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

✓ 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.

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Pathogenesis of intracranial germ cell tumors reconsidered

Journal of Neurosurgery ◽

10.3171/jns.1999.90.2.0258 ◽

1999 ◽

Vol 90 (2) ◽

pp. 258-264 ◽

Cited By ~ 108

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.

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Intracranial germ-cell tumors: natural history and pathogenesis

Journal of Neurosurgery ◽

10.3171/jns.1985.63.2.0155 ◽

1985 ◽

Vol 63 (2) ◽

pp. 155-167 ◽

Cited By ~ 524

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.

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

Journal of Neurosurgery ◽

10.3171/jns.1985.63.5.0733 ◽

1985 ◽

Vol 63 (5) ◽

pp. 733-739 ◽

Cited By ~ 42

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.

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

Neuro-Oncology ◽

10.1093/neuonc/noaa222.250 ◽

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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Radiation therapy for intracranial germ cell tumors: Predictive value of tumor response as evaluated by computed tomography

International Journal of Clinical Oncology ◽

10.1007/bf02488875 ◽

1997 ◽

Vol 2 (2) ◽

pp. 67-72 ◽

Cited By ~ 3

Author(s):

Kazuhiko Ogawa ◽

Takafumi Toita ◽

Yasumasa Kakinohana ◽

Keiichiro Yamaguchi ◽

Koichi Miyagi ◽

...

Keyword(s):

Computed Tomography ◽

Radiation Therapy ◽

Germ Cell ◽

Predictive Value ◽

Tumor Response ◽

Germ Cell Tumors ◽

Intracranial Germ Cell Tumors

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End Results of Surgical Treatment of 622 Cases of Tumors of the Salivary Glands.

Tumori Journal ◽

10.1177/030089167406000406 ◽

1974 ◽

Vol 60 (4) ◽

pp. 307-316 ◽

Cited By ~ 9

Author(s):

Carmelo Vinicio Catania ◽

Emanuele Galante ◽

Gaetano Bandieramonte ◽

Bruno Salvadori

Keyword(s):

Salivary Glands ◽

Radical Surgery ◽

Malignant Tumors ◽

Survival Rates ◽

High Rate ◽

Surgical Removal ◽

Minor Salivary Glands ◽

Benign Lesions ◽

Submandibular Glands ◽

Mixed Tumors

Surgery was performed on 622 patients with tumors of the salivary glands from 1929 to 1972; the gland affected was the parotid in 527 cases, the submandibular glands in 50, and minor salivary glands of palate and other sites in 45. The analysis of distant results point out that both for benign and malignant tumors total sialoadenectomy must be the treatment of choice, as demonstrated by the high rate of recurrence after local excision or partial resection of the gland, even in cases of surely benign lesions such as mixed tumors. It is worthy of being quoted the high rate of recurrence from cylindromas, especially of the palate (48.5%), after surgical removal. As to malignancies of parotid and submandibular glands 5-years survival rates after radical surgery were of 52 % and 30 % respectively.

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Radiation Therapy for Intracranial Germ Cell Tumors

Progress in Neurological Surgery - Pineal Region Tumors ◽

10.1159/000210056 ◽

2009 ◽

pp. 96-105 ◽

Cited By ~ 9

Author(s):

Hidefumi Aoyama

Keyword(s):

Radiation Therapy ◽

Germ Cell ◽

Germ Cell Tumors ◽

Intracranial Germ Cell Tumors

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Precocious puberty in a girl with an hCG-secreting suprasellar immature teratoma

Journal of Neurosurgery ◽

10.3171/jns.1994.81.4.0601 ◽

1994 ◽

Vol 81 (4) ◽

pp. 601-604 ◽

Cited By ~ 13

Author(s):

Chifumi Kitanaka ◽

Masao Matsutani ◽

Shigeo Sora ◽

Sachiko Kitanaka ◽

Ayako Tanae ◽

...

Keyword(s):

Brain Tumors ◽

Precocious Puberty ◽

Germ Cell Tumors ◽

Immature Teratoma ◽

Content Type ◽

Intracranial Germ Cell Tumors ◽

Hcg Secretion ◽

High Level ◽

Very High ◽

Fine Print

✓ Although precocious puberty is common in boys with human chorionic gonadotropin (hCG)-secreting brain tumors, it is extremely rare in girls. The authors describe a 6-year-old girl with an hCG-secreting suprasellar immature teratoma who presented with diabetes insipidus, increased intracranial pressure, and precocious puberty. On admission, breast budding was observed. The serum hCG level was 1230 mIU/ml. Both luteinizing hormone (LH) and follicle-stimulating hormone (FSH) remained below detectable levels, even after gonadotropin-releasing hormone stimulation. Serum estrogen and androgen were moderately elevated. After chemotherapy, breast budding disappeared with normalization of serum hCG. It has been believed that hCG does not produce precocious puberty in girls in the absence of FSH, and this has been used as an explanation for the rarity of precocious puberty in girls with hCG-secreting brain tumors. However, it has also been reported that hCG has not only LH activity but also intrinsic, although weak, FSH-like activity. In the present case, this FSH-like activity was considered to have played a role in the development of precocious puberty. It is speculated that a very high level of serum hCG can produce precocious puberty in girls. The rarity of intracranial germ-cell tumors with a high potential of hCG secretion may be one of the reasons why hCG-induced precocious puberty is uncommon in girls.

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Combination chemotherapy with cisplatin and etoposide for malignant intracranial germ-cell tumors

Journal of Neurosurgery ◽

10.3171/jns.1989.70.5.0676 ◽

1989 ◽

Vol 70 (5) ◽

pp. 676-681 ◽

Cited By ~ 71

Author(s):

Tatsuya Kobayashi ◽

Jun Yoshida ◽

Junzo Ishiyama ◽

Satoshi Noda ◽

Akira Kito ◽

...

Keyword(s):

Germ Cell ◽

Combination Chemotherapy ◽

Germ Cell Tumors ◽

Intracranial Germ Cell Tumor ◽

Content Type ◽

Total Response Rate ◽

Intracranial Germ Cell Tumors ◽

Dna Histograms

✓ Antitumor activity against intracranial malignant teratoma by combination chemotherapy with cisplatin and etoposide was evaluated in experimental and clinical studies. A human teratoma cell line (Tera 2) was exposed in vitro to cisplatin and/or etoposide, after which cell growth inhibition and alterations of deoxyribonucleic acid (DNA) histograms were observed. The results indicated that a synergistic cytotoxic effect was achieved by use of both agents in combination. Four cases of recurrent intracranial germ-cell tumor (three malignant teratomas and one germinoma) were treated with cisplatin and etoposide. With this combinationtherapy, regression of the tumor was observed in all four cases (three complete and one partial), for a total response rate of 100%. During a follow-up period of 9 to 22 months, no recurrence or progression has been noted in three of these cases.

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