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.

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 P14.50 Clinicopathological spectrum of intracranial germ cell tumors: an Indian tertiary cancer center experience

2019 ◽  
Vol 21 (Supplement_3) ◽  
pp. iii78-iii78
Author(s):  
A Sahay ◽  
G Chinnaswamy ◽  
V Bhat ◽  
V Patil ◽  
T Gupta ◽  
...  
Keyword(s):  
Brain Tumors ◽  
Germ Cell ◽  
Tumor Markers ◽  
Posterior Fossa ◽  
Germ Cell Tumors ◽  
Clinicopathological Features ◽  
Spinal Tumors ◽  
Equal Distribution ◽  
Pineal Tumors ◽  
Intracranial Germ Cell Tumors

Abstract BACKGROUND Incidence of intracranial germ cell tumors (ICGCT) in Western literature is low (0.3–0.6 %) as compared to East Asia (3–4 %), & their clinicopathological features are well documented. However, there are scant studies on ICGCT from India. MATERIAL AND METHODS Retrospective observational study of all ICGCT histologically diagnosed in our hospital from 2007–2018. Metastasis were excluded. Clinicopathological features were retrieved from hospital’s electronic medical records. RESULTS We diagnosed 82 primary ICGCT, forming approx. 0.54 % of all primary brain tumors, & 3.5% of pediatric brain tumors. Age range: 2 months-32 yrs (Median age 14 yrs). M:F ratio: 1.82:1 (53M,29F). Nearly 80% patients were pediatric (<18 yrs), & 8 very young (<3 yrs, 7M1F). Majority were suprasellar & pineal (31/82, 37% each), with one bifocal presentation. Other rarer sites: posterior fossa (4), midbrain (1), corpus callosum (1) & 11 non midline (4 thalamic, 4 frontal, 2 cerebellar, 1 CP angle). Predominant histology was germinoma (G) (51/82, 62%), while non germinomatous (NGGCT) were 31/82 (38%), of which 9 were mixed. Pure teratoma were 11 (9 immature (IT), 2 mature), & 5 pure yolk sac tumor (YST). Interestingly, all very young age group patients (<3 yrs), showed only NGGCT histology (5/8 IT, 3/8 pure YST). In contrast, G histology formed nearly 70% of all patients >3 yrs. Females were associated mainly with G (21/29, 72%). NGGCT were predominantly seen in males (M:F=2.9:1). Also, pure IT (9) were seen only in males. Posterior fossa tumors were all IT (4/4). Spinal tumors were NGGCT (1 mature teratoma, 1 YST). Majority of suprasellar tumors (25/31, 80.6%), other midline locations like corpus callosal, midbrain, & all thalamic tumors were G. However, pineal tumors showed equal distribution of G (15/31) & NGGCT (16/31). Spine screening was positive in 8 patients (6 G, 2 IT)- 7 on MRI and 1 only on CSF cytology. Serum tumor markers were raised in 13/54 cases- 6/34G(17.6%), vs 7/20 NGGCT (35%). CSF tumor markers were raised in 14/34-10/23 G (43.4%), vs 4/11 NGGCT (36.3%). Follow up was available for 37 patients (Duration 3 months-10 yrs, median 2 yrs). On f/u 6/15 (40%) NGGCT showed progression/death, while only 2/32 G relapsed (6.5%). Four deaths in G group were not directly attributable to the tumor. CONCLUSION Frequency of ICGCT in our hospital similar to western data rather than Asian, albeit with less striking male preponderance. ICGCT were tumors of 2nd decade & majority occurred in pineal/suprasellar areas. About 2/3rd were pure G on histology, and showed good prognosis. NGGCT were common in infants, males and in posterior fossa. IT were seen exclusively in males and pure YST mainly in males. Although majority of ICGCT are in midline, rarely non midline involvement also occurs, and it’s essential to exclude metastasis before considering primary ICGCT. Expectedly, NGGCT showed poorer prognosis, compared to pure germinomas.

scholarly journals Blood to cerebrospinal fluid human chorionic gonadotropin-beta ratios in intracranial germ cell tumors

1998 ◽  
Vol 5 (1) ◽  
pp. E6 ◽  
Author(s):  
Paul B. Rogers ◽  
Eliot C. Sims ◽  
Nicholas Plowman
Keyword(s):  
Cerebrospinal Fluid ◽  
Germ Cell ◽  
Chorionic Gonadotropin ◽  
Human Chorionic Gonadotropin ◽  
Germ Cell Tumors ◽  
Intrathecal Administration ◽  
Pineal Region ◽  
Elevated Blood ◽  
Intracranial Germ Cell Tumors ◽  
Human Chorionic Gonadotropin Beta

Levels of human chorionic gonadotropin-beta (HCG-beta) are elevated in up to 43% of patients with intracranial germ cell tumors (GCTs) and are useful in the diagnosis of these tumors and the follow up of such patients. The ratio of blood HCG-beta to lumbar cerebrospinal fluid (CSF) HCG-beta in these patients at presentation has not been defined. Twenty-two patients with intracranial GCTs have been treated at St. Bartholomew's Hospital over the past 15 years. Two (17%) of 12 germinomas and seven (70%) of 10 nongerminomatous GCTs had elevated blood HCG-beta at presentation. Four cases of pineal region GCTs (one of 12 germinomas and three of 10 nongerminomatous GCTs) had paired, elevated, blood and lumbar CSF HCG-beta levels. The mean blood to CSF ratio was 1:10 (range 1.7-18.4), which is substantially lower than the ratio of 286:1 reported in systemic GCTs. The authors confirm the finding of a previous single report showing that ventricular CSF HCG-beta sampling via an accessible ventriculoperitoneal shunt reservoir may give a spuriously negative result, and they discuss the pathophysiology of the blood-brain barrier in the pineal region and the implications of the intrathecal administration of chemotherapy.

Diagnosis and management of pineal tumors

1983 ◽  
Vol 58 (5) ◽  
pp. 654-665 ◽  
Author(s):  
Rashid Jooma ◽  
Brian E. Kendall
Keyword(s):  
Tumor Markers ◽  
Germ Cell Tumors ◽  
Ct Scanning ◽  
Individual Case ◽  
Pineal Region ◽  
Pineal Tumors ◽  
Content Type ◽  
Pineal Region Tumors ◽  
Primary Surgery ◽  
Fine Print

✓ The management of pineal region tumors remains controversial. Advocates of a conservative approach emphasize the excellent results of radiotherapy, particularly with germinomas, while a number of recent reports have demonstrated the safety of direct surgery. In order to improve treatment planning, attempts have been made to distinguish the various tumor types by computerized tomography (CT) and by the use of markers for germ-cell tumors. This paper reports a study of 35 patients with pineal tumors including two ectopic germinomas. Pretreatment CT was performed in all 35 patients, and human chorionic gonadotropin and alpha-fetoprotein levels were assayed in 11. Histological verification was available for 33 tumors; the other two were characterized by clinical and CT features plus tumor markers. Of the 27 tumor operations, 21 were for a pineal mass and six for ectopic germinoma or metastasis. There was no operative mortality. Morbidity was minimal, and surgery did not increase the incidence of subarachnoid seeding. Each histological type of tumor has a typical appearance on CT scanning, although, in the individual case, a firm diagnosis is not always possible. However, consideration of CT scans together with the clinical features, cerebrospinal fluid cytology, tumor markers, and (if there is still doubt) response to a small dose of irradiation will generally allow a specific diagnosis with a high degree of probability. In this way, germinomas may be selected for radiotherapy and the tumors less likely to respond may be subjected to primary surgery.

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

1989 ◽  
Vol 70 (5) ◽  
pp. 676-681 ◽  
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.

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.

scholarly journals Right occipital transtentorial approach for a pineal malignant germ cell tumor

2021 ◽  
Vol 5 (1) ◽  
pp. V3
Author(s):  
Brian J. A. Gill ◽  
Dominique M. Higgins ◽  
Matei A. Banu ◽  
Michael G. Argenziano ◽  
Neil A. Feldstein ◽  
...  
Keyword(s):  
Germ Cell ◽  
Germ Cell Tumors ◽  
Pineal Region ◽  
First Line ◽  
Intracranial Germ Cell Tumors ◽  
Somatic Transformation ◽  
Malignant Germ Cell Tumor ◽  
Occipital Transtentorial Approach ◽  
Malignant Lesions ◽  
Transtentorial Approach

Germ cell tumors account for up to 53% of the malignant lesions found in the pineal region and are typically managed with a combination of radiation therapy and chemotherapy. Malignant somatic transformation of intracranial germ cell tumors is exceedingly rare and has only been reported on two other occasions. Here the authors present the case of a pineal yolk sac tumor that failed optimum first-line treatment and underwent malignant somatic transformation to an enteric mucinous adenocarcinoma requiring surgical intervention. This video demonstrates the technical nuances of the occipital transtentorial approach and the safe microsurgical dissection of lesions within the pineal region. The video can be found here: https://stream.cadmore.media/r10.3171/2021.4.FOCVID2151.

scholarly journals RTHP-31. EMERGENCY RADIOTHERAPY IN INTRACRANIAL GERM CELL TUMORS (GCTS) PATIENTS WITH KPS≤ 40: ARETROSPECTIVE ANALYSIS OF 27 CASES

2019 ◽  
Vol 21 (Supplement_6) ◽  
pp. vi216-vi216
Author(s):  
Linbo Cai ◽  
Mingyao Lai ◽  
Juan Li ◽  
Cheng Zhou ◽  
Qingjun Hu ◽  
...  
Keyword(s):  
Overall Survival ◽  
Survival Rate ◽  
Germ Cell ◽  
Germ Cell Tumors ◽  
Progression Free Survival ◽  
Intracranial Germ Cell Tumors ◽  
Direct Cell ◽  
Emergency Radiotherapy

Abstract OBJECTIVES To evaluate the potential role of emergency radiotherapy in intracranial germ cell tumors GCTs) patients with KPS ≤ 40. METHODS A total of 27 primary intracranial germ cell tumors (GCTs) patients with KPS ≤ 40 between Jan 2007 and Dec 2018 were retrospectively evaluated. The median age at initial diagnosis was 15 years (range, 528 years). Among those, 11 patients were germinoma and 16 patients were nonseminomatous germ-cell tumors (NGGCTs). There were 9 solitary pineal, 5 suprasellar, 3 basal ganglia and 10 multifocal and disseminated tumors. All patients received emergency radiotherapy (2 Gy/fx/d). Prior to radiotherapy, 11 patients were manifested with hydrocephalus, 10 with hypopituitarism and 5 with intracranial tumo apoplexy. RESULTS The average follow up time was 44.4 months. The 5 year progression free survival rate and overall survival rate were 29.6% and 33.3%. The median overall survival time was 38 months. In particular, the median intracranial hypertension symptoms relief time was 2 days. The median KPS following radiotherapy was 80 comparing to 30 prior to radiotherapy (P < 0.05). A significant improvement on KPS of 46.7±27.3 was observed in this study. CONCLUSION Emergency radiotherapy is implicated as a promising intervention for GCTs patients with elevated intracranial pressure (ICP). These advantages can be interpreted as direct cell killing effect and fast tumor shrinkage by ionizing radiation. However, to substantiate our findings, further investigations were highly warranted.

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.

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.

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