Sano

Object. To determine the pathogenesis of intracranial germ cell tumors (GCTs), the author studied 153 cases of these tumors encountered through 1994, 62.7% of which showed monotypic histological patterns and 37.3% of which were shown to be mixed tumors.Methods. Six patients died soon after admission and underwent autopsy; the other patients underwent surgery followed by radio- and/or chemotherapy. One hundred thirty-four cases were followed through the end of 1997. All patients with a choriocarcinoma died within 1 year. Patients with a yolk sac tumor (endodermal sinus tumor) or an embryonal carcinoma also had poor outcomes. Patients with a mature teratoma had 5- and 10-year survival rates of 93% each. Patients with an immature teratoma had 5- and 10-year survival rates of 86% each, whereas patients who had a teratoma with malignant transformation had a 3-year survival rate of 50%. Patients with a germinoma had a 5-year survival rate of 96% and a 10-year survival rate of 93%. These results may bring into question the validity of the germ cell theory because germinoma, which should be the most undifferentiated tumor according to the theory, was the most benign and choriocarcinoma and yolk sac tumor (endodermal sinus tumor), which should be the most differentiated tumors, were the most malignant according to results obtained during the follow-up study.Conclusions. Germ cell tumors other than germinomas may not originate from one single type of cell (primordial germ cells). The embryonic cells of various stages of embryogenesis may perhaps be misplaced in the bilaminar embryonic disc at the time of the primitive streak formation, becoming involved in the stream of lateral mesoderm and carried to the neural plate area to become incorrectly enfolded into the brain at the time of neural tube formation. The author propounds the following hypothesis: tumors composed of cells resembling the cells that appear in the earlier stages of embryogenesis (ontogenesis) are more malignant than those composed of cells resembling the cells that appear in the later stages of embryogenesis.


1998 ◽  
Vol 5 (1) ◽  
pp. E4 ◽  
Author(s):  
Ryo Nishikawa ◽  
Masao Matsutani

Primary intracranial germ cell tumors (GCTs) comprise 3.1% of all brain tumors and 13.6% of those in patients younger than 15 years of age in Japan. They are classified into five basic histological types: germinoma, teratoma, choriocarcinoma, yolk sac tumor, and embryonal carcinoma; or into mixed tumor types when they consist of two or more components. Radiation therapy with or without chemotherapy has proven effective in the treatment of germinoma, whereas there is a poor prognosis for choriocarcinoma, yolk sac tumor, embryonal carcinoma, and mixed tumors having components of the group of malignant intracranial GCTs. The underlying mechanisms for such different responses to radio- and chemotherapies of intracranial GCTs remain unknown. In this study, the authors analyzed the expression of p53 and p21WAF1/Cip1 proteins by immunohistochemical analysis in 35 intracranial GCTs. Expression of p53 protein was observed in 33 (94%) of 35 intracranial GCTs. Expression of p21WAF1/Cip1 was detected in seven (20%) of 35 intracranial GCTs. None of the 15 germinomas was immunoreactive for p21WAF1/Cip1 protein, whereas in a group of malignant intracranial GCTs, four (80%) of five cases showed immunoreactivity for p21WAF1/Cip1 protein. Analysis of the data suggests that overexpression of p21WAF1/Cip1 in intracranial GCTs may correlate with decreased sensitivity to radio- and chemotherapy and suggest a poor prognosis.


2017 ◽  
Vol 26 (3) ◽  
pp. 319-324 ◽  
Author(s):  
Akitake Mukasa ◽  
Shunsuke Yanagisawa ◽  
Kuniaki Saito ◽  
Shota Tanaka ◽  
Keisuke Takai ◽  
...  

Primary spinal germ cell tumors are rare, and spinal nongerminomatous germ cell tumors represent an even rarer subset for which no standard therapy has been established. The authors report the case of a 24-year-old woman with multifocal primary spinal germ cell tumors scattered from T-12 to L-5 that consisted of yolk sac tumor and mature teratoma. After diagnostic partial resection, the patient was treated with 30 Gy of craniospinal irradiation and 30 Gy of local spinal irradiation, followed by 8 courses of chemotherapy based on ifosfamide, cisplatin, and etoposide (ICE). Salvage surgery was also performed for residual mature teratoma components after the third course of ICE chemotherapy. Chemotherapy was continued after the operation, but ifosfamide was entirely eliminated from the ICE regimen because severe myelosuppression was observed after previous courses. The patient remains recurrence free as of more than 5 years after the completion of chemotherapy. This case suggests that this treatment strategy is an effective option for primary spinal yolk sac tumor.


1984 ◽  
Vol 61 (5) ◽  
pp. 931-937 ◽  
Author(s):  
Hirofumi Naganuma ◽  
Hiroshi Inoue ◽  
Shuzo Misumi ◽  
Masaru Nakamura ◽  
Masaru Tamura

✓ Histological and immunohistochemical findings in three cases with primary intracranial germ-cell tumors are reported. Based on histological examination, they were diagnosed, respectively, as an endodermal sinus tumor with immature teratoid differentiation, teratoma and embryonal carcinoma, and choriocarcinoma and germinoma. The first case was presumed to be a combination of endodermal sinus tumor and germinoma. An immunohistochemical examination showed positive reactions to alpha-fetoprotein (AFP) and carcinoembryonic antigen (CEA) in the endodermal sinus tumor, and to human chorionic gonadotropin (HCG) in the choriocarcinoma, but showed no reaction in the embryonal carcinoma. Human chorionic gonadotropin was demonstrated in syncytiotrophoblastic cells and CEA in a gland-like structure. The value of measuring CEA in cases of germ-cell tumors, in addition to AFP and HCG assays, is stressed, and the characteristics of CEA-positive tissue are discussed.


2003 ◽  
Vol 21 (5) ◽  
pp. 781-786 ◽  
Author(s):  
G. Calaminus ◽  
D.T. Schneider ◽  
J.P.M. Bökkerink ◽  
H. Gadner ◽  
D. Harms ◽  
...  

Purpose: To evaluate the prognostic value of metastases, extension into bone, and alpha-fetoprotein (AFP) elevation in children with malignant sacrococcygeal germ cell tumors (GCTs) prospectively collected in two cooperative Maligne Keimzelltumoren (MAKEI) protocols (83/86 and 89). Patients and Methods: Between October 1983 and October 1995, 76 of 210 registered patients with sacrococcygeal primaries presented either with pure yolk sac tumor, embryonal carcinoma (EC), or yolk sac tumor and EC mixed with immature and mature teratoma elements. Stages T1 and T2 disease were diagnosed in 15 and 61 children, respectively, 41 patients had metastases, and 35 children presented with extension into bone. At diagnosis, 22 children had an AFP elevation of less than 10,000 ng/mL. Thirty-six children showed an AFP level between 10,000 and 100,000 ng/mL, and 12 patients had values of greater than 100,000 ng/mL. Five patients died of complication during treatment and were excluded from further evaluation. Seventy-one patients could be analyzed. Results: The 5-year relapse-free survival rate (RFS, Kaplan-Meier) was 0.76 ± 0.03 (54 of 71 patients; median observation time, 54 months after diagnosis). The RFS of patients with and without metastases was different, but not significantly so (0.71 v 0.82). The outcome of patients with extension into bone (n = 31) and without this extension (n = 40) was 0.71 versus 0.80 (RFS, 5 years). Above-normal AFP level had no prognostic significance (P = .52). Conclusion: In children with malignant sacrococcygeal GCTs treated with an intensive, short-interval, platinum-based regimen, the stage, extent of metastases, extension into bone, and AFP level had no prognostic significance.


2016 ◽  
Vol 2016 ◽  
pp. 1-4 ◽  
Author(s):  
Sammy G. Nakhla ◽  
Srinath Sundararajan

Mediastinal germ cell tumors are extragonadal germ cell tumors (EGGCTs) commonly seen in children and young adults. They are more common in men. Clinically they are classified as teratomas, seminomas, and nonseminomatous germ cell tumors. Primary mediastinal yolk sac neoplasm is an extremely rare tumor. We present here a very rare case of primary yolk sac tumor of the anterior mediastinum in a 73-year-old male. Mediastinal germ cell tumors have a worse prognosis than gonadal germ cell tumors. Chemotherapy followed by adjuvant surgery improves overall response in EGGCTs. However, comorbidities can render treatment with chemotherapy and surgery challenging in elderly patients.


1987 ◽  
Vol 113 (2) ◽  
pp. 200-203 ◽  
Author(s):  
R. J. Stanley ◽  
B. W. Scheithauer ◽  
E. I. Thompson ◽  
D. B. Kispert ◽  
L. H. Weiland ◽  
...  

2020 ◽  
Vol 35 (2) ◽  
pp. 119-122
Author(s):  
SM Rashed Zahangir Kabir ◽  
Md Waheed Akhtar ◽  
Farida Yasmin

Introduction: Germ cell tumors are a group of tumors with different clinical presentation and histological and biological characteristics. Malignant germ cell tumors occur at all ages with a trend of bimodal distribution in infancy and adolescence. Objective: To evaluate the demographic characteristics, distribution of different types of germ cell tumor, treatment modalities and outcome of germ cell tumor in children in a tertiary care hospital of Bangladesh. Methods: In this retrospective study, data regarding age and sex distribution, location, types of tumors, management of germ cell tumor in children were retrieved from the medical records of pediatric oncology department in NICRH, Dhaka from 2008 to 2014. Results: Out of total 87 patients female were 50 and male 37. Most of the patients were up to 5 years of age. The gonadal germ cell tumors (80%) were more than extragonadal tumor (20%) in both male and female patients. The most common germ cell tumor was dysgerminoma (32%) followed by yolk sac tumor (29.8%) and teratoma (19.5%). Yolk Sac Tumor (51.4%) was the most common in male and dysgerminoma (56%) the commonest in female. Out of 87, seventy two (82.7%) received chemotherapy following surgery. Among those 72 patients who received chemotherapy 49 (68 %) patients completed their treatment. Until the last follow up 71.4% patients remained alive and tumor free. Conclusion: Germ cell tumors are the most variable tumor of all childhood malignancies that has difference in age, sex, location and histological subtypes. Gonadal tumors have better prognosis than extragonadal tumors in both the sex. DS (Child) H J 2019; 35(2) : 119-122


Sign in / Sign up

Export Citation Format

Share Document