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At least 19 recordsLinked to original sources
PubMed1992

The recurrence of primary intracranial germinomas. Special reference to germinoma with STGC (syncytiotrophoblastic giant cell).

Twenty three cases of primary intracranial germinomas including five cases of germinomas with syncytiotrophoblastic giant cells are studied and analyzed, with special reference to the recurrence under radiotherapy. The follow-up period for all cases was 7 months to 12 years (average: 5.8 years) with that for pure germinomas ranging from 8 months to 12 years (average: 5.7 years) and that for germinomas with syncytiotrophoblastic giant cells ranging from 7 months to 11 years (average: 6.3 years). Late recurrence was observed in three cases (3/23, 13%), developing outside of the initial irradiation field. With regard to recurrence, significant correlation to radiation fields was evident, while it was not to radiation doses. Furthermore, germinoma with syncytiotrophoblastic giant cells showed a more significant tendency to recur than pure germinoma. The radiotherapy of germinomas is discussed and the clinical features of germinoma with syncytiotrophoblastic giant cells are presented.

Adolescent↗
PubMed1990

Pineal germinoma with unilateral blindness. Seeding of germinoma cells in optic nerve sheath.

A 13-year-old boy presented with blindness and loss of the papillomacular bundle in the left eye, bilateral papilledema sparing the area of axonal loss, and Parinaud's syndrome. Computerized tomography (CT) revealed an enlarged kinked left optic nerve and enlarged optic canal as well as a tumor in the pineal area producing hydrocephalus. Magnetic resonance (MR) scans showed multiple spinal metastases. The histological diagnosis was germinoma. On completion of four courses of chemotherapy with cis-platin, vinblastine, and bleomycin, repeated CT of orbita and MR imaging of the spine demonstrated the disappearance of the tumor surrounding the left optic nerve and of the spinal metastases. This is believed to be the first case report of seeding of germinoma into the perioptic arachnoid space.

Adolescent↗
PubMed1997

Prognosis of intracranial germinoma with syncytiotrophoblastic giant cells treated by radiation therapy.

PURPOSE: The limited information in the literature suggests that intracranial germinoma with syncytiotrophoblastic giant cells (STGC) producing human chorionic gonadotropin (HCG) is associated with a higher recurrence rate compared with pure germinoma producing no HCG. To determine whether the poorer prognosis for germinoma with STGC is an inevitable finding, we retrospectively analyzed data for our patients. METHODS AND MATERIALS: Data were analyzed for 44 patients who had pretreatment examination of HCG titers in the serum and/or cerebrospinal fluid (CSF) and underwent radiotherapy between 1978 and 1993. The diagnosis of germinoma was made histologically in 19 patients and clinically in 25 according to the established criteria. The treatment volume was the primary tumor site in 9 patients, the cerebrospinal axis in 29, and other in 6. The median follow-up period was 90 months. RESULTS: Twenty (45%) of the 44 patients had elevated HCG titer. The abnormal HCG levels ranged from 4.9 to 189 mIU/ml (median: 18 mIU/ml) in serum and 8.2 to 660 mIU/ml (median: 26 mIU/ml) in CSF. No difference was found between the two groups in any patient or treatment characteristics including tumor size and incidence of CSF dissemination. The mean radiation dose to the primary tumor site was 46.4 Gy for pure germinoma and 47.5 Gy for germinoma with STGC. The 10-year survival and relapse-free survival rates were both 100% for the patients with germinoma with STGC and both 89% for those with pure germinoma. Among these patients, only two with pure germinoma developed recurrence. CONCLUSIONS: Our data suggest that the prognosis of intracranial germinoma with STGC treated with adequate radiation therapy does not differ from that of pure germinoma. Our current policy of delivering 40-45 Gy for tumors < 4 cm in diameter seems to be a reasonable treatment for germinoma with STGC.

Adolescent↗
PubMed1988

Isoenzymes of alkaline phosphatase in germinoma cells.

Utilizing 18 germinomas, the characteristics of isoenzymes of alkaline phosphatase (ALP) in germinoma cells were examined by light and electron microscopic enzymohistochemical and immunohistochemical techniques and biochemical methods including inhibition tests, electrophoresis and electrosyneresis. ALP in germinoma cells was generally located on the cell surface. In a few germinoma cells, however, ALP was seen not only on the cell membrane but also in the endoplasmic reticulum. Enzymohistochemically as well as biochemically, ALP in germinoma cells was little sensitive to L-phenylalanine, moderately sensitive to L-homoarginine and somewhat resistant to heat in its inhibition tests. Immunohistochemical examinations and electrosyneresis showed the presence of the placental type of ALP in germinoma cells and biochemical analyses revealed that the heat-stable component of ALP in germinoma cells was consistent with the D-variant of the placental type of ALP. Therefore, germinoma cells possessed mixed isoenzymes of ALP, consisting mainly of the liver/bone type and a small amount of D-variant of the placental type on the cell surface. The prominent expression of ALP in germinoma cells may be due to the enhanced expression of gonadal genes active in the germinoma genome.

Adult↗
PubMed1989

Suprasellar germinomas in childhood. A reappraisal.

Germinomas in childhood may arise in both the suprasellar and pineal region, and outcome has been reported to be worse for suprasellar germinomas with a 5-year survival rate of 20% as compared to 60% for pineal germinoma. To determine the factors impacting on outcome, the results of a uniform treatment approach were evaluated and included primary surgical debulking (PSD) and systemic craniospinal axis radiation (CSRT) for suprasellar germinomas. Between 1976 and 1985 ten consecutive patients (seven females, three males) with the pathologically confirmed diagnosis of suprasellar germinoma were treated. Outcome was compared to four male patients with pineal germinoma treated over the same time period and series of patients reported in the literature. At diagnosis the mean age of patients with suprasellar germinoma was 13.9 years (range, 8.9 to 9.4 years). Symptoms were present for a mean of 18 months (range, 2 to 72 months) prior to diagnosis and included diabetes insipidus, anterior pituitary dysfunction, decreased vision, headache, vomiting, and diplopia. Staging studies, including myelography (n = 4) and cerebrospinal fluid cytology (n = 7), disclosed dissemination in only one child. Surgical treatment included biopsy in three cases, partial resection in five, and total resection in two; no permanent postsurgical complications were noted. The mean radiation therapy dose to the tumor site was 4953 cGy (range, 4400 to 5250 cGy) and to the spine 3354 cGy (range, 3000 to 4000 cGy). Patients were followed for a mean period of 5.1 years (range, 1.9 to 10.5 years). One patient with SG who did not receive treatment initially developed a pineal tumor after diagnosis; she was treated with PSD and CSRT and is asymptomatic 5 years later. All the remaining patients are alive and remain disease-free. Surgical resection and CSRT results in excellent disease control for children with suprasellar germinomas, and outcome is similar to those patients with pineal germinoma.

Adolescent↗
PubMed1997

Intracranial germinoma: the case for lower dose radiation therapy.

PURPOSE: To determine the optimal dose and treatment outcome of patients treated with radiation for intracranial germinoma. METHODS AND MATERIALS: Between 1975 and 1995, 40 patients with the diagnosis of intracranial germinoma were treated with radiation (RT) to the central nervous system. All patients received whole-brain (WB) RT (median dose: 32.4 Gy, range: 15-44.37 Gy) and a boost to the tumor volume (median total tumor volume dose: 52 Gy, range: 45-59.5 Gy). Thirty patients received RT to the spine (median dose: 26 Gy, range: 18.75-37.5 Gy). Four patients were treated with cisplatin-based chemotherapy and WB RT with a boost to the tumor volume (dose range: 51-54 Gy). A low-dose RT only group was defined as < or = 25.5 Gy to the WB (9 patients); < 50 Gy to the primary site (14 patients); and < 22 Gy to the spine (9 patients). Seventeen tumors were biopsy-proven germinoma, and 17 patients presented with multiple midline germinomas (MMG). Among 26 patients who had tumor markers measured, 27% had elevation of beta-human chorionic gonadotropin and by definition, no patient had an elevation of AFP. Twenty-four percent of 26 patients who had spine imaging or cerebral spinal fluid cytology had evidence of tumor seeding at diagnosis. The male to female ratio was 1.9:1. Median age at diagnosis was 14 years for male patients and 9.5 years for female patients (p = 0.02), (overall age ranges: 0.5-31 years). Median follow-up was 62 months (range: 3-226 months). Late effects of 29 patients with follow-up of > or = 20 months and adequate documentation in their medical records were analyzed. RESULTS: The 5-year actuarial rate of disease-free survival (DFS) and overall survival (OS) for biopsy-proven germinomas and presumed germinomas was 97%. No patient died of germinoma. There were no local failures regardless of the dose of RT, elevation of HCG tumor marker, or CSF dissemination at presentation. At presentation 22 patients had evidence of at least one endocrine abnormality. At follow-up there were no new patients diagnosed with an endocrine abnormality; however, 13 out of 22 patients had an increase in the number of endocrine deficiencies requiring hormone replacement. At presentation, 14 patients showed evidence of growth retardation. At follow-up there were no new cases of growth failure in the remaining patients. CONCLUSIONS: Germinomas are highly curable with RT alone. Lower doses of RT to the craniospinal axis without chemotherapy appear to produce equally effective DFS and OS as do higher doses of RT or combination chemotherapy and RT. Craniospinal RT may be indicated for patients with MMG or patients with evidence of spinal seeding. Long-term effects of growth retardation, and other endocrine deficiencies appear to be correlated with disease at presentation rather than solely with treatment.

Adolescent↗
PubMed1985

Intranuclear membranous profiles in germinoma cells--a variant of nuclear pockets and intranuclear annulate lamellae.

When ultrastructurally examining 24 germinomas comprising 12 seminomas, 4 dysgerminomas, 1 mediastinal germinoma, and 7 intracranial germinomas, intranuclear membranous profiles were noticed in 17 germinomas, ranging from 20-100 nm in width and 3 microns in length. With occasional connections to the nuclear envelope through a small hole, intranuclear membranous profiles in germinoma cells were considered as clefts of the nuclear envelope. While most frequently situated under the inner nuclear membrane, they varied in configuration as well as distribution. As sequestered round mass of nuclear material, they were a variant of nuclear pockets containing nucleoplasm. Intranuclear annulate lamellae were occasionally present apart from the nuclear envelope and connected with nuclear clefts. Eleven of the twelve seminomas and 6 of the twelve non-seminomatous germinomas showed intranuclear membranous profiles, and the incidence of such profiles was much higher in seminomas than in non-seminomatous germinomas. Intranuclear membranous profiles facing the inner nuclear membrane were also noted in spermatogonia in adolescents and adults. It was suggested that intranuclear membranous profiles in germinoma cells could be structures following ones occasionally seen in spermatogonia.

Adolescent↗
PubMed1991

Magnetic resonance imaging of neurohypophyseal germinomas.

The authors reviewed magnetic resonance (MR) images in seven cases of germinoma in the hypothalamoneurohypophyseal axis (HNA). The intrasellar portions were clearly identified in six germinomas. Two small germinomas of these six were located only in the neurohypophysis. The major parts of the four large germinomas were located below the optic chiasm, and the large intrasellar portions were demonstrated. The remaining one small germinoma was localized from the pituitary stalk to the third ventricular floor. These findings strongly suggest that the primary site of germinomas in the HNA is the neurohypophysis. In the four large germinomas, the tumor shape was similar to that of pituitary adenoma. The authors believe that age (limited to first three decades), symptoms (diabetes insipidus), MR findings (absence of normal hyperintense signal of the posterior pituitary on T1-weighted (T1WI) images, and homogeneous hypointensity to the pons on T1WI images/isointensity on T2-weighted images are important in differential diagnosis.

Adolescent↗
PubMed1997

Analysis of long-term treatment of intracranial germinoma.

PURPOSE: To determine an appropriate treatment policy for intracranial germinoma. METHODS AND MATERIALS: From 1976 to 1992, 51 patients with germinoma (18 with pathologically verified germinoma and 33 diagnosed as having germinoma by neuroimagings) were treated by radiation therapy. Various radiation doses and treatment fields were used. RESULTS: The 10-year cause-specific survival rate for pathologically verified and unverified germinoma was 100% and 96%, respectively. Relapses were noted in four patients, three of whom died from the disease. Two of the four patients with a tumor larger than 4 cm in gross diameter experienced relapse. Two relapses occurred in a nonirradiated spinal canal and two occurred in the irradiation area treated by 25 Gy in 10 fractions and 30 Gy in 20 fractions. No relapse was noted in patients in whom the whole ventricle field was determined precisely using three-dimensional treatment planning. Five patients who were followed at an outpatient clinic experienced significant late neurocognitive dysfunction, which set in after radiotherapy. CONCLUSION: After pathological confirmation, 40 Gy whole-ventricle irradiation using precise three-dimensional treatment planning is appropriate as a standard treatment for most intracranial germinoma.

Adolescent↗
PubMed1995

Aromatase expression in human germinomas with possible biological effects.

Gonadal aromatase expression has been demonstrated in human Leydig, granulosa, and thecal cells, but never in human germ cells. In an attempt to explain the unique occurrence of isosexual precocious puberty in a young girl with a hCG-secreting suprasellar germinoma, we demonstrated the presence of aromatase expression in the germ cell component of this tumor. Immunohistochemical staining for P450-aromatase and hCG using a peroxidase-labeled streptaviden-biotin technique was performed on tumor specimens from the above patient and from four other subjects with central nervous system germinoma. Cytoplasmic aromatase staining was present in the germinoma cells of four of five cases of central nervous system germinoma studied. Staining was absent in the lymphocytic element within the tumor and in negative control tissues. The demonstration of aromatase activity in the malignant element of human germinomas indicates that aromatase expression can occur in human germ cells after malignant transformation. This parallels the finding that the transformation of Sertoli cells to sex cord tumor with annular tubules in Peutz Jeghers syndrome is associated with the induction of marked aromatase expression and systemic estrogen effect. We propose that tumor aromatase played a similar role in the unique occurrence of isosexual precocity in a girl with a suprasellar germinoma.

Aromatase↗
PubMed1997

Management of primary intracranial germinomas: diagnostic surgery or radical resection?

Because intracranial germinomas are readily curable with radiation and chemotherapy or radiation therapy alone, the role of radical surgery has become debatable. This study assesses the optimum degree of surgical resection for intracranial germinomas. Twenty-nine patients who underwent surgery for germinoma were retrospectively analyzed (male/female ratio 27:2, median age 18 years). Among these 29 patients there were 10 solitary pineal, seven solitary neurohypophyseal/hypothalamic, and 12 multifocal or disseminated tumors. Biopsy samples were obtained in 16 patients (stereotactically in eight, transsphenoidally in four, and via frontotemporal craniotomy in four). Partial resection was attained in five patients (via a frontotemporal approach in three and occipitotranstentorially in two). Gross-total resection was achieved via an occipitotranstentorial route in eight patients with pineal masses. After surgery, 10 patients were treated with radiotherapy alone, and 19 received radiation and chemotherapy; complete remission was achieved in all 29 patients. The overall tumor-free survival rate was 100% at a median follow-up period of 42 months. There was no significant difference in outcome related to the extent of surgical resection. Postoperative neurological improvement was seen in only two patients, whereas transient postoperative complications, mainly upgaze palsy, were observed in six. One patient experienced a slight hemiparesis, bringing the surgical morbidity rate to 3% (one of 29). It is concluded that radical resection of intracranial germinomas offers no benefit over biopsy. The primary goal of surgery should be to obtain a sufficient volume of tumor tissue for histological examination. If there is strong evidence of germinoma on radiological studies, biopsy samples should be obtained. When a perioperative histological diagnosis of pure germinoma is made during craniotomy, no risk should be taken in continuing the resection.

Adolescent↗
PubMed1998

Induction chemotherapy followed by reduced-volume radiation therapy for newly diagnosed central nervous system germinoma.

OBJECT: Although curative, radiation, which is conventionally administered for germinomas, causes significant neurological sequelae. This study aimed at reducing the volume and dose of radiation to a localized level of 24 Gy by pretreating the patient with chemotherapy. METHODS: Seventeen patients were divided into two risk groups based on serological findings and the extent of tumor. They were treated with chemotherapy prior to receiving localized radiation therapy. Six patients with solitary pure germinomas were treated with three or four cycles of cisplatin and etoposide (EP regimen) followed by 24-Gy local radiation therapy. Eleven patients with human chorionic gonadotropin (HCG)-secreting, multifocal, or disseminated germinomas received four to five cycles of ifosfamide, cisplatin, and etoposide (ICE regimen) followed by 24-Gy local radiation therapy. Craniospinal ports were used only in three cases of germinomas with dissemination. Gross-total resection was performed in three patients. Fourteen patients were able to be evaluated for their responses to chemotherapy. All patients achieved a complete response within three cycles. At a median follow-up duration of 24 months, 16 patients (94%) were alive without recurrence. One patient with an HCG-secreting germinoma experienced recurrence 38 months after surgery. That patient underwent successful salvage therapy using the same protocol. Thus, all 17 patients became free of disease with a 70 to 100% Karnofsky Performance Scale status. Toxicities associated with this study's chemotherapy regimen were mostly transient. No patient showed neurological or endocrinological deterioration during the follow-up period. CONCLUSIONS: The EP and ICE regimens were highly effective in treating the central nervous system germinomas and permitted dose and volume reduction of the radiotherapy. Localized 24-Gy irradiation was sufficient for disease control.

Adolescent↗
PubMed1993

Histochemistry with Helix pomatia agglutinin in human germ cell tumors: detection of nongerminomatous components and correlation between HPA reactivity and radiosensitivity in germinomas.

Binding sites of Helix pomatia agglutinin (HPA) were examined in 32 patients with intracranial human germ cell tumors. HPA reactivity was found in vascular endothelial cells and erythrocytes of patients with blood type A or AB. HPA-positive neoplastic cells were seen in one yolk sac carcinoma in a patient with blood group A, and in embryonal carcinomas and teratomas irrespective of blood group type. Although in 10 out of 18 germinomas neoplastic cells were totally negative for HPA, another 8 germinomas showed HPA-positive neoplastic cells which were distributed sporadically or in an area and independent of blood group types. HPA-negative germinoma patients showed a very good response to radiotherapy, whereas 4 out of 8 HPA-positive tumors showed poor radiosensitivity, with a residual lesion seen on computed tomography even after the total radiation dose of 40-50 Gy. These findings suggest that HPA-positive neoplastic cells in germinomas indicate components of differentiation of non-germinomatous germ cells. HPA-positive germinomas might be less radiosensitive than HPA-negative germinomas.

ABO Blood-Group System↗
PubMed1990

Radiation therapy for intracranial germinoma: the case for limited volume treatment.

Although radiation therapy has been universally accepted as the treatment of choice for primary intracranial germinomas, the optimal treatment technique continues to be a matter of controversy. Some authors advocate routine prophylactic craniospinal irradiation for all patients with localized intracranial germinomas whereas others have recommended partial brain fields. A retrospective analysis was performed on all 30 patients with tumors of the pineal and suprasellar regions irradiated at our institution between 1967 and 1987. Definitive histology was obtained in 23 patients. There were 13 germinomas and 10 non-germinomatous germ cell tumors. The 13 patients with biopsy proven germinomas constitute one of the largest modern series and will form the basis of this report. In all 13 patients, the tumor was confined to a single site within either the pineal region (9 patients) or the suprasellar region (4 patients). One patient with a pineal germinoma had CSF cytology positive for malignant cells and this patient was treated with craniospinal irradiation. Twelve patients were treated using partial brain fields encompassing either the tumor plus margin (10 patients) or the entire ventricular system followed by a boost to the primary tumor (2 patients). With the exception of one patient, all patients received a total dose between 4900 and 5500 cGy to the primary (median 5040 cGy). The patient receiving less (3960 cGy) suffered from both local and spine relapse 26 months after diagnosis. The remaining 12 patients were free of disease at 81 months median follow-up time. We suggest that for patients with biopsy proven pineal or suprasellar germinomas, irradiation of partial brain is sufficient and craniospinal irradiation should be administered only to those patients with disease involving more than one intracranial site, demonstrated meningeal seeding, or positive CSF cytology.

Adolescent↗
PubMed1985

Idiopathic growth hormone (GH) deficiency, and GH deficiency secondary to hypothalamic germinoma: effect of single and repeated administration of human GH-releasing factor (hGRF) on plasma GH level and endogenous hGRF-like immunoreactivity level in cerebrospinal fluid.

Plasma GH responses to iv administered synthetic human GH-releasing factor-(1-44)-NH2 (hGRF) and the concentration of endogenous hGRF-like immunoreactivity (hGRF-LI) in the cerebrospinal fluid (CSF) were examined in 16 children with GH deficiency (GHD). Ten patients had idiopathic GHD, and six had GHD secondary to germinoma. An iv bolus hGRF (1 microgram/kg BW) injection test was performed the day before and the day after treatment, with a daily 1-h iv infusion of hGRF (2 micrograms/kg BW) for 3 days. Plasma GH increases (greater than 5 ng/ml) after the first iv bolus injection of hGRF occurred in 2 of the 10 idiopathic GHD children and in 4 of the 6 GHD patients with germinoma whereas the first bolus hGRF injection failed to elicit GH release in the remaining 10 patients. The mean +/- SEM peak plasma GH level after the first bolus hGRF dose in the patients with germinoma (8.2 +/- 2.2 ng/ml) was significantly higher than that in the idiopathic GHD patients (2.9 +/- 0.9 ng/ml; P less than 0.05), but significantly lower than that in normal children with short stature (18.5 +/- 2.5 ng/ml; P less than 0.05). In the 2 patients with germinoma and in 5 of the 8 idiopathic GHD children who did not respond to the first hGRF bolus dose, a significant plasma GH response to hGRF occurred during a daily iv infusion of hGRF for 3 consecutive days, whereas the remaining 3 idiopathic GHD children failed to respond to the daily hGRF infusions. The plasma GH response after the second hGRF bolus dose given after treatment with daily hGRF infusions for 3 days was not different from that after the first hGRF bolus in patients with germinoma or that in the idiopathic GHD children. hGRF-LI was not detected (less than 5.8 pg/ml) in the CSF in any of 5 patients with germinoma, whereas it was present in 5 idiopathic GHD patients (mean, 17.5 +/- 0.9 pg/ml), 3 of whom were nonresponders to daily hGRF infusions. From these results, GHD secondary to destruction of hypothalamic GRF neurons might be defined by the following findings: 1) lack of a GH response to the standard provocative tests acting through the hypothalamus; 2) significant increase in plasma GH after a single bolus and/or repetitive iv administration of hGRF; and 3) undetectable or extremely low levels of endogenous hGRF-LI in the CSF. Most of the idiopathic GHD patients responded to the repetitive hGRF infusion, suggesting insufficient secretion of hypothalamic hGRF as the primary defect. However, since hGRF-LI was detectable in the CSF in some of the idiopathic GHD patients, its pathogenesis must be multifactorial.

Adolescent↗
PubMed1978

Suprasellar germinoma.

The clinical features of 18 cases of suprasellar germinoma were reviewed, and the following points stressed: 1) The incidence of intracranial germinoma and teratoma seems to be much higher in Japan than elsewhere. 2) Germinomas in the pineal region, the third ventricle, and the lateral ventricle were observed to be predominant in males. In suprasellar germinoma, however, males were not affected predominantly and the male:female ratio was 1:1. 3) Suprasellar germinomas caused three symptoms: diabetes insipidus, visual disturbances, and pituitary dysfunctions. 4) Pituitary functions were disturbed to various degrees. Some cases showed hypopituitarism but some showed an elevation of plasma concentrations of cortisol or luteinizing hormone and follicle stimulating hormone. 5) Some abnormalities showing a suprasellar mass were usually found on neuroradiological examination. 6) Suprasellar germinomas were very sensitive to radiotherapy and some of them rapidly disappeared on follow-up computerized tomography scanning after irradiation with as little as 1200 rads.

Adolescent↗