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1. Cheon HC, Jung S, Moon KS, Lee MC, Kim IY, Jung TY, Kim SH, Kang SS: Primary endodermal sinus tumor of the cerebellar hemisphere: a case report with review of the literature. J Neurooncol; 2006 Apr;77(2):173-6
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  • [Title] Primary endodermal sinus tumor of the cerebellar hemisphere: a case report with review of the literature.
  • Primary intracranial endodermal sinus tumors, which have been regarded as a rare histologic subtype, usually arise in the pineal and suprasellar regions and are often associated with components of other germ cell tumors.
  • We report an extremely rare case of pure primary endodermal sinus tumor found in the cerebellar hemisphere.
  • Radical surgical removal of the tumor was performed, followed by adjuvant chemotherapy, consisting of etoposide, carboplatin and bleomycin.
  • The patient has since attended regular follow-ups, without any neurological deficit or signs of recurrence in the 4 years since diagnosis.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Cerebellar Neoplasms / pathology. Cerebellar Neoplasms / therapy. Endodermal Sinus Tumor / pathology. Endodermal Sinus Tumor / therapy
  • [MeSH-minor] Bleomycin / therapeutic use. Carboplatin / therapeutic use. Chemotherapy, Adjuvant. Child, Preschool. Etoposide / therapeutic use. Humans. Magnetic Resonance Imaging. Male. Neurosurgical Procedures

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  • (PMID = 16314958.001).
  • [ISSN] 0167-594X
  • [Journal-full-title] Journal of neuro-oncology
  • [ISO-abbreviation] J. Neurooncol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 11056-06-7 / Bleomycin; 6PLQ3CP4P3 / Etoposide; BG3F62OND5 / Carboplatin
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2. Friedman JA, Lynch JJ, Buckner JC, Scheithauer BW, Raffel C: Management of malignant pineal germ cell tumors with residual mature teratoma. Neurosurgery; 2001 Mar;48(3):518-22; discussion 522-3
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  • [Title] Management of malignant pineal germ cell tumors with residual mature teratoma.
  • OBJECTIVE: The treatment of intracranial mixed germ cell tumors presents a unique challenge, since eradication of malignant tumor by radiation and/or chemotherapy may spare the benign tumor component.
  • We reviewed our surgical experience with residual malignant pineal germ cell tumors after neoadjuvant therapy.
  • METHODS: Between 1987 and 1997, 16 patients with malignant intracranial germ cell tumors were treated at the Mayo Clinic with a protocol of neoadjuvant chemotherapy and radiation therapy.
  • After the diagnosis was confirmed by histopathological examination, all patients were treated with four cycles of etoposide and cisplatin as well as external beam radiation therapy (range, 3030-5940 cGy).
  • Six patients had an incomplete response to therapy, as demonstrated by observation of residual tumor on magnetic resonance imaging scans.
  • Initial pathology in these six patients was germinoma in four and combinations of yolk sac tumor, embryonal carcinoma, malignant teratoma, and germinoma in two.
  • Two patients had synchronous pineal and suprasellar tumors, with leptomeningeal dissemination.
  • Tumor markers were elevated in four of the six patients at presentation.
  • CONCLUSION: Residual pineal tumor occurring after treatment of malignant intracranial germ cell tumor with neoadjuvant therapy is likely to be mature teratoma.
  • [MeSH-minor] Adolescent. Adult. Algorithms. Child. Combined Modality Therapy. Female. Follow-Up Studies. Humans. Male. Neoplasm, Residual


3. Kan P, Gottfried ON, Blumenthal DT, Liu JK, Salzman KL, Townsend J, Jensen RL: Primary spinal yolk sac tumor with brain metastasis: case report and review of the literature. J Neurooncol; 2006 Jul;78(3):249-53
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  • [Title] Primary spinal yolk sac tumor with brain metastasis: case report and review of the literature.
  • OBJECT: Central nervous system primary germ cell tumors are typically pineal or suprasellar.
  • Primary germ cell tumors of the spinal axis are very rare, with only a few case reports of germinomas and teratomas described in the literature.
  • METHODS: We present the unique case of a 25-year-old woman with an intradural, extramedullary primary yolk sac tumor (YST) at and below the level of the conus medullaris.
  • She was subsequently treated with four cycles of chemotherapy (intravenous cisplatin and etoposide), 40-Gy fractionated focal external beam radiation to the spine, and consolidation with four additional cycles of chemotherapy (intravenous carboplatin, vinblastine, etoposide, and bleomycin).
  • Despite an initial reduction in tumor size and clinical improvement in her neurologic exam, she re-presented a year after surgery with gross enlargement of her spinal tumor and CSF dissemination with metastasis to her brain.
  • Despite further chemotherapy and radiotherapy, the patient died from her disseminated YST.
  • When feasible (no evidence of CSF dissemination, metastasis, or multifocal disease), optimal treatment includes as extensive resection of tumor as possible followed by adjuvant chemotherapy and radiation.
  • The authors review the available literature on the treatment of intracranial malignant germ cell tumors, extrapolated to apply to the much rarer spinal lesions.
  • [MeSH-major] Brain Neoplasms / secondary. Endodermal Sinus Tumor / secondary. Neoplasm Recurrence, Local / pathology. Spinal Neoplasms / pathology

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  • (PMID = 16773223.001).
  • [ISSN] 0167-594X
  • [Journal-full-title] Journal of neuro-oncology
  • [ISO-abbreviation] J. Neurooncol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 16
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4. Janmohamed S, Grossman AB, Metcalfe K, Lowe DG, Wood DF, Chew SL, Monson JP, Besser GM, Plowman PN: Suprasellar germ cell tumours: specific problems and the evolution of optimal management with a combined chemoradiotherapy regimen. Clin Endocrinol (Oxf); 2002 Oct;57(4):487-500
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Suprasellar germ cell tumours: specific problems and the evolution of optimal management with a combined chemoradiotherapy regimen.
  • OBJECTIVE: Suprasellar germ cell tumours are rare, and there are few series of patients outlining the problems in diagnosis and management, and providing clear guidelines for optimal therapy.
  • PATIENTS AND DESIGN: A retrospective case review assessment of all patients seen within a given time.
  • Clinical, biochemical and radiological findings were reviewed, the types of therapy administered noted, and the responses to treatment analysed.
  • RESULTS: In the years 1977-2001, 11 patients with suprasellar (SS) germ cell tumours (GCT) were seen (germinomatous : nongerminomatous = 8 : 3).
  • The median age at presentation was 20 years (range 6-49 years) with a median duration of symptoms before diagnosis of 17 months (range 1-35 months).
  • Positron emission tomography scanning was occasionally useful in the evaluation of suprasellar tumours/pituitary stalk lesions deemed too risky to biopsy.
  • Treatment did not correct previously abnormal endocrine function although it did improve vision in three of six patients.
  • CONCLUSIONS: We therefore emphasize the use of techniques other than biopsy in the diagnosis of these patients, note the problems in the management of their fluid control, and highlight the favourable response to a combined chemotherapy-radiotherapy protocol.
  • [MeSH-major] Brain Neoplasms / diagnosis. Brain Neoplasms / therapy. Neoplasms, Germ Cell and Embryonal / diagnosis. Neoplasms, Germ Cell and Embryonal / therapy. Sella Turcica
  • [MeSH-minor] Adolescent. Adult. Algorithms. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Biomarkers, Tumor / analysis. Child. Combined Modality Therapy. Diabetes Insipidus / etiology. Disease-Free Survival. Female. Humans. Magnetic Resonance Imaging. Male. Middle Aged. Retrospective Studies. Treatment Outcome. Vision Disorders / etiology

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  • (PMID = 12354131.001).
  • [ISSN] 0300-0664
  • [Journal-full-title] Clinical endocrinology
  • [ISO-abbreviation] Clin. Endocrinol. (Oxf)
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Biomarkers, Tumor
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5. Starzyk J, Starzyk B, Bartnik-Mikuta A, Urbanowicz W, Dziatkowiak H: [Precocious puberty in girls depending on choriogonadotropin - case reports and literature review]. Endokrynol Diabetol Chor Przemiany Materii Wieku Rozw; 2001;7(1):27-32
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  • Tumor markers beta-HCG and AFP were markedly elevated and a suprasellar germ cell tumor 25x15 mm in size was visualized by MRI.
  • Combined chemotherapy followed by radiotherapy resulted in normalization of pubertal features along with estrogen and marker levels.
  • These data indicate a possible development of precocious puberty in girls induced by choriogonadotropin (hCG) released by a supracellar germ cell tumor.
  • Based on our observations and the literature on the subject the authors believe that the autonomous presence of aromatase inside the tumor may be responsible for precocious puberty in girls with hCG-releasing germ cell tumors, regardless of their location.

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  • (PMID = 12818144.001).
  • [ISSN] 1234-625X
  • [Journal-full-title] Endokrynologia, diabetologia i choroby przemiany materii wieku rozwojowego : organ Polskiego Towarzystwa Endokrynologów Dziecięcych
  • [ISO-abbreviation] Endokrynol Diabetol Chor Przemiany Materii Wieku Rozw
  • [Language] pol
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Poland
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6. Suzuki S, Nishio S, Takata K, Morioka T, Fukui M: Radiation-induced brain calcification: paradoxical high signal intensity in T1-weighted MR images. Acta Neurochir (Wien); 2000;142(7):801-4
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • METHOD: Three girls had each been diagnosed as having a suprasellar germ cell tumour and were treated with conventional fractionated radiotherapy in their childhood.
  • In one case, chemotherapy was given prior to the CNS irradiation.
  • [MeSH-major] Brain Diseases / etiology. Brain Neoplasms / radiotherapy. Calcinosis / etiology. Neoplasms, Germ Cell and Embryonal / radiotherapy. Radiation Injuries / etiology. Radiotherapy / adverse effects


7. Plowman PN, Besser GM, Shipley J, Summersgill B, Geddes J, Afshar F: Dramatic response of malignant craniopharyngioma to cis-platin-based chemotherapy. Should craniopharyngioma be considered as a suprasellar 'germ cell' tumour? Br J Neurosurg; 2004 Oct;18(5):500-5
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Dramatic response of malignant craniopharyngioma to cis-platin-based chemotherapy. Should craniopharyngioma be considered as a suprasellar 'germ cell' tumour?
  • The case is described of a 21-year-old woman, who developed a malignant tumour arising from a craniopharyngioma 14 years after the original diagnosis.
  • The remarkable response of this malignant tumour ex-craniopharyngioma to cis-platin based chemotherapy, together with other midline tumour characteristics of craniopharyngioma, raise the question as to whether craniopharyngioma should any longer be separately considered from suprasellar germ cell tumour.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Craniopharyngioma / drug therapy. Pituitary Neoplasms / drug therapy
  • [MeSH-minor] Adolescent. Adult. Child. Chromosome Aberrations. Cisplatin / administration & dosage. Etoposide / administration & dosage. Fatal Outcome. Female. Follow-Up Studies. Humans. Magnetic Resonance Imaging. Neoplasm Recurrence, Local / drug therapy

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  • (PMID = 15799153.001).
  • [ISSN] 0268-8697
  • [Journal-full-title] British journal of neurosurgery
  • [ISO-abbreviation] Br J Neurosurg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 6PLQ3CP4P3 / Etoposide; Q20Q21Q62J / Cisplatin
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8. Eom KS, Kim JM, Kim TY: Mixed germ cell tumors in septum pellucidum after radiochemotherapy of suprasellar germinoma: de novo metachronous or recurrent neoplasms? Childs Nerv Syst; 2008 Nov;24(11):1355-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Mixed germ cell tumors in septum pellucidum after radiochemotherapy of suprasellar germinoma: de novo metachronous or recurrent neoplasms?
  • INTRODUCTION: Mixed germ cell tumors (GCTs) consisting of a germinoma and a mature teratoma in the septum pellucidum have never been described previously; the patient we present here is the first reported example.
  • Five years ago, he received five cycles of chemotherapy using cisplatin and ectoposide and 24G of local radiotherapy for clinical diagnosis of suprasellar germinoma in another hospital.
  • The tumor was then completely resolute.
  • Magnetic resonance imaging in our hospital revealed a large fatty mass located primarily in the septum pellucidum and some portions of the corpus callosum; a heterogeneous enhancing tumor was observed in the surrounding area.
  • The second tumor was completely removed.
  • The histological diagnosis was mixed GCTs containing the component of a germinoma and a mature teratoma.
  • CONCLUSION: This case is characterized by a second GCT occurring at a different site and with a different histological type, long after complete resolution of suprasellar germinoma.
  • [MeSH-major] Brain Neoplasms / pathology. Germinoma / pathology. Neoplasms, Germ Cell and Embryonal / pathology. Neoplasms, Second Primary / pathology. Septum Pellucidum / pathology
  • [MeSH-minor] Adolescent. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Combined Modality Therapy. Humans. Immunohistochemistry. Magnetic Resonance Imaging. Male. Neurosurgical Procedures. Pituitary Neoplasms / pathology. Pituitary Neoplasms / therapy. Radiotherapy

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  • (PMID = 18560841.001).
  • [ISSN] 1433-0350
  • [Journal-full-title] Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
  • [ISO-abbreviation] Childs Nerv Syst
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Germany
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9. Cuccia V, Alderete D: Suprasellar/pineal bifocal germ cell tumors. Childs Nerv Syst; 2010 Aug;26(8):1043-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Suprasellar/pineal bifocal germ cell tumors.
  • PURPOSE: Intracranial germ cell tumors (GCT) arise from embryonal rests of germinal cells.
  • The aim of this report is to analyze a small group of GCT located simultaneously in the suprasellar and pineal regions without seeding either between both tumors or to other places.
  • We named this group as suprasellar/pineal bifocal germ cell tumors (SPBT).
  • METHODS: A retrospective review of a series of 25 GCT showed a) 16 cases of unifocal non-disseminated pineal or sellar GCT, b) one case of unifocal disseminated pineal GCT, c) three cases with suprasellar and pineal double tumors with dissemination, and d) five cases with SPBT.
  • Chemotherapy and radiotherapy were performed in all SPBT.
  • Prognosis for bifocal groups was similar to unifocal tumors of the same histological type.
  • CONCLUSIONS: SPBT seem to be an entity defined by a) one tumor in the suprasellar and another in the pineal region, b) GCT with predominance of PG, but not exclusively, and c) MRI and endoscopy without any dissemination.
  • [MeSH-major] Brain Neoplasms / pathology. Neoplasms, Germ Cell and Embryonal / pathology. Pineal Gland / pathology. Pinealoma / pathology
  • [MeSH-minor] Adolescent. Antineoplastic Agents / therapeutic use. Child. Combined Modality Therapy. Female. Humans. Male. Neurosurgical Procedures. Radiotherapy. Retrospective Studies. Treatment Outcome

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  • (PMID = 20221609.001).
  • [ISSN] 1433-0350
  • [Journal-full-title] Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
  • [ISO-abbreviation] Childs Nerv Syst
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Antineoplastic Agents
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10. Moschovi M, Alexiou GA, Dastamani A, Stefanaki K, Prodromou N, Hatzigiorgi H, Karamolegou K, Tzortzatou-Stathopoulou F: Alpha-fetoprotein secretion in a craniopharyngioma. Are craniopharyngiomas part of the germ cell tumor family? Acta Neurol Belg; 2010 Sep;110(3):272-5
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Alpha-fetoprotein secretion in a craniopharyngioma. Are craniopharyngiomas part of the germ cell tumor family?
  • Brain CT and MRI revealed a suprasellar cystic and partially solid mass with calcifications.
  • These findings were suggestive for a brain germ cell tumor.
  • Therefore, systemic chemotherapy was started.
  • After two courses there was a reduction in the levels of AFP but the tumor size remained unchanged.
  • Subtotal tumor excision was performed that revealed the presence of a craniopharyngioma.
  • One month later there was enlargement of the cystic part of the tumor, while serum AFP was elevated.
  • The child received again systemic chemotherapy and placement of a reservoir into the cystic part of the tumor.
  • These observations support the theory of a germ cell tumor family, in which craniopharyngioma and germ cell tumor present the two sides of the same entity, while between them a wide variety of tumors, with variable type of secretion of AFP and/or beta-HCG, may exist.
  • [MeSH-major] Craniopharyngioma. Neoplasms, Germ Cell and Embryonal / classification. Pituitary Neoplasms. alpha-Fetoproteins / secretion
  • [MeSH-minor] Female. Humans. Infant. Tomography, X-Ray Computed

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  • (PMID = 21114138.001).
  • [ISSN] 0300-9009
  • [Journal-full-title] Acta neurologica Belgica
  • [ISO-abbreviation] Acta Neurol Belg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Belgium
  • [Chemical-registry-number] 0 / AFP protein, human; 0 / alpha-Fetoproteins
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11. Tsugu H, Oshiro S, Ueno Y, Abe H, Komatsu F, Sakamoto S, Matsumoto S, Nabeshima K, Fukushima T, Inoue T: Primary yolk sac tumor within the lateral ventricle. Neurol Med Chir (Tokyo); 2009 Nov;49(11):528-31
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  • [Title] Primary yolk sac tumor within the lateral ventricle.
  • A 13-year-old girl presented with an exceedingly rare case of primary yolk sac tumor located within the lateral ventricle, manifesting as headache, nausea, and diplopia.
  • The tumor was removed subtotally via left middle temporal corticotomy.
  • The histological and immunohistochemical diagnosis was pure yolk sac tumor.
  • The patient underwent radiotherapy (whole brain, 30 Gy; tumor bed, 21 Gy; whole spinal axis, 30 Gy) and chemotherapy (ifosfamide, cisplatin, etoposide).
  • After three treatment cycles, the serum AFP level had decreased to 4.5 ng/ml.
  • However, the tumor recurred with cerebrospinal fluid dissemination after nine cycles of chemotherapy.
  • The possibility of germ cell tumor should be considered in pediatric patients with brain tumors occurring outside the pineal or suprasellar region.
  • [MeSH-major] Brain / pathology. Cerebral Ventricle Neoplasms / diagnosis. Endodermal Sinus Tumor / diagnosis. Lateral Ventricles / pathology
  • [MeSH-minor] Adolescent. Biomarkers, Tumor / analysis. Biomarkers, Tumor / blood. Chorionic Gonadotropin, beta Subunit, Human / blood. Diplopia / etiology. Drug Therapy. Fatal Outcome. Female. Headache / etiology. Humans. Hydrocephalus / etiology. Intracranial Hypertension / etiology. Magnetic Resonance Imaging. Nausea / etiology. Neoplasm Metastasis. Neoplasm Recurrence, Local. Neurosurgical Procedures. Radiotherapy. Ventriculostomy. alpha-Fetoproteins / metabolism

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  • (PMID = 19940403.001).
  • [ISSN] 1349-8029
  • [Journal-full-title] Neurologia medico-chirurgica
  • [ISO-abbreviation] Neurol. Med. Chir. (Tokyo)
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Japan
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Chorionic Gonadotropin, beta Subunit, Human; 0 / alpha-Fetoproteins
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12. Shinoda J, Sakai N, Yano H, Hattori T, Ohkuma A, Sakaguchi H: Prognostic factors and therapeutic problems of primary intracranial choriocarcinoma/germ-cell tumors with high levels of HCG. J Neurooncol; 2004 Jan;66(1-2):225-40
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  • [Title] Prognostic factors and therapeutic problems of primary intracranial choriocarcinoma/germ-cell tumors with high levels of HCG.
  • OBJECTIVE: Primary intracranial choriocarcinoma (PICCC)/germ-cell tumors (GCTs) with high levels of human chorionic gonadotropin (HCG) (PICCC/GCTs with HL-HCG) are rare and malignant.
  • The goal of this study was to report our 3 cases of PICCC/GCTs with HL-HCG and to review the literature to elucidate the clinical problems and prognostic factors and to discuss the therapeutic modalities of this rare tumor.
  • Significance of the differences among survival curves concerning each parameter (age, sex, tumor location, serum HCG/beta-HCG level, precocious puberty, extent of surgery, radiotherapy, chemotherapy, mixture of other non-germinomatous GCT elements and extraneural metastasis) was tested using univariate and multivariate analyses.
  • RESULTS: The median survival time and the 1- and 2-year survival rates were 22 months, 61.2% and 49.8%, respectively.
  • In univariate analysis, male, subtotal removal or more, radiotherapy and chemotherapy were revealed to be significantly good prognostic factors.
  • However, suprasellar region and tumor hemorrhage were poor prognostic factors.
  • Multivariate analysis showed that extent of surgery, radiotherapy and chemotherapy were independent prognostic factors.
  • CONCLUSIONS: Although, we should mind the limitations of this study design because of case selection bias, different treatment protocols and incomplete follow-up of patients, this study led the following results and suggestive conclusions.
  • Tumor hemorrhage and progressive extraneural and cerebrospinal fluid metastasis were characteristic clinical problems of PICCC/GCTs with HL-HCG.
  • In the cases with extremely elevated levels of HCG, biopsy for histological diagnosis may be no longer needed.
  • Initial biopsy and radiotherapy may lead to tumor hemorrhage.
  • To prevent tumor hemorrhage, gross tumor removal followed by radiotherapy and chemotherapy should be aimed for.
  • A few courses of chemotherapy before surgery may prevent metastasis.
  • Stereotactic radiotherapy and high dose chemotherapy may be promising options for treatment.
  • [MeSH-major] Brain Neoplasms / metabolism. Brain Neoplasms / therapy. Choriocarcinoma / metabolism. Choriocarcinoma / therapy. Chorionic Gonadotropin / metabolism. Germinoma / metabolism. Germinoma / therapy

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  • (PMID = 15015791.001).
  • [ISSN] 0167-594X
  • [Journal-full-title] Journal of neuro-oncology
  • [ISO-abbreviation] J. Neurooncol.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Chorionic Gonadotropin
  • [Number-of-references] 82
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13. Zanetta F, Di Dio G, Savastio S, Saccagno A, Petri A, Bellone S, Maghnie M, Bona G: [Germinoma: a rare cerebral tumor causing central diabetes insipidus in childhood]. Minerva Pediatr; 2008 Feb;60(1):129-33
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  • [Title] [Germinoma: a rare cerebral tumor causing central diabetes insipidus in childhood].
  • Germinoma represents 7.8% of cerebral tumors in pediatric age and 50-65% of germ cell cerebral tumors.
  • Generally it is a definite lesion of the pineal gland or suprasellar region, frequently occurring in the first three decades of life.
  • Clinical presentation depends on tumor localization.
  • In the absence of these classic signs and symptoms, however, the diagnosis of germinoma can be difficult.
  • The patient started therapy with desmopressin (Minirin) with good hydro-electrolytic balance.
  • A second MRI demonstrated a bifocal lesion with dyshomogeneous and cystic appearance, suggesting the diagnosis of germinoma.
  • On the basis of this case report we would like to point out the importance of an early diagnosis in order to improve the prognosis of the disease and the necessity of a careful follow-up of these patients.
  • [MeSH-major] Diabetes Insipidus / diagnosis. Diabetes Insipidus / etiology. Germinoma / complications. Germinoma / diagnosis. Pituitary Neoplasms / complications. Pituitary Neoplasms / diagnosis
  • [MeSH-minor] Antidiuretic Agents / therapeutic use. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Child. Deamino Arginine Vasopressin / therapeutic use. Diagnosis, Differential. Drug Therapy, Combination. Humans. Male. Treatment Outcome


14. Smith AA, Weng E, Handler M, Foreman NK: Intracranial germ cell tumors: a single institution experience and review of the literature. J Neurooncol; 2004 Jun;68(2):153-9
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  • [Title] Intracranial germ cell tumors: a single institution experience and review of the literature.
  • There is little literature to guide therapy in children and young adults with intracranial germ cell tumors.
  • We present 17 consecutively diagnosed intracranial germ cell tumors at The Children's Hospital, Denver, from 1995 to 2001.
  • Of 17 patients, 3 had considerable delay in diagnosis.
  • Two with suprasellar tumors presented with dementia, blindness and pan-hypopituitarism and another with recurrent subarachnoid hemorrhage.
  • Seven had germinoma, three were metastatic at diagnosis.
  • Ten had non-germinomatous germ cell tumors (NGGCT), 5/10 were alpha feto-protein (AFP) positive only, one beta-human chorionic growth (betaHCG) factor positive only, 3 positive for AFP and betaHCG, and 1 malignant teratoma.
  • Therapy for metastatic patients consisted of chemotherapy followed by craniospinal radiation (CSI).
  • Patients with localized disease received chemotherapy followed by focal radiation.
  • Two patients received chemotherapy only, one because she died of sepsis while receiving chemotherapy and one because of neurologic injury incurred during surgery parents elected for no therapy.
  • Three patients have died, one of tumor recurrence, one from a remote complication of surgery and one of sepsis.
  • All five children with only AFP positivity, treated with chemotherapy and focal radiation are alive without evidence of disease at 10, 16, 22, 41 and 41 months.
  • Thus, there is little evidence that CSI is necessary in non-metastatic germinomas and AFP positive NGGCTs when combined chemotherapy and radiation therapy is used.
  • However, complications of delayed diagnosis, surgery and chemotherapy are important causes of mortality, with only one patient dying of tumor.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Brain Neoplasms / pathology. Germinoma / pathology
  • [MeSH-minor] Adult. Child. Combined Modality Therapy. Humans. Retrospective Studies

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  • (PMID = 15218952.001).
  • [ISSN] 0167-594X
  • [Journal-full-title] Journal of neuro-oncology
  • [ISO-abbreviation] J. Neurooncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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15. Echevarría ME, Fangusaro J, Goldman S: Pediatric central nervous system germ cell tumors: a review. Oncologist; 2008 Jun;13(6):690-9
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  • [Title] Pediatric central nervous system germ cell tumors: a review.
  • Central nervous system (CNS) germ cell tumors (GCTs) represent approximately 3% of primary pediatric brain tumors and encompass a wide pathologic spectrum.
  • CNS GCTs are most commonly located in the pineal and suprasellar regions of the brain and can be divided into major groups including germinomas and nongerminomatous GCTs (NGGCTs), with teratomas often considered a separate category.
  • Neuroimaging studies cannot differentiate GCTs from other tumors, and therefore, the diagnosis usually requires histologic confirmation.
  • The rare exceptions are the cases where characteristic elevations of tumor markers, including alpha-fetoprotein and/or beta-human chorionic gonadotropin are documented in the serum and/or cerebrospinal fluid.
  • In these cases, the imaging findings along with the tumor marker elevation may be diagnostic in themselves without the need for tissue confirmation.
  • Treatment and prognosis differ greatly between groups.
  • More recently, the use of chemotherapy in addition to radiation therapy has afforded the ability to decrease the dose and volume of radiation therapy without affecting survival rates.
  • NGGCTs are less radiosensitive than germinomas, but the use of adjuvant chemotherapy has improved survival rates in this group as well.
  • Treatment regimens aimed to improve progression-free and overall survival times are ongoing.
  • [MeSH-major] Central Nervous System Neoplasms / pathology. Neoplasms, Germ Cell and Embryonal / pathology
  • [MeSH-minor] Child. Combined Modality Therapy. Humans

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  • (PMID = 18586924.001).
  • [ISSN] 1083-7159
  • [Journal-full-title] The oncologist
  • [ISO-abbreviation] Oncologist
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 75
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16. Kumabe T, Kusaka Y, Jokura H, Ikeda H, Shirane R, Yoshimoto T: [Recurrence of intracranial germinoma initially treated with chemotherapy only]. No Shinkei Geka; 2002 Sep;30(9):935-42
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  • [Title] [Recurrence of intracranial germinoma initially treated with chemotherapy only].
  • Eight consecutive patients with intracranial germinoma underwent combination chemotherapy with BEP (bleomycin, etoposide, and cisplatin) or PE (cisplatin and etoposide) without irradiation between 1996 and 1997.
  • The diagnosis was based on endoscopic or stereotactic biopsy, or transsphenoidal surgery in 7 cases.
  • These 7 patients obtained complete response (CR) after treatment with chemotherapy only.
  • The diagnosis of pure germinoma was based on neuroradiographic appearances and the normal levels of tumor markers in patients with suprasellar and pineal-region germ cell tumors.
  • Although all patients obtained CR, 5 patients relapsed at a mean period of 19 months after the initial therapy.
  • Additional chemotherapy and total ventricle irradiation (24 Gy) achieved CR, but delayed seeding in the optic nerves outside the irradiated field was detected.
  • Another patient obtained CR after PE therapy, but there was not histological verification.
  • However, 35 months after the initial therapy, surgical extirpation of the recurrent tumor revealed immature teratoma.
  • One patient refused treatment for recurrence at the initial tumor site and died 42 months after the initial treatment.
  • Adequate initial therapy is absolutely essential for the treatment of intracranial germinoma.
  • The chemotherapy regimens in use today cannot be recommended as initial therapy without irradiation because of the high recurrence rates.
  • [MeSH-major] Brain Neoplasms / drug therapy. Germinoma / drug therapy. Neoplasm Recurrence, Local
  • [MeSH-minor] Adolescent. Adult. Antineoplastic Combined Chemotherapy Protocols / administration & dosage. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Bleomycin / administration & dosage. Child. Cisplatin / administration & dosage. Etoposide / administration & dosage. Fatal Outcome. Follow-Up Studies. Humans. Male. Radiotherapy, Adjuvant. Treatment Outcome

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  • (PMID = 12233091.001).
  • [ISSN] 0301-2603
  • [Journal-full-title] No shinkei geka. Neurological surgery
  • [ISO-abbreviation] No Shinkei Geka
  • [Language] jpn
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Japan
  • [Chemical-registry-number] 11056-06-7 / Bleomycin; 6PLQ3CP4P3 / Etoposide; Q20Q21Q62J / Cisplatin; BEP protocol
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17. Vervenne WL, Bakker PJ, Stalpers LJ, Bosch DA: [Malignant intracranial germ cell tumor treated with chemotherapy and radiotherapy without histopathological confirmation]. Ned Tijdschr Geneeskd; 2000 Mar 11;144(11):527-31
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  • [Title] [Malignant intracranial germ cell tumor treated with chemotherapy and radiotherapy without histopathological confirmation].
  • [Transliterated title] Maligne intracerebrale kiemceltumor behandeld met chemotherapie en radiotherapie zonder histopathologische diagnose.
  • In two men aged 19 and 24 years, a rare malignant intracranial germ cell tumour was diagnosed in the pineal gland region and in the second patient in a suprasellar position as well.
  • The clinical picture of a young patient with an intracerebral tumour localised in the midline of the brain and increased levels of the tumour markers alpha-foetoprotein and/or human chorion gonadotrophin (beta-HCG) in blood and/or CSF makes any other diagnosis highly unlikely.
  • There is no place for radical surgery in the first-line treatment of malignant intracerebral germ cell tumours because of the sensitivity to radio- and chemotherapy.
  • Also, the sensitivity to chemotherapy makes it possible to reduce radiation volume and dose in an effort to avoid the serious complications of craniospinal irradiation.
  • Both patients responded well to chemotherapy based on cisplatin followed by radiotherapy.
  • [MeSH-major] Biomarkers, Tumor / metabolism. Brain Neoplasms / diagnosis. Brain Neoplasms / therapy. Neoplasms, Germ Cell and Embryonal / diagnosis. Neoplasms, Germ Cell and Embryonal / therapy
  • [MeSH-minor] Adult. Chemotherapy, Adjuvant. Chorionic Gonadotropin, beta Subunit, Human / blood. Chorionic Gonadotropin, beta Subunit, Human / cerebrospinal fluid. Diagnosis, Differential. Humans. Magnetic Resonance Imaging. Male. Radiotherapy, Adjuvant. Tomography, X-Ray Computed. Treatment Outcome. alpha-Fetoproteins / cerebrospinal fluid

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  • (PMID = 10735140.001).
  • [ISSN] 0028-2162
  • [Journal-full-title] Nederlands tijdschrift voor geneeskunde
  • [ISO-abbreviation] Ned Tijdschr Geneeskd
  • [Language] dut
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] NETHERLANDS
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Chorionic Gonadotropin, beta Subunit, Human; 0 / alpha-Fetoproteins
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18. Kendi TK, Caglar S, Huvaj S, Bademci G, Kendi M, Alparslan S: Suprasellar germ cell tumor with subarachnoid seeding MRI and MR spectroscopy findings. Clin Imaging; 2004 Nov-Dec;28(6):404-7
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  • [Title] Suprasellar germ cell tumor with subarachnoid seeding MRI and MR spectroscopy findings.
  • A case of suprasellar germinoma with subarachnoid seeding is presented.
  • Patient had a history of depression unresponsive to drug therapy and recently developed diabetes insipidus.
  • MR imaging revealed a huge suprasellar mass, heterogenous in signal intensity due to cystic components.
  • [MeSH-major] Brain Neoplasms / diagnosis. Germinoma / diagnosis. Magnetic Resonance Imaging / methods. Neoplasm Invasiveness / pathology. Tomography, Emission-Computed, Single-Photon / methods

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  • (PMID = 15531139.001).
  • [ISSN] 0899-7071
  • [Journal-full-title] Clinical imaging
  • [ISO-abbreviation] Clin Imaging
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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19. Starzyk J, Starzyk B, Bartnik-Mikuta A, Urbanowicz W, Dziatkowiak H: Gonadotropin releasing hormone-independent precocious puberty in a 5 year-old girl with suprasellar germ cell tumor secreting beta-hCG and alpha-fetoprotein. J Pediatr Endocrinol Metab; 2001 Jun;14(6):789-96
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Gonadotropin releasing hormone-independent precocious puberty in a 5 year-old girl with suprasellar germ cell tumor secreting beta-hCG and alpha-fetoprotein.
  • Tumor markers beta-hCG and AFP were markedly elevated and a 2.5 x 1.5 cm suprasellar germ cell tumor (GCT) was visualized by MRI.
  • Combined chemotherapy followed by radiotherapy resulted in normalization of pubertal features along with estrogen and marker levels.
  • Our observations support the possibility of hCG-dependent precocious puberty (PP) in girls caused by suprasellar hCG-secreting tumor.
  • We hypothesize that the rarity of isosexual PP in girls with hCG-secreting suprasellar GCT results not only from the lower occurrence of these tumors in girls than in boys, but above all from a rare simultaneous concomitant incidence of both high tumor aromatase activity and hCG secreting potency.
  • [MeSH-minor] Child, Preschool. Combined Modality Therapy. Female. Humans. Magnetic Resonance Imaging. Sella Turcica

  • Genetic Alliance. consumer health - Precocious puberty.
  • MedlinePlus Health Information. consumer health - Brain Tumors.
  • MedlinePlus Health Information. consumer health - Childhood Brain Tumors.
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  • (PMID = 11453531.001).
  • [ISSN] 0334-018X
  • [Journal-full-title] Journal of pediatric endocrinology & metabolism : JPEM
  • [ISO-abbreviation] J. Pediatr. Endocrinol. Metab.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Chorionic Gonadotropin, beta Subunit, Human; 0 / alpha-Fetoproteins; 33515-09-2 / Gonadotropin-Releasing Hormone
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20. Koh EJ, Phi JH, Park SH, Kim IO, Cheon JE, Wang KC, Cho BK, Kim SK: Mixed germ cell tumor of the midbrain. Case Report. J Neurosurg Pediatr; 2009 Aug;4(2):137-42
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Mixed germ cell tumor of the midbrain. Case Report.
  • The histopathological diagnosis was of a mixed germ cell tumor (GCT) comprising mature teratoma and germinoma cells with syncytiotrophoblastic giant cells.
  • The patient underwent postoperative chemotherapy and radiotherapy, and no tumor progression was found during 1 year of follow-up.
  • Intracranial GCTs arise mainly in the pineal and the suprasellar area.
  • Germ cell tumors in the brainstem are rare, with only 12 reported cases.
  • [MeSH-major] Brain Stem Neoplasms / diagnosis. Brain Stem Neoplasms / therapy. Neoplasms, Germ Cell and Embryonal / diagnosis. Neoplasms, Germ Cell and Embryonal / therapy

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  • (PMID = 19645547.001).
  • [ISSN] 1933-0707
  • [Journal-full-title] Journal of neurosurgery. Pediatrics
  • [ISO-abbreviation] J Neurosurg Pediatr
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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21. Wong JM, Chi SN, Marcus KJ, Levine BS, Ullrich NJ, MacDonald S, Lechpammer M, Goumnerova LC: Germinoma with malignant transformation to nongerminomatous germ cell tumor. J Neurosurg Pediatr; 2010 Sep;6(3):295-8
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Germinoma with malignant transformation to nongerminomatous germ cell tumor.
  • The authors describe the case of a young girl with suprasellar germinoma.
  • Six weeks after this diagnosis, just prior to initiation of therapy, serum and CSF marker analysis revealed sudden and marked elevation of alpha-fetoprotein, indicating transformation of her germinoma to a nongerminomatous germ cell tumor.
  • She underwent chemotherapy and radiation therapy per the national treatment approach for the new diagnosis, with subsequent return of her serum and CSF tumor markers to normal levels.
  • To the authors' knowledge, this is the first case in the English-language literature of a nongerminomatous germ cell tumor resulting from conversion of germinoma at the original site of presentation.
  • [MeSH-major] Brain Neoplasms / diagnosis. Germinoma / diagnosis. Neoplasms, Germ Cell and Embryonal / diagnosis


22. Kyritsis AP: Management of primary intracranial germ cell tumors. J Neurooncol; 2010 Jan;96(2):143-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Management of primary intracranial germ cell tumors.
  • Primary intracranial germ cell tumors are rare and usually localized in the pineal and the suprasellar regions.
  • They are divided into the following histologic types: germinoma, teratoma (mature, immature, malignant), choriocarcinoma, embryonal carcinoma, endodermal sinus tumor (yolk sac tumor), and mixed tumors.
  • Localized germinomas are treated with radiation therapy and exhibit a relatively good prognosis.
  • Chemotherapy is reserved for disseminated germinomas.
  • The rest of germ cell tumors are managed with various combinations of surgery, chemotherapy, and radiotherapy depending on the tumor type.
  • If the tumors secrete beta-human chorionic gonadotrophin (hCG) or alpha-fetoprotein (FP), these tumor markers can be used to accurately monitor response to treatment.
  • [MeSH-major] Neoplasms, Germ Cell and Embryonal / classification. Neoplasms, Germ Cell and Embryonal / therapy. Pinealoma / therapy
  • [MeSH-minor] Carcinoma, Embryonal / therapy. Germinoma / therapy. Humans. Magnetic Resonance Imaging / methods. Nerve Tissue Proteins / metabolism. Teratoma / therapy

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  • (PMID = 19588227.001).
  • [ISSN] 1573-7373
  • [Journal-full-title] Journal of neuro-oncology
  • [ISO-abbreviation] J. Neurooncol.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Nerve Tissue Proteins
  • [Number-of-references] 42
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