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Objective:To investigate the clinical and imaging features of basal ganglia germinoma in children and adolescents, so as to improve the accuracy of early diagnosis.Methods:The clinical symptoms and imaging findings of 10 cases of basal ganglia germinoma diagnosed by surgical examination or diagnostic radiotherapy in Xinhua Hospital Affiliated to Medical College of Shanghai Jiaotong University from January 2010 to December 2019 were analyzed retrospectively.Results:There were 9 males and 1 female, aged from 5 to 15 years, with an average age of 9.7 years.There were 10 cases of limb weakness or mild hemiplegia, 1 case of headache and vomiting, and 1 case of extrapyramidal tract sign. There were 9 cases of computed tomography (CT) examination, 5 cases of high density, 4 cases of mixed density, and 3 cases of intrafocal calcification. 10 cases of routine magnetic resonance imaging (MRI) examination, MR T1-weighted imaging (T1WI): 5 cases of low signal, 3 cases of slightly low signal, 1 case of equal low signal, 1 case of equal slightly low signal; MR T2-weighted imaging (T2WI): 3 cases of high signal, 5 cases of slightly high signal, 2 cases of high and low mixed signal; T2 fluid attenuated inversion recovery (T2FLAIR): 1 case of low signal, 2 cases of slightly high signal, 1 case of iso-high signal, 5 cases of high signal, 1 case of high and low mixed signal; Diffusion weighted imaging (DWI): 2 cases of low signal, 1 case of equal signal, 1 case of equal slightly high signal, 4 cases of slightly high signal, 2 cases of high signal; 1 case of functional imaging susceptibility-weighted imaging (SWI): 1 case of low signal; 10 cases of MRI enhancement: 2 cases of no enhancement, 5 cases of mild enhancement, 3 cases of moderate/obvious enhancement, and the enhancement methods can be seen in spot, patch, mass and ring enhancement; Functional imaging magnetic resonance angiography (MRA) in 2 cases: 1 case had slender middle cerebral artery, fewer branch arteries than the opposite side, and 1 case showed no obvious abnormality; 2 cases of positron emission (PET)-CT: the metabolism of methionine (MET) on the affected side was significantly increased in 2 cases.Conclusions:Germinoma in the basal ganglia of children and adolescents are more common in men, with weakness or mild hemiplegia as the main symptom. The imaging findings have certain characteristics. Imaging findings combined with relevant clinical data can improve the accuracy of early diagnosis of germinoma in the basal ganglia.
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OBJECTIVE@#To summarize and analyze the clinical characteristics of children with basal ganglia germinoma and to improve the level of early clinical diagnosis.@*METHODS@#The clinical data of children diagnosed with basal ganglia germinoma admitted to the Pediatric Surgery Ward of Peking University First Hospital from January 2013 to December 2020 were retrospectively analyzed, and descriptive statistics were used to analyze the clinical characteristics of children with basal ganglia germinoma.@*RESULTS@#A total of 30 patients were included in the study, 28 were male, 2 were female, the mean age at onset was (9.7±2.2) years, the median disease duration was 7 months, 27 had unilateral disease, and 3 had bilateral disease. The clinical manifestations were decreased limb muscle strength, cognitive function disorders, polydipsia, precocious puberty, intracranial hypertension, dysphonia and swallowing dysfunction. The serum and cerebrospinal fluid tumor marker alpha-fetoprotein (AFP) were normal in the 30 patients, and the serum and cerebrospinal fluid tumor marker β-human chorionic gonadotropin (β-HCG) were normal in 8 patients.The serum β-HCG was normal in 11 patients but the cerebrospinal fluid β-HCG was slightly elevated, and the serum and cerebrospinal fluid β-HCG were slightly elevated in 11 patients. A total of 33 lesions with irregular shapes were found by imaging examination, including 15 (45.5%) patchy lesions, 10 (30.3%) patchy lesions, and 8 (24.2%) round-like high-density lesions. Tumors showed obvious high-density shadows on computed tomography (CT) scan. Magnetic resonance imaging (MRI) scan of the tumors showed low or isointensity on T1WI and isointensity on T2WI, accompanied by mild peritumoral edema, hemispheric atrophy, cerebral peduncle atrophy, calcification, cystic degeneration, ventricular dilatation and wallerian degeneration. On contrast-enhanced scans, the tumor showed no enhancement or heterogeneous enhancement.@*CONCLUSION@#The main age of onset of germ cell tumors in the basal ganglia in children is about 10 years old, and males are absolutely dominant. The clinical features and imaging manifestations have certain characteristics. With both combined, the early diagnosis of germ cell tumors in the basal ganglia can be improved.
Subject(s)
Child , Female , Humans , Male , Atrophy/pathology , Basal Ganglia/pathology , Biomarkers, Tumor , Brain Neoplasms/diagnostic imaging , Chorionic Gonadotropin, beta Subunit, Human , Germinoma/pathology , Magnetic Resonance Imaging , Neoplasms, Germ Cell and Embryonal , Retrospective StudiesABSTRACT
Los tumores del sistema nervioso central representan la segunda enfermedad oncológica más habitual en niños y adolescentes. Entre los tumores intracraneales, los de células germinales son infrecuentes. Los síntomas que desencadenan son cefalea, náuseas, vómitos, déficits hormonales, alteraciones visuales, pérdida de peso, pobre crecimiento y pubertad precoz. Menos frecuentemente, producen trastornos del movimiento o psiquiátricos. Algunos de estos tumores pueden ser asintomáticos un largo período, lo que desencadena un diagnóstico tardío.Se presenta a una paciente femenina de 14 años con pérdida de peso y falla del crecimiento, con diagnóstico erróneo de trastorno de la conducta alimentaria. Tras estudios pertinentes, se arribó al diagnóstico de germinoma del sistema nervioso central. Al ser esta patología infrecuente y de presentación variable, requiere alto sentido de alerta por parte de la familia involucrada y del equipo de salud para evitar retrasos en el diagnóstico y el tratamiento
Central nervous system tumors are the second most frequent oncological disease among children and teenagers. Among the intracranial tumors, the germ cells ones are infrequent. The symptoms they cause are headaches, nausea and vomiting, hormonal deficits, visual disturbances, weight loss, poor growth and early puberty. Less frequently, they produce movement or psychiatric disorders. Some of these tumors can be asymptomatic for a long period leading to a late diagnosis.The case of a 14-year-old female patient is presented. She showed weight loss and growth failure, with wrong diagnosis of eating disorder. After proper study methods, we arrived to central nervous system germinoma diagnosis. Because this pathology is rare and has a variable form of presentation, it requires that the family involved and the health team to be alert, to avoid delays in diagnosis and treatment.
Subject(s)
Humans , Female , Adolescent , Germinoma/diagnostic imaging , Brain Neoplasms , Weight Loss , Germinoma/therapy , Failure to Thrive , HypopituitarismABSTRACT
RESUMO: O hipopituitarismo é a deficiência de dois ou mais hormônios hipofisários, que se expressa por sintomas dependentes do tipo e grau de déficit hormonal. A adequada condução destes pacientes é de fundamental importância para que não acarrete atraso no crescimento e desenvolvimento, óbito ou mudanças na qualidade de vida dos indivíduos. Objetiva-se, neste estudo, relatar caso clínico de abordagem de paciente pediátrico com pan-hipopituitarismo e descrever o manejo adotado, bem como a importância do acompanhamento pelo endocrinologista pediátrico. Trata-se de paciente do sexo masculino, 14 anos, com pan-hipopituitarismo iniciado na infância, secundário ao surgimento de germinoma e ao tratamento realizado para o mesmo. A primeira deficiência hormonal apresentada foi diabetes insipidus, seguida, após a realização de quimioterapia e radioterapia, de múltiplas deficiências. O menor segue em acompanhamento especializado, e faz uso de levotiroxina, desmopressina, somatropina, testosterona e prednisolona. A partir do presente relato, percebe-se a importância do diagnóstico oportuno e da adequada abordagem da criança com pan-hipopituitarismo e de seu seguimento a fim de se manter uma qualidade de vida satisfatória. (AU)
ABSTRACT: Hypopituitarism is the deficiency of two or more pituitary hormones. Its symptoms depend on the type and degree of hormonal deficit. Proper care of these patients is of fundamental importance to avoid delay in growth and development, death, or changes in the quality of life. The objective of this study is to report a clinical case of a pediatric patient with pan-hypopituitarism and describe the care adopted, as well as the importance of monitoring by the pediatric endocrinologist. The patient was a 14-year-old boy, with pan-hypopituitarism beginning in childhood, secondary to the appearance of germinoma and the treatment performed for it. The first hormonal deficiency presented was diabetes insipidus, followed by multiple deficiencies after chemotherapy and radiotherapy. The patient is under specialized monitoring and takes levothyroxine, desmopressin, somatropin, testosterone, and prednisolone. From the present report, timely diagnosis and adequate approach to a child with pan-hypopituitarism and its follow-up are important to maintain a satisfactory quality of life. (AU)
Subject(s)
Humans , Male , Adolescent , Pituitary Hormones , Quality of Life , Germinoma , Failure to Thrive , Medication Therapy Management , Transitional Care , HypopituitarismABSTRACT
Intracranial germ cell tumor is more common in Asian countries, including Japan, than in Western countries. The disease is characterized by juvenile onset with a mean age at diagnosis of 18 years. Most patients with intracranial germ cell tumors in the basal ganglia manifest paralytic symptoms, but few of these patients have been reported to have long-term progression of motor paralysis and rehabilitation interventions.A young male patient was diagnosed as having right basal ganglia germinoma and left hemiplegia at the age of 10 years. He received intervention and long-term follow-up for upper limb function. He underwent hybrid assistive neuromuscular dynamic stimulation therapy at the age of 14 years and modified constraint-induced movement therapy (modified CI therapy) at the age of 20 years. With such a gradual neurorehabilitation intervention, the Fugl-Meyer assessment score for the upper limb improved from 41 to 58 points, and the frequency of use of the paralyzed hand also improved. We hope that this report will provide guidance when considering treatment options for similar diseases in the future.
ABSTRACT
Intracranial germ cell tumor is more common in Asian countries, including Japan, than in Western countries. The disease is characterized by juvenile onset with a mean age at diagnosis of 18 years. Most patients with intracranial germ cell tumors in the basal ganglia manifest paralytic symptoms, but few of these patients have been reported to have long-term progression of motor paralysis and rehabilitation interventions.A young male patient was diagnosed as having right basal ganglia germinoma and left hemiplegia at the age of 10 years. He received intervention and long-term follow-up for upper limb function. He underwent hybrid assistive neuromuscular dynamic stimulation therapy at the age of 14 years and modified constraint-induced movement therapy (modified CI therapy) at the age of 20 years. With such a gradual neurorehabilitation intervention, the Fugl-Meyer assessment score for the upper limb improved from 41 to 58 points, and the frequency of use of the paralyzed hand also improved. We hope that this report will provide guidance when considering treatment options for similar diseases in the future.
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We report a case of sellar/suprasellar germinoma in a 17-year-old female which was misdiagnosed as pituitary adenoma both clinically and on imaging. Patient presented with severe headache of short duration and diminished visual acuity in left eye. Endocrinological work up revealed hyperprolactinemia and markedly reduced blood cortisol. MRI revealed homogenously enhanced intrasellar mass with suprasellar extension showing characteristic dumbbell conguration (snowman sign). Provisional diagnosis of pituitary adenoma was made and transsphenoidal resection of tumor was performed which on histology and immunohistochemistry proved to be germinoma. Sellar/suprasellar germinoma without diabetes insipidus is considered to be very difcult to diagnose preoperatively and the differentiation from pituitary adenoma based on MRI ndings was not possible in this case. However it is suggested that in a child with visual impairment, endocrine dysfunction and sellar/suprasellar mass a possibility of germ cell tumor should always be considered in differential diagnosis and a frozen section has to be performed to avoid excessive dissection.
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Background@#Rapid visual acuity (VA) decline was a common complaint in patients with sellar/suprasellar germinoma. In our hospital, 3.4 Gy/2f of emergency irradiation was applied to save patient VA and enable subsequent chemoradiotherapy. This study aimed to investigate the efficacy of emergency irradiation with 3.4 Gy/2f in patients with sellar/suprasellar germinoma who had rapid VA decline.@*Methods@#From January 2014 to December 2017, 33 patients with sellar/suprasellar germinoma who complained of VA decline within 3 months received 3.4 Gy/2f of emergency irradiation in Beijing Tiantan Hospital. The best-corrected VA (BCVA) and mean deviation (MD) were measured. Correlations between visual function change and clinical factors, including age at diagnosis, duration of VA decline, extent of tumor regression, serum level of tumor markers, were analyzed.@*Results@#Among 33 patients with sellar/suprasellar germinoma, the median diameter and volume of sellar/suprasellar lesions were 32 mm (range: 5–55 mm) and 12.9 cm3 (range 0.6–58.5 cm3), respectively. Data on pre- and post-emergency-irradiation BCVA were obtained in 32 patients. For the right eyes, BCVA was improved in 23 patients (71.9%), unchanged in 7 (21.9%), and worsened in 2 (6.2%); and for the left eyes, these numbers were 27 (84.4%), 4 (12.5%), and 1 (3.1%), respectively. In terms of the logarithm of the minimum angle of resolution (logarithm of the minimum angle of resolution = Log (1/BCVA) score, the improvement was significant in both eyes (P < 0.001). In terms of MD, six patients had paired data and the improvement was marginal in the right eyes (P = 0.068) and significant in the left eyes (P = 0.043). However, no clinical factor was found to have correlation with visual function improvement.@*Conclusion@#In sellar/suprasellar germinoma patients with VA decline, 3.4 Gy/2f of emergency irradiation was effective in improving visual function.
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@#Introduction: Intratumoral calcification is a feature that is more often observed in pineal parenchymal tumour than germinoma. We describe a 13-year-old male with pineal region germinoma demonstrating extensive intratumoral calcification. Case report: He presented with worsening headache that was associated with fatigue, nausea and vomiting. Radiologic examination revealed a multilobular mass in the pineal region with internal calcifications. Biopsy showed a pure germinoma with unusually extensive calcification. Discussion: Although a diagnosis may be suggested with a careful evaluation of imaging, there is no pathognomonic pattern. Thus, histologic verification is necessary for most pineal region masses.
Subject(s)
GerminomaABSTRACT
Central nervous system germ cell tumors are rare and they occur in the first two decades of life. Optic nerve germinomas can sometimes mimic optic nerve inflammation. In this case report, we discuss an 11-year-old girl who presented with features of presumed bilateral optic neuritis and developed polyuria and polydipsia, subsequently she was diagnosed to have infiltrative etiology. Her clinical and radiological presentations were initially consistent with inflammatory optic neuropathy. Poor visual recovery to steroid therapy and progressive visual loss warranted the need for optic nerve biopsy which revealed germinoma.
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A 32-year old male came to our hospital with chief complaint of paraplegia. He had symptom of radiating pain to right leg 3 months ago before paraplegic symptom appeared. Magnetic resonance (MR) imaging from outside hospital showed intramedullary mass involving from T8 to T10 level of spinal cord. According to the imaging result, tumor removal with total laminectomy was performed between T8 and T10 level in our hospital. Pathologic result was compatible with germinoma. Spine radiation (39.6 Gy/22 fx) from T7 to T12 level without chemotherapy was performed 3 weeks later since tumor removal. Follow-up MR imaging showed no recurrence without any distant metastasis. And our patient's neurologic symptom had been improved. According to this case, postoperative radiotherapy is thought to be effective to primary spinal germinoma.
Subject(s)
Humans , Male , Drug Therapy , Follow-Up Studies , Germinoma , Laminectomy , Leg , Magnetic Resonance Imaging , Neoplasm Metastasis , Neurologic Manifestations , Paraplegia , Radiotherapy , Recurrence , Spinal Cord , SpineABSTRACT
Germinoma is the most common type of intracranial germ cell tumors (GCTs). Pineal gland and suprasellar region are the most frequent sites of central nervous system (CNS) involvement. Intracranial masses caused by Langerhans cell histiocytosis (LCH) mimics features of CNS GCTs. LCH frequently involve spine and is the most common cause of vertebra plana in children. A 15-year-old boy presented with progressing symptoms of polydipsia, polyuria, general headache, nausea and severe back pain. Brain MRI showed brain tumor with simultaneous involvement of suprasellar region and pineal gland. An excisional biopsy of suprasellar mass was done. The pathologic assessment confirmed the diagnosis of germinoma. Patient's treatment continued accordingly. A spine MRI, done due to persistent backache, showed a vertebra plana. We reevaluated the primary diagnosis suspecting LCH. Germinoma of CNS was confirmed and a biopsy of vertebral lesion resulted in hemangioma. Thus we report a case of CNS germinoma with co-occurrence of vertebra plana. We emphasized the importance of histopathologic diagnosis of pineal/suprasellar masses and primary investigation of other CNS regions including spine for possible metastasis or comorbidities.
Subject(s)
Adolescent , Child , Humans , Male , Back Pain , Biopsy , Brain , Brain Neoplasms , Central Nervous System , Comorbidity , Diagnosis , Germinoma , Headache , Hemangioma , Histiocytosis, Langerhans-Cell , Magnetic Resonance Imaging , Nausea , Neoplasm Metastasis , Neoplasms, Germ Cell and Embryonal , Pineal Gland , Polydipsia , Polyuria , SpineABSTRACT
Intracranial germ cell tumors (iGCTs) are a heterogeneous group of tumors with peculiar characteristics clearly distinguished from other brain tumors of neuroepithelial origin. Diverse histology, similarity to gonadal GCT, predilection to one sex, and geographic difference in incidence all present enigmas and fascinating challenges. The treatment of iGCT has advanced for germinoma to date; thus, clinical attention has shifted from survival to long-term quality of life. However, for non-germinomatous GCT, current protocols provide only modest improvement and more innovative therapies are needed. Recently, next-generation sequencing studies have revealed the genomic landscape of iGCT. Novel mutations in the KIT-RAS-MAPK and AKT-MTOR pathways were identified. More importantly, methylation profiling revealed a new method to assess the pathogenesis of iGCT. Molecular research will unleash new knowledge on the origin of iGCT and solve the many mysteries that have lingered on this peculiar neoplasm for a long time.
Subject(s)
Brain Neoplasms , Germ Cells , Germinoma , Gonads , Incidence , Methods , Methylation , Neoplasms, Germ Cell and Embryonal , Quality of Life , Therapies, InvestigationalABSTRACT
Malignant ovariangerm cell tumors (MOGCTs) is second only to epithelial tumor which often occur in young women and young women,with high malignancy and high mortality.Effective treatment is particularly important in clinical practice.The prognosis is improved for valid chemotherapy scheme foun ded in recent years.Surgery still play a crucial role in the therapy of MOGCTs no matter for the primary operation or re-operation.Since the 70s,the comprehensive surgical staging (CSS) has improved the prognosis in patients with malignant ovarian cell tumors.Retroperitoneal lymphadenectomy is an integral part of the complete staging in MOGCT.The paper discusses various aspects of the clinical value of comprehensive surgical staging in MOGCTs.
ABSTRACT
Intracranial germ cell tumors (iGCTs) are a heterogeneous group of tumors with peculiar characteristics clearly distinguished from other brain tumors of neuroepithelial origin. Diverse histology, similarity to gonadal GCT, predilection to one sex, and geographic difference in incidence all present enigmas and fascinating challenges. The treatment of iGCT has advanced for germinoma to date; thus, clinical attention has shifted from survival to long-term quality of life. However, for non-germinomatous GCT, current protocols provide only modest improvement and more innovative therapies are needed. Recently, next-generation sequencing studies have revealed the genomic landscape of iGCT. Novel mutations in the KIT-RAS-MAPK and AKT-MTOR pathways were identified. More importantly, methylation profiling revealed a new method to assess the pathogenesis of iGCT. Molecular research will unleash new knowledge on the origin of iGCT and solve the many mysteries that have lingered on this peculiar neoplasm for a long time.
Subject(s)
Brain Neoplasms , Germ Cells , Germinoma , Gonads , Incidence , Methods , Methylation , Neoplasms, Germ Cell and Embryonal , Quality of Life , Therapies, InvestigationalABSTRACT
Objective To improve diagnosis accuracy of pineocytoma (PC) by joint analysis of CT,MRI imaging features and differential diagnosis with other lesions in pineal region.Methods Totally 6 pineocytoma patients confirmed surgically and pathologically had their clinical history,CT and MRI data collected and analyzed on lesion morphology,cystic solid changes,existence of necrosis,complications of hemorrhage and or calcification,MRI and enhanced scan of solid component,complications with hydrocephalus and etc.Results Plain scan found 1 case of solid nodule and 5 cases of cystic-solid nodules,2 cases with clearly-bordered lesions and 4 one not as well as 4 cases with significant hydrocephalus and 2 ones with light hydrocephalus.Enhanced scan showed 5 cases of moderate to marked enhancement and one case with no obvious enhancement.CT examination proved there were 1 case of calcification and 1 case of hemorrhage.Conclusion Pineocytoma has the characteristics of benign tumor,and has to be differentiated with other tumors frequently occurring in this region in case of obvious clinical signs due to crushing brain parenchyma or blocking aqueduct cerebri by oversized lesions.
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Background: An underlying identifiable organic cause is present in up to 50% cases of central precocious puberty in male patients. Case characteristics: A 7-years-8-months-old presented with delayed puberal development. Analytical examinations showed suppressed basal and stimulated levels of testosterone, LH and FSH. Abdominal ultrasound and contrast cranial magnetic resonance results were initially negative. Outcome: Germinoma was found on cranial computer tomography. Conclusion: There is often a wide time-lapse between symptoms and diagnosis of germinoma, so frequent monitoring is vital.
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Central nervous system germ cell tumor is a rare but important tumor in childhood brain tumors. It requires a multidisciplinary approach to increase survival and promote quality of life, and all three treatment modalities including surgery, radiotherapy and chemotherapy has its own distinct role for germ cell tumor. For germinoma, radiotherapy alone can cure the disease but, the effort to limit the long term toxicity and the proper combination of chemotherapy and radiotherapy are under investigation. Craniospinal irradiation is reserved only for the disseminated germinoma or nongerminomatous germ cell tumor (NGGCT). For germinoma, craniospinal irradiation of 20 to 24 Gy is sufficient to control microscopic disease in the spinal axis. Chemotherapy and radiotherapy composed of 30 to 40 Gy of local field radiotherapy and 20 to 24 Gy of whole ventricular irradiation are required for localized germinoma, but the proper combination of two modalities has yet to be defined. For NGGCT, both the chemotherapy and radiotherapy should be performed, and survival rate is substantially increasing with modern treatment protocols. The omission of craniospinal irradiation is being tried for the localized NGGCT in international cooperative group trials. Surgery has its role for the resection of residual disease after the treatment, and the extent of resection in NGGCT has the prognostic implication. Bifocal germ cell tumors and basal ganglia germ cell tumor have distinctive clinical course and mandate special attention. To advance clinical and biological perspectives in central nervous germ cell tumor, the cooperation and communication of the multidisciplinary specialists are essential.
Subject(s)
Axis, Cervical Vertebra , Basal Ganglia , Brain Neoplasms , Central Nervous System , Clinical Protocols , Craniospinal Irradiation , Drug Therapy , Germ Cells , Germinoma , Neoplasms, Germ Cell and Embryonal , Quality of Life , Radiotherapy , Specialization , Survival RateABSTRACT
Objective To investigate the treatment outcomes of 41 patients with suprasellar and pineal germinoma who are treated in our department in recent 18 years,and to explore related therapeutic strategies.Methods A total of 41 patients with concurrent suprasellar and pineal germinoma who were treated in our department from January 1996 to August 2013 were enrolled.There were 35 male patients and 6 female patients,and the median age was 16 years (range 5-39 years).Five patients had pathologically confirmed germinoma and 36 patients had clinically diagnosed germinoma.Conventional radiotherapy was performed for 33 patients, and intensity-modulated radiotherapy was performed for 8 patients.Combined-modality chemoradiotherapy was performed for the 5 patients with pathologically diagnosed germinoma.The 6 MV X-ray was applied for radiotherapy;6 patients received whole ventricular irradiation and a boost in tumor region, 16 received whole-brain radiotherapy and a boost in tumor region,and 19 received craniospinal irradiation and a boost in tumor region.The median radiation dose for tumor region was 45.0 Gy (37.8-50.0 Gy),and the median dose for prophylactic irradiation was 25.0 Gy (17.8-35.0 Gy).The survival was caculated using Kaplan-Meier method.Results The number of patients followed were 26 at 5-years.The 5-year overall survival rate and relapse-free survival rate were 95% and 85%,respectively.Relapse and metastasis were noted in 8 patients,and 3 of them died.There were 4 patients with spinal cord metastasis,1 patient with ventricular dissemination,and 3 patients with periventricular relapse.Among the 22 patients who did not undergo spinal irradiation,4(18.2%) experienced spinal cord metastasis,and all the other 19 patients who underwent spinal irradiation did not experience spinal cord metastasis.The 8 patients undergoing intensity-modulated radiotherapy and the 5 patients undergoing combined-modality chemoradiotherapy did not experience treatment failure during follow-up.Conclusions Radiotherapy for intracranial germinoma has a good therapeutic effect,and the patients with suprasellar and pineal germinoma who do not undergo spinal cord irradiation have a high failure rate.It is suggested to perform spinal cord irradiation for patients with suprasellar and pineal lesions.
ABSTRACT
Central nervous system germ cell tumor is a rare but important tumor in childhood brain tumors. It requires a multidisciplinary approach to increase survival and promote quality of life, and all three treatment modalities including surgery, radiotherapy and chemotherapy has its own distinct role for germ cell tumor. For germinoma, radiotherapy alone can cure the disease but, the effort to limit the long term toxicity and the proper combination of chemotherapy and radiotherapy are under investigation. Craniospinal irradiation is reserved only for the disseminated germinoma or nongerminomatous germ cell tumor (NGGCT). For germinoma, craniospinal irradiation of 20 to 24 Gy is sufficient to control microscopic disease in the spinal axis. Chemotherapy and radiotherapy composed of 30 to 40 Gy of local field radiotherapy and 20 to 24 Gy of whole ventricular irradiation are required for localized germinoma, but the proper combination of two modalities has yet to be defined. For NGGCT, both the chemotherapy and radiotherapy should be performed, and survival rate is substantially increasing with modern treatment protocols. The omission of craniospinal irradiation is being tried for the localized NGGCT in international cooperative group trials. Surgery has its role for the resection of residual disease after the treatment, and the extent of resection in NGGCT has the prognostic implication. Bifocal germ cell tumors and basal ganglia germ cell tumor have distinctive clinical course and mandate special attention. To advance clinical and biological perspectives in central nervous germ cell tumor, the cooperation and communication of the multidisciplinary specialists are essential.