Pathogenesis of intracranial germ cell tumors reconsidered

1999 ◽  
Vol 90 (2) ◽  
pp. 258-264 ◽  
Author(s):  
Keiji Sano
Keyword(s):  
Survival Rate ◽  
Germ Cell ◽  
Survival Rates ◽  
Germ Cell Tumors ◽  
Yolk Sac ◽  
Endodermal Sinus Tumor ◽  
Content Type ◽  
Intracranial Germ Cell Tumors ◽  
Sinus Tumor ◽  
Fine Print

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.

scholarly journals Pathogenesis of intracranial germ cell tumors reconsidered

1998 ◽  
Vol 5 (1) ◽  
pp. E3
Author(s):  
Keiji Sano
Keyword(s):  
Survival Rate ◽  
Germ Cell ◽  
Mature Teratoma ◽  
Germ Cell Tumors ◽  
Yolk Sac ◽  
Yolk Sac Tumor ◽  
Single Type ◽  
Endodermal Sinus Tumor ◽  
Intracranial Germ Cell Tumors ◽  
Sinus Tumor

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.

Primary intracranial germ cell tumors: a clinical analysis of 153 histologically verified cases

1997 ◽  
Vol 86 (3) ◽  
pp. 446-455 ◽  
Author(s):  
Masao Matsutani ◽  
Keiji Sano ◽  
Kintomo Takakura ◽  
Takamitsu Fujimaki ◽  
Osamu Nakamura ◽  
...  
Keyword(s):  
Radiation Therapy ◽  
Germ Cell ◽  
Malignant Tumors ◽  
Survival Rates ◽  
Germ Cell Tumors ◽  
Surgical Removal ◽  
Content Type ◽  
Intracranial Germ Cell Tumors ◽  
Mixed Tumors ◽  
Fine Print

✓ The authors analyzed 153 cases of histologically verified intracranial germ cell tumors. The histological diagnosis was germinoma in 63 patients (41.2%), teratoma in 30 (19.6%), and other types of tumors in 60 patients (39.2%). The patients were treated by a consistent policy of surgical removal with histological verification followed by radiation therapy with or without chemotherapy. The 10- and 20-year survival rates of patients with pure germinoma were 92.7% and 80.6%, respectively. The 10-year survival rates of patients with mature teratoma and malignant teratoma were 92.9% and 70.7%, respectively. Patients with pure malignant germ cell tumors (embryonal carcinoma, yolk sac tumor, or choriocarcinoma) had a 3-year survival rate of 27.3%. The mixed tumors were divided into three subgroups: 1) mixed germinoma and teratoma; 2) mixed tumors whose predominant characteristics were germinoma or teratoma combined with some elements of pure malignant tumors; and 3) mixed tumors with predominantly pure malignant elements. The 3-year survival rates were 94.1% for the first group, 70% for the second group, and 9.3% for the third group, and the differences were statistically significant. Twenty-six patients with malignant tumors received chemotherapy that consisted of cisplatin and carboplatin combinations with or without radiation therapy. However, chemotherapy was not significantly more effective than radiation therapy alone. From these treatment results, the authors classified tumors into three groups with different prognoses and proposed a treatment guideline appropriate for the subgroups.

Intracranial germ-cell tumors

1984 ◽  
Vol 61 (5) ◽  
pp. 931-937 ◽  
Author(s):  
Hirofumi Naganuma ◽  
Hiroshi Inoue ◽  
Shuzo Misumi ◽  
Masaru Nakamura ◽  
Masaru Tamura
Keyword(s):  
Germ Cell ◽  
Chorionic Gonadotropin ◽  
Human Chorionic Gonadotropin ◽  
Embryonal Carcinoma ◽  
Germ Cell Tumors ◽  
Endodermal Sinus Tumor ◽  
Content Type ◽  
First Case ◽  
Intracranial Germ Cell Tumors ◽  
Sinus Tumor

✓ 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.

Primary pineal endodermal sinus tumor with elevated serum and CSF alphafetoprotein levels

1980 ◽  
Vol 53 (2) ◽  
pp. 244-248 ◽  
Author(s):  
Norio Arita ◽  
Shoji Bitoh ◽  
Yukitaka Ushio ◽  
Toru Hayakawa ◽  
Hiroshi Hasegawa ◽  
...  
Keyword(s):  
Therapeutic Response ◽  
Elevated Serum ◽  
Germ Cell Tumors ◽  
Pineal Region ◽  
Endodermal Sinus Tumor ◽  
Immunofluorescence Method ◽  
Content Type ◽  
Intracranial Germ Cell Tumors ◽  
Sinus Tumor ◽  
Sensitive Marker

✓ A 12-year-old girl with an alphafetoprotein-producing brain tumor in the pineal region is presented. The marker was detected in both serum and cerebrospinal fluid. The amount of the marker decreased after irradiation of the tumor and systemic chemotherapy and increased prior to tumor recurrence. At autopsy, the tumor was histopathologically diagnosed as an endodermal sinus tumor, and alphafetoprotein was demonstrated in the tumor cells by the immunofluorescence method. Alphafetoprotein may represent a specific and sensitive marker for predicting tumor histopathology and for monitoring the therapeutic response in patients with primary intracranial germ-cell tumors.

Intracranial germ-cell tumors in children

1991 ◽  
Vol 74 (4) ◽  
pp. 545-551 ◽  
Author(s):  
Harold J. Hoffman ◽  
Hiroshi Otsubo ◽  
E. Bruce Hendrick ◽  
Robin P. Humphreys ◽  
James M. Drake ◽  
...  
Keyword(s):  
Survival Rate ◽  
Adjuvant Therapy ◽  
Germ Cell ◽  
Germ Cell Tumors ◽  
Pineal Region ◽  
Pineal Tumors ◽  
Content Type ◽  
Suprasellar Region ◽  
Intracranial Germ Cell Tumors ◽  
Sick Children

✓ All patients with confirmed intracranial germ-cell tumors treated at the Hospital of Sick Children during the period January, 1952, to December, 1989, were reviewed. Of the 51 tumors reviewed, 16 were located in the suprasellar region, 32 in the pineal region, and three in both the pineal and the suprasellar regions. Forty-nine patients underwent surgical resection which was total in seven and partial in 20, and consisted of a biopsy in 22. Two patients were managed on the basis of serum and cerebrospinal fluid markers. Surgical tools such as the operating microscope, the ultrasonic surgical aspirator, and the laser beam allowed safe debulking and removal of the deep-seated tumors in the pineal region. There were no operative deaths in the 36 patients treated since 1972, who included 23 with pineal tumors. Twenty-five patients with germinomas received radiotherapy and had a 5-year survival rate of 85.1%. Thirteen patients with non-germinoma germ-cell tumors received radiotherapy and had a 5-year survival rate of 45.5%. On the basis of this review, the authors recommend resection of pineal and suprasellar germ-cell tumors in order to firmly establish an accurate histological diagnosis to guide the extent of adjuvant therapy. In the case of a pure germinoma without evidence of dissemination, adjuvant therapy consists only of local radiotherapy. On the other hand, for malignant non-germinoma germ-cell tumors, adjuvant therapy must include chemotherapy as well as craniospinal axis radiotherapy.

Intracranial germ-cell tumors: natural history and pathogenesis

1985 ◽  
Vol 63 (2) ◽  
pp. 155-167 ◽  
Author(s):  
Mark T. Jennings ◽  
Rebecca Gelman ◽  
Fred Hochberg
Keyword(s):  
Spinal Cord ◽  
Germ Cell ◽  
Third Ventricle ◽  
Age Distribution ◽  
Germ Cell Tumors ◽  
Content Type ◽  
Intracranial Germ Cell Tumors ◽  
Suprasellar Cistern ◽  
Extent Of Disease ◽  
Fine Print

✓ The natural history of primary intracranial germ-cell tumors (GCT's) is defined from 389 previously published cases, of which 65% were germinomas, 18% teratomas, 5% embryonal carcinomas, 7% endodermal sinus tumors, and 5% choriocarcinomas. Intracranial GCT's display specificity in site of origin. Ninety-five percent arise along the midline from the suprasellar cistern (37%) to the pineal gland (48%), and an additional 6% involve both sites. The majority of germinomas (57%) arise in the suprasellar cistern, while most nongerminomatous GCT's (68%) preferentially involve the pineal gland (p < 0.0001). The age distribution of afflicted patients is unimodal, centering with an abrupt surge in frequency in the early pubertal years; 68% of patients are diagnosed between 10 and 21 years of age. Nongerminomatous GCT's demonstrate an earlier age of onset than do germinomas (p < 0.0001). Prolonged symptomatic intervals prior to diagnosis are common in germinomas (p = 0.0007), in suprasellar GCT's (p = 0.001), and among females (p = 0.02). Parasellar germinomas commonly present with diabetes insipidus, visual field defects, and hypothalamic-pituitary failure. Nongerminomatous GCT's present as posterior third ventricular masses with hydrocephalus and midbrain compression. Germ-cell tumors may infiltrate the hypothalamus (11%), or disseminate to involve the third ventricle (22%) and spinal cord (10%). Among a subpopulation of 263 conventionally treated patients, two factors were of prognostic significance: 1) histological diagnosis; germinomas were associated with significantly longer survival than nongerminomatous GCT's (p < 0.0001); and 2) staging of the extent of disease; this emphasizes the ominous character of involvement of the hypothalamus (p = 0.0002), third ventricle (p = 0.02), or spinal cord (p = 0.01). Specific recommendations regarding the necessity of histological diagnosis and staging of the extent of disease are made in light of modern chemotherapeutic advances. The pathogenesis of GCT's may be revealed by their specificity of origin within the positive (suprasellar cistern-suprachiasmatic nucleus) and negative (pineal) regulatory centers for gonadotropin secretion within the diencephalon. The abrupt rise in age distribution at 10 to 12 years suggests that the neuroendocrine events of puberty are an “activating” influence in the malignant expression of these embryonal tumors.

Primary intracranial endodermal sinus tumor

1979 ◽  
Vol 50 (2) ◽  
pp. 246-252 ◽  
Author(s):  
Thomas J. Eberts ◽  
Robert C. Ransburg
Keyword(s):  
Pineal Region ◽  
Yolk Sac ◽  
Endodermal Sinus Tumor ◽  
Cranial Cavity ◽  
Content Type ◽  
Suprasellar Region ◽  
Sinus Tumor ◽  
Fine Print

✓ Endodermal sinus tumor (yolk sac carcinoma), a germinal neoplasm, is rarely primary in the cranial cavity. The authors add a third case with the tumor located in the anterior third ventricular (suprasellar) region to the literature. Seventeen previously reported intracranial cases, mostly in the pineal region, are briefly reviewed.

Immunohistochemical study of placental alkaline phosphatase in primary intracranial germ-cell tumors

1985 ◽  
Vol 63 (5) ◽  
pp. 733-739 ◽  
Author(s):  
Jun Shinoda ◽  
Yoshiaki Miwa ◽  
Noboru Sakai ◽  
Hiromu Yamada ◽  
Hiroto Shima ◽  
...  
Keyword(s):  
Alkaline Phosphatase ◽  
Germ Cell ◽  
Tumor Cells ◽  
Embryonal Carcinoma ◽  
Germ Cell Tumors ◽  
Positive Staining ◽  
Placental Alkaline Phosphatase ◽  
Content Type ◽  
Intracranial Germ Cell Tumors ◽  
Fine Print

✓ Indirect immunoperoxidase staining by the peroxidase-antiperoxidase (PAP) technique was carried out on 23 human primary intracranial germ-cell tumors (17 germinomas, one embryonal carcinoma, one yolk-sac tumor, three teratomas, and one teratoma with embryonal carcinoma) and on six human primary pineal nongerm-cell tumors (one pineocytoma, two pineoblastomas, two astrocytomas, and one glioblastoma multiforme). The technique used specific rabbit antisera against placental alkaline phosphatase (PLAP), alpha-fetoprotein (AFP), and human chorionic gonadotropin (HCG). Thirteen of 17 intracranial germinomas (76.5%) showed positive staining for PLAP mainly on the tumor cell membrane. In six primary intracranial non-seminomatous germ-cell tumors, there was weak positive staining indicating the presence of PLAP in only a few cells of one embryonal carcinoma, and in some glandular epithelial cells of one teratoma; this staining was limited to the cytoplasm. None of the other six primary pineal non-germ-cell tumors showed any positive PLAP reaction. From these results, PLAP was shown to be very useful in histopathology as a diagnostic tumor marker of intracranial germinoma. Positive AFP staining was seen in several yolk-sac tumor cells and a few embryonal carcinoma cells. However, no intracranial germinomas and non-germ-cell tumors of the pineal region showed positive reaction. As for HCG, only one suprasellar germinoma and one pineal embryonal carcinoma among 29 specimens contained a few positive-staining tumor cells.

scholarly journals GCT-30. TREATMENT OF PRIMARY INTRACRANIAL GERM CELL TUMORS: SINGLE INSTITUTION EXPERIENCE OF 74 CASES WITHOUT HISTOLOGICAL CONFIRMATION

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii334-iii334
Author(s):  
Chengcheng Guo ◽  
Qunying Yang ◽  
Jian Wang ◽  
Yonggao Mou ◽  
Zhongping Chen
Keyword(s):  
Germ Cell ◽  
Tumor Therapy ◽  
Survival Rates ◽  
Gamma Knife Radiosurgery ◽  
Germ Cell Tumors ◽  
Cancer Center ◽  
Chemotherapy Group ◽  
Tumor Group ◽  
Intracranial Germ Cell Tumors ◽  
Histological Confirmation

Abstract BACKGROUND AND OBJECTIVE Primary intracranial germ cell tumors (PIGCTs) are a group of heterogeneous tumors. It is very difficult to treat those patients without pathological diagnosis. This study retrospectively analyzed the clinical data and outcomes of patients with clinically diagnosed (without histologically confirmed) PIGCTs in SunYat-sen University Cancer Center. METHODS Patients who were clinically diagnosed as PIGCTs without histological diagnosis through surgical resection or biopsy were included in this study. Patients were analyzed for clinical characteristics, treatment patterns, outcomes and adverse effects. RESULTS From May 2002 to July 2014, 74 patients clinically diagnosed with PIGCTs received chemotherapy and/or radiotherapy at the Sun Yat-sen University Cancer Center. The median age was 16.5 years old (4–45 years old, majority was teenagers). The most of tumors were found in male, and located in the pineal and suprasellar regions. When the patients were grouped into diagnostic chemotherapy group (57 cases), diagnostic radiotherapy group (5 cases) and gamma knife radiosurgery group (12 cases) based on their initial anti-tumor therapy. The 5-year survival rates were 84.3%, 75.0% and 75.0%, respectively. There was a trend that the chemotherapy group got a better survival. Patients were allocated to secretory tumor group (49 cases) and non-secretory tumor group (25 cases) based on their levels of tumor makers (α-FP and β-hCG). The 5- year survival rates were 80% and 77.8% (P value = 0.966), respectively. CONCLUSION Clinical diagnosed PIGCT (without histological confirmation) patients may obtain good responses when receiving comprehensive treatments of chemotherapy combined with radiotherapy.

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