• Brain Tumor Research and Treatment
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• v.6 no.2
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• pp.73-77
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• 2018

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.

• Journal of Korean Neurosurgical Society
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• v.30 no.10
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• pp.1245-1249
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• 2001

Germinomas of the central nervous system are rare embryonal tumors(accounting for less than 1% of intracranial neoplasms) that may be located in the pineal region, in the floor of the third ventricle, or in the suprasellar area. We report a case of germinoma developed in periventricular deep white matter without pineal region tumors or suprasellar masses. The 19-year-old male patient presented with slowly progressing headache, dizziness, photophobia, and dysarthria. Initial brain MRI revealed a irregular and dense enhancement from lateral ventricles to 4th ventricle. The stereotactic biopsy of tumor and histologic examination revealed the germinoma. Craniospinal axis radiation therapy was performed. After radiation therapy patient was improved and no neurologic sequelae was seen at discharge. Periventricular germinomas without pineal or suprasellar lesion are very rare. The radiation therapy, as in our case, is beneficial as with other intracranial germinomas. Stereotactic biopsy of periventricular germinoma provides precise pathologic diagnosis and thus allows more specific management.

• Journal of Korean Neurosurgical Society
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• v.57 no.5
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• pp.315-322
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• 2015

Intracranial germ cell tumors (ICGCT) occur in 2-11% of children with brain tumors between 0-19 years of age. For treatment of germinoma, relatively low radiation doses with or without chemotherapy show excellent 10 year survival rate of 80-100%. Past studies showed that neoadjuvant chemotherapy combined with focal radiotherapy resulted in unacceptably high rates of periventricular tumor recurrence. The use of generous radiation volume which covers the whole ventricular space with later boost treatment to primary site is considered as standard treatment of intracranial germinomas. For non-germinomatous germ cell tumors (NGGCT), 10-year overall survival rate is still much inferior than that of intracranial germinoma despite intensive chemotherapy and high-dose radiotherapy. Craniospinal radiotherapy combined with cisplatin-based chemotherapy provides the best treatment outcome for NGGCT; 60-70% of overall survival rate. There is a debate on the surgical role whether surgery can contribute to improved treatment outcome of NGGCT when added to combined chemoradiotherapy. Because higher dose of radiotherapy is required for treatment of NGGCT than for germinoma, it is tested whether whole ventricular irradiation can replace craniospinal irradiation in intermediate risk group of NGGCT to minimize radiation-related late toxicity in the recent studies. To minimize the treatment-related neural deficit and late sequelae while maintaining long-term survival rate of ICGCT patients, optimized administration of chemotherapy and radiotherapy should be selected. Use of technically upgraded radiotherapy modalities such as intensity-modulated radiotherapy or proton beam therapy is expected to bring an improved neurocognitive outcome with longitudinal assessment of the patients.

• Radiation Oncology Journal
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• v.15 no.3
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• pp.207-213
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• 1997

Purpose : Intracranial germinoma is the most radiocurable tumor of theprimary intracranial neoplasm. But, the optimum radiation dose and target volume remain controversial In this retrospective study, we analysed the spreading pattern at presentation and the pattern of the failure and survival of intracranial germinoma, Materials and Methods : From 1989 to 1996, 23 Patients were treated for intracranial germinoma at Department of Radiation Oncology, Twenty-one Patients were treated at their initial Presentation and 2 Patients were treated for recurrent disease. Six patients had multiple tumor masses on MRI and 7patients had ventricular seeding on MRI. The examination of cerebrospinal fluid cytology was done in 15 patients and 3 out of 15 patients had positive cerebrospinal cytology. In tumor marker study of $\alpha-FP\;and\;\beta-hCG$, 6 patients had mildly elevated $\beta-hCG$ in serum or cerebrospinal fluid. Twentyone Patients were treated with whole craniospinal axis irradiation and 2 Patients were given whole ventricular radiation therapy. The total dose was ranged between 4500cGy and 5600cGy to primary tumor site (median 5580cGy) Dose to the entire ventricular system ranged from 1980cGy to 3960 cGy (median 2700cGy) and dose to the spinal axis ranged from 2160cGy to 3900cGy (median 2700cGy) Results : Of 23 patients, 21 Patients are alive without evidence of diseasefor median 4 years follow-up. One Patient who had markedly elevated $\alpha-FP\;and\;\beta-hCG$, suffered from Persistent disease after radiation therapy and received 2 cycles of chemotherapy. She died 9 months after chemotherapy One patient who developed ventricular seeding after gamma-knife was treated with whole craniospinal irradiation, he died after 1 year due to probably brain necrosis. The hematologic toxicity of 3 or 4 grade were seen in 7 patients and patient's endocrinologic dysfunction was not deteriorated after radiation therapy. One patient had been treated with growth hormone replacement due to short stature. Conclusions : This retrospective study has confirmed the excellent result of radiation therapy in intracranial germinoma. The complication rate during or after radiation therapy is considered within acceptable range. ft is necessary to further investigate the optimal dose and treatment volume of radiation therapy. The role of chemotherapy in the treatment of intracranial germinoma should be further investigated.

• Journal of Korean Neurosurgical Society
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• v.61 no.3
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• pp.333-342
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• 2018

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.

• The Korean Journal of Cytopathology
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• v.7 no.2
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• pp.177-184
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• 1996

Intraoperative cytologic examination of intracranial tumors using crush preparation provides useful information in operative decision making. The diminutive nature of many biopsy specimens, particularly those obtained by stereotactic neurosurgical procedures emphasizes the importance of combining the cytologic smear method with conventional frozen section interpretation. The great advantage of the cytologic smear method resides in its suitability for the study of minute fragments of tissue, allowing retention of the majority of the specimen for optimal processing. We present the cytologic features of 3 cases of intracranial germ cell tumors(2 germinomas and 1 endodermal sinus tumor), using crush preparation during intraoperative diagnosis and compare them with histologic findings. The cytologic features of the germ cell tumors were similar to those of the respective gonadal counterparts. The cytologic differential diagnosis of both types of germ cell tumors is described.

• Radiation Oncology Journal
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• v.20 no.2
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• pp.93-99
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• 2002

Purpose : We intended to decrease late CNS reaction after radical radiotherapy for an intracranial germinoma by using combined neoadjuvant chemotherapy and involved-field radiotherapy. The efficacy in terms of its acute toxicity and short-term relapse patterns was analyzed. Materials and Methods : Eighteen patients were treated with combined neoadjuvant chemotherapy and radiotherapy between 1995 and 2001. The chemotherapy regimen used was the Children's Cancer Group (CCG) 9921A (cisplatin, cyclophosphamide, VP-16, vincristine) for 5 patients younger than 16 years, BEP (bleomycin, VP-16, cisplatin) for 12 patients, and EP (VP-16, cisplatin) for 1 patient. The radiotherapy covered the whole craniospinal axis for 5 patients, the whole brain for 1, and the partial brain (involved field) for 12. the primary lesion received tumour doses between 3,960 and 5,400 cGy. Results : The male to female ratio was 16:2 and the median age was 16 years old. The tumors were located in the pineal gland in 12 patients, in the suprasellar region in 1, in the basal ganglia In 1, in the thalamus in 1. Three patients had multiple lesions and ventricular seedings were shown at MRI. In 3 patients, tumor cells were detected in the cerebrospinal fluid and MRI detected a spinal seeding in 2 patients. The response to neoadjuvant chemotherapy was complete remission in 5 patients, partial remission in 12, and no response in 1. However, after radiotherapy, all except 1 patient experienced complete remission. The toxicity during or after chemotherapy greater than or equal to grade III was remarkable; hematologic toxicity was observed in 11 patients, liver toxicity in none, kidney toxicity in none, and gastrointestinal toxicity in one. One patient suffered from bleomycin-induced pneumonitis. Radiotherapy was therefore stopped and the patient eventually died of respiratory failure. The other 17 are alive without any evidence of disease or relapse during an average of 20 months follow-up. Conclusion : A high response rate and disease control was experienced, which was the same as observed other studies and the morbidity from chemotherapy-induced toxicity was similar. With these results, the results from adjuvant chemotherapy and involved-field radiotherapy cannot be concluded to be equal to those from extended-field radiotherapy. The long term follow-up study on later complications are required in order to draw definite conclusions on the optimal management with minimum side effects.

• Journal of Korean Neurosurgical Society
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• v.30 no.3
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• pp.366-370
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• 2001

Objective : The authors present three cases of brain tumors in which epidural hematomas(EDHs) were developed postoperatively in the remote areas from craniotomy sites. The preventive tactics as well as possible mechanisms of development of remote EDH are discussed. Material and Methods : The magnetic resonance imagings of three patients revealed a left lateral ventricular mass located just aside of foramen Monro in a 27-year-old male, a large cystic mass in the temporal lobe in a 35-year-old male, and a partially calcified pineal mass in a 27-year-old male patient. The surgical removals of these tumors were performed without any noticeable events during surgery via left frontal transcortical transventricular approach for lateral ventricular tumor, left temporal craniotomy for cystic temporal tumor, and right occipital transtentorial approach for pineal tumor. Results : Postoperative EDHs remote from the sites of craniotomy were detected by the immediate postoperative computerized tomographic scans. We obtained good outcomes without any morbidity in all three patients with emergent evacuation of the hematoma. The pathologic diagnoses were lateral ventricular ependymoastrocytoma, temporal craniopharyngioma and mixed germinoma of the pineal region. Conclusion : It is postulated that a sudden reduction of intracranial pressure(ICP) at the time of tumor removal may strip the dura from the inner table of the skull to cause EDH from the remote site of craniotomy. Gradual reduction of ICP with slow drainage of cerebrospinal fluid before tumor removal as well as lowering the head position of patient during surgery might be helpful for preventing this unusual complication.

• Clinical and Experimental Pediatrics
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• v.48 no.2
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• pp.186-190
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• 2005

Purpose : The purpose of this study was to evaluate the responses and toxicities of risk-adapted chemotherapy in pediatric intracranial germ cell tumors(IC-GCT). Methods : Fourteen patients who were diagnosed as IC-GCT from October 2002 to December 2003 received chemotherapy as an initial treatment modality. The low risk(LR) group was defined as follows : Pure germinoma and normal AFP level. Beta-hCG level 50 mIU/mL or less. The others belonged to the high risk(HR) group. Chemotherapy was composed of cisplatin, cyclophosphamide, etoposide and vincristine. Double doses of cisplatin and cyclophosphamide was used in HR patients. Results : Pathologic confirmation was done in all but one. Median age at diagnosis was 11.6 yr (1.2-18.7 yr), and nine patients belonged to the HR group. Tumor markers were normalized after chemotherapy in all patients whose tumor markers had been elevated. Four LR patients(80 percent) and seven HR patients(77.8 percent) showed complete response(CR) at the end of chemotherapy. An additional two of the three patients with partial response(PR) achieved CR after radiation therapy (RT), and the remaining one relapsed before RT. Four LR and all HR patients experienced infectious episodes that required hospitalization. Four of the nine HR patients(44.4 percent) suffered from tinnitus, three of whom developed sensorineural hearing loss. All but one are surviving, event-free, with a median follow-up of 13.9 mo(8.1-22.3 mo). Conclusion : Risk-adapted cisplatin-based chemotherapy was effective even in HR patients, but regimen modification seems to be necessary to avoid an unacceptably high toxicity rate.

• Investigative Magnetic Resonance Imaging
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• v.1 no.1
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• pp.119-124
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• 1997

Purpose: To assess the utility of magnetic resonance(MR) cerebral blood volume (CBV) map in the evaluation of brain tumors. Materials and Methods: We performed perfusion MR imaing preoperatively in the consecutive IS patients with intracranial masses(3 meningiomas, 2 glioblastoma multiformes, 3 low grade gliomas, 1 lymphoma, 1 germinoma, 1 neurocytoma, 1 metastasis, 2 abscesses, 1 radionecrosis). The average age of the patients was 42 years (22yr -68yr), composed of 10 males and S females. All MR images were obtained at l.ST imager(Signa, CE Medical Systems, Milwaukee, Wisconsin). The regional CBV map was obtained on the theoretical basis of susceptibility difference induced by first pass circulation of contrast media. (contrast media: IScc of gadopentate dimeglumine, about 2ml/sec by hand, starting at 10 second after first baseline scan). For each patient, a total of 480 images (6 slices, 80 images/slice in 160 sec) were obtained by using gradient echo(CE) single shot echo-planar image(EPI) sequence (TR 2000ms, TE SOms, flip angle $90^{\circ}$, FOV $240{\times}240mm,{\;}matrix{\;}128{\times}128$, slice-thick/gap S/2.S). After data collection, the raw data were transferred to CE workstation and rCBV maps were generated from the numerical integration of ${\Delta}R2^{*} on a voxel by voxel basis, with home made software (${\Delta}R2^{*}=-ln (S/SO)/TE). For easy visual interpretation, relative RCB color coding with reference to the normal white matter was applied and color rCBV maps were obtained. The findings of perfusion MR image were retrospectively correlated with Cd-enhanced images with focus on the degree and extent of perfusion and contrast enhancement. Results: Two cases of glioblastoma multiforme with rim enhancement on Cd-enhanced Tl weighted image showed increased perfusion in the peripheral rim and decreased perfusion in the central necrosis portion. The low grade gliomas appeared as a low perfusion area with poorly defined margin. In 2 cases of brain abscess, the degree of perfusion was similar to that of the normal white matter in the peripheral enhancing rim and was low in the central portion. All meningiomas showed diffuse homogeneous increased perfusion of moderate or high degree. One each of lymphoma and germinoma showed homogenously decreased perfusion with well defined margin. The central neurocytoma showed multifocal increased perfusion areas of moderate or high degree. A few nodules of the multiple metastasis showed increased perfusion of moderate degree. One radionecrosis revealed multiple foci of increased perfusion within the area of decreased perfusion. Conclusion: The rCBV map appears to correlate well with the perfusion state of brain tumor, and may be helpful in discrimination between low grade and high grade gliomas. The further study is needed to clarify the role of perfusion MR image in the evaluation of brain tumor.