The event-free survival (EFS) for pediatric acute lymphoblastic leukemia (ALL) has shown remarkable improvement in the past several decades. In Korea also, a recent study showed 10-year EFS of 78.5%. Much of the improved outcome for pediatric ALL stems from the accurate identification of prognostic factors, the designation of risk group based on these factors, and treatment of appropriate duration and intensity according to risk group, done within the setting of cooperative clinical trials. The schema of first-line therapy for ALL remains mostly unchanged, although many groups have now reported on the elimination of cranial irradiation in all patients with low rates of central nervous system relapse. Specific high risk subgroups, such as Philadelphia chromosome-positive (Ph+) ALL and infant ALL continue to have significantly lower survival than other ALL patients. The introduction of tyrosine kinase inhibitors into therapy has led to enhanced outcome for Ph+ ALL patients. Infant ALL patients, particularly those with MLL rearrangements, continue to have poor outcome, despite treatment intensification including allogeneic hematopoietic cell transplantation. Relapsed ALL is a leading cause of mortality in pediatric cancer. Recent advances in immunotherapy targeting the CD19 of the ALL blast have shown remarkable efficacy in some of these relapsed and refractory patients. With improved survival, much of the current focus is on decreasing the long-term toxicities of treatment.
Phi, Ji Hoon;Wang, Kyu-Chang;Lee, Ji Yeoun;Kim, Seung-Ki
Journal of Korean Neurosurgical Society
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v.57
no.6
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pp.408-414
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2015
Moyamoya-like vasculopathy develops in association with various systemic diseases and conditions, which is termed moyamoya syndrome. Relatively common diseases and conditions are related to moyamoya syndrome, including neurofibromatosis type 1, Down syndrome, thyroid disease, and cranial irradiation. Moyamoya syndrome shares phenotypical characteristics with idiopathic moyamoya disease. However, they differ in other details, including clinical presentations, natural history, and treatment considerations. The study of moyamoya syndrome can provide clinicians and researchers with valuable knowledge and insight. Although it is infrequently encountered in clinical practice, moyamoya-like vasculopathy can severely complicate outcomes for patients with various underlying diseases when the clinician fails to expect or diagnose moyamoya syndrome development. Furthermore, moyamoya syndrome could be used as a doorway to more enigmatic moyamoya disease in research. More comprehensive survey and investigation are required to uncover the secrets of all the moyamoya-like phenomena.
The authors encountered a case of simultaneous radiation-induced multiple meningiomas and ventriculoperitoneal [VP] shunt-related pneumocephalus. A 35-year-old man, who had undergone surgery for medulloblastoma 21 years previously and subsequently received high dose craniospinal irradiation with adjuvant chemotherapy and later underwent a VP shunt because of hydrocephalus, presented with a severe headache and weakness of both lower extremities. Computed tomography showed an air pocket lesion in the left temporal lobe and a large amount of pneumocephalus with a bony defect of the left tegmen tympani. In addition, a 3 cm sized well enhancing mass was noted in the in the right middle cranial fossa and additional small enhancing nodule in the left frontal pole. He was treated by left temporal craniotomy and repair of the bony and dural defects of the left tegmentum tympanum through extradural and intradural approaches, respectively. Afterwards, he underwent right temporal craniotomy and gross total removal of a rapidly growing right middle fossa mass and a left frontal mass. The histological examination was consistent with atypical meningioma, WHO grade II. In conclusion, physicians have to consider the serious long term complications of high dose radiation therapy and VP shunt placement and need to perform the neuroradiologic follow-up after such treatments for several decades.
Purpose : Since the mid cranial fossa is composed of various thickness of bone, the tissue inhomogeneity caused by bone would produce dose attenuation in cobalt-60 gamma knife irradiation. The correction factor for bone attenuation of cobalt-60 which is used for gamma knife source is -3.5$\%$. More importantly, nearly all the radiosurgery treatment planning systems assume a treatment volume of unit density: any perturbation due to tissue inhomogeneity is neglected, This study was performed to confirm the bone attenuation in mid cranial fossa using gamma knife. Materials and Methods : Computed tomography was performed after Leksell stereotactic frame had been liked to the Alderson Rando Phantom (human phantom) skull area. Kodak X-omat V film was inserted into two sites of pituitary adenoma point and acoustic neurinoma point, and irradiated by gamma knife with 14mm and 18mm collimator. An automatic scanning densitometer with a 1mm aperture is used to measure the dose profile along the x and y axis. Results : Isodose curve constriction in mid cranial fossa is observed with various ranges. Pituitary tumor point is greater than acoustic neurinoma point (0.2-3.0 mm vs 0.1-1.3 mm) and generally 14 mm collimator is greater than 18mm collimator (0.4-3.0 mm vs. 0.2-2.2 mm) Even though the isodose constriction is found, constriction of 50$\%$ isodose curve which is used for treatment reference line does not exceed 1 mm. This range is too small to influence the treatment planning and treatment results. Conclusion : Radiosurgery planning system of gamma knife does not show significant error to be corrected without consideration of bone attenuation.
Purpose: Brain metastases are present in approximately 10-16% of small cell lung cancer patients at diagnosis. Brain metastasis is an important clinical problem associated with increasing the survival rate, with a cumulative incidence of up to 80% in patients surviving 2 years. Prophylactic cranial irradiation(PCI) reduces the incidence of brain matastasis and may prolong survival in patients with limited small-cell lung cancer who achieved complete remission. This study was performed to analyze the incidence of brain metastasis, survival and clinical aspects after PCI in patients with limited small-cell lung cancer who achieved complete remission. Methods : Between 1989 and 1999, forty-two patients with limited small-cell lung cancer who achived achieved complete remission after therapy were enrolled into this study retrospectively. All patients received etoposide and cisplatin(VPP) alternating with cytoxan, adriamycin, and vincristine(CAV) every 3 weeks for at least 6 cycles initially. All patients received thoracic radiotherapy: concurrent(38.1%) and sequential(61.9%). All patients received late PCI. Results : Most patients(88.1%) were men, and the median age was 58 years. The median follow-up duration was 18.1 months. During the follow-up period, 57.1% of the patients developed relapse. The most frequent site of relapse was chest(35.7%), followed by brain(14.3%), liver(11.9%), adrenal gland(44%), and bone(2.2%). With the Kaplan-Meier method, the average disease-free interval was 1,090 days(median 305 days). The average time to development of brain relapse after PCI and other sites relapse(except brain) were 2,548 days and 1,395 days(median 460 days), respectively. The average overall survival was 1,233 days(median 634 days, 21.1 months), and 2-year survival rates was 41.7%. The average overall survival in the relapse group was 642 days(median 489 days) and in the no relapse group was 2,622 days(p<0.001). The average overall survival in the brain relapse group was 928 days(median 822 days) and in the no brain relapse group was 1,308 days(median 634 days)(p=0.772). In most patients(85.7%), relapse(except brain) or systemic disease was the usual cause of death. Brain matastasis was the cause of death in 14.3% of the cases. Conclusions : We may conclude that PCI reduces and delays brain metastasis in patients with limited small cell lung cancer who achieved complete remission. We found decreased survival in relapse group but, no significant survival difference was noted according to brain matastasis. And relapse(except brain) or systemic disease was the usual cause of death. In order to increase survival, new treatment strategies for control methods for relapse and systemic disease are required.
Background: Factors associated with the prognosis of patients with small cell lung cancer (SCLC) is relatively unknown, than of those with non-small cell lung cancer. This study was undertaken to identify the prognostic factors of SCLC. Methods: The medical records of 333 patients diagnosed with SCLC at tertiary hospital from January 1, 2008, to December 31, 2012 were retrospectively reviewed. Patients were categorized by age (${\leq}65$ years vs. >65 years) and by extent of disease (limited disease [LD] vs extensive disease [ED]). Overall survival and progression free survival rates were determined. Factors associated with prognosis were calculated using Cox's proportional hazard regression model. Results: Most baseline characteristics were similar in the LD and ED groups. Eastern Cooperative Oncology Group (ECOG) performance status (PS), first chemotherapy regimen, and prophylactic cranial irradiation (PCI) differed significantly in patients with LD and ED. Mean ECOG PS was significantly lower (p<0.001), first-line chemotherapy with etoposide-cisplatin was more frequent than with etoposide-carboplatin (p<0.001), and PCI was performed more frequently (p=0.019) in LD-SCLC than in ED-SCLC. Prognosis in the LD group was better in younger (${\leq}65$ years) than in older (>65 years) patients, but prognosis in the ED group was unrelated to age. Conclusion: This study showed that overall survival (OS) was significantly improved in younger than in older patients with LD-SCLC. Univariate and multivariate analyses showed that age, PCI and the sum of cycles were significant predictors of OS in patients with LD-SCLC. However, prognosis in the ED group was unrelated to age.
Kim, Eui-Hyun;Park, Yong-Sook;Chang, Jong-Hee;Chang, Jin-Woo;Park, Yong-Gou
Journal of Korean Neurosurgical Society
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v.38
no.3
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pp.184-189
/
2005
Objective : Hemangioblastomas are highly vascular and benign neoplasm of the central nervous system[CNS]. They can often be found as multiple lesions, as is commonly observed in von Hippel-Lindau[VHL] disease. The aim of this study is to determine the proper management for multiple hemangioblastomas. Methods : Since 1990, 78cases of hemangioblastoma have been encountered. Among these, 9cases were multiple hemangioblastomas that were treated with surgical resection with or without radiosurgery. The medical, radiological, surgical and histological records were reviewed retrospectively and analyzed statistically. Results : Nine patients presented with multiple hemangioblastomas and were diagnosed as VHL disease. The mean follow-up duration was 75.7months [$6.6{\sim}159.2months$] after the first surgical treatment. Three patients were treated with surgical resection alone and six patients were treated by both surgical resection and radiosurgery. Twenty-one surgical procedures [13 surgical resections and 8 radiosurgery] were performed. One patient required ventriculoperitoneal shunt and a posterior fossa decompressive craniectomy because of post-radiation brain swelling. Another patient refused additional treatment for the newly developed lesions after the successful treatment of initial lesions. The other patient who presented with numerous lesions in the whole brain and spine underwent cranio-spinal irradiation. Remaining patients showed good results. Conclusion : The surgical outcomes for the patients with a single lesion of the CNS hemangioblastoma are favorable. However. the treatment of multiple hemangioblastoma is more difficult, and should be treated by surgical resection and radiosurgery with careful consideration.
Joo, Hyunjin;Choi, Hoon;Yang, Kwang-hee;Keum, Dong-kwon;Kim, Hee sun
Journal of Radiation Protection and Research
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v.40
no.3
/
pp.168-173
/
2015
The present investigation was planned to estimate potential possibility of striped field mice, Apodemus agrarius coreae (A. a. coreae), as a biological dosimeter in radio-environmental ecology. We bred captured wild A. a. coreae at laboratory and classified taxonomically based on external, cranial and tooth characters. Organ weights and splenocytic apoptosis were observed in order to establish a basic data on radiation biology of A. a. coreae (male, 40 weeks old). The biological effects was observed at 24hrs following irradiation (doses : 0, 0.5, 1, 2 Gy, dose rate : $0.8Gymin^{-1}$, $^{137}Cs$). Only thymus weights was significantly decreased. Splenocytic apoptosis was increased after irradiation. But splenocytic apoptosis was decreased in 0.5 Gy ${\gamma}$-irradiated mice compared to those of 0, 1, 2 Gy (P < 0.05). These data suggested that events in thymus and spleen of Korean dark-striped field mice, A. a. coreae THOMAS, could be a potential radio-biological indicator in human environments.
Kim, Hae-Kyu;Kim, Seong-Tae;Jung, Jin-Woo;Keoun, Jae-Young;Kim, In-Se;Chung, Kyoo-Sub
The Korean Journal of Pain
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v.5
no.2
/
pp.258-262
/
1992
Herpes zoster is an acute infectious viral disease which affects the posterior spinal root ganglion of the spinal nerve. A single posterior spinal root ganglion or a small number of adjacent ones may be affected, usually on the same side. The corresponding ganglia of the cranial nerve may also be similarly affected. The causative virus, varicella zoster, belongs to the group of host-specific DNA viruses. Postherpetic neuralgia is a continuation of herpes zoster in older patients. Although spontaneous resolution of herpes zoster may be expected in most patients, a significant number experience intractable pain. Postherpetic neuralgia is one of the most difficult problems encountered by physicians. There are many methods for management of postherpetic neuralgia, but there is no method that results in complete remission. Laser has lately come into use to reduce several acute or chronic pains. In order to determine the degree of pain relief by laser, 27 patients of postherpetic neuralgia were irradiated with He Ne, Infrared, and $CO_2$ combine scan moded lasers two to three times per week. The results were as follows: 1) The most frequent site was thoracic vertebral nerve area. 2) Patients younger than 70 years of age showed an improvement rate of 57% vs 27% for those patients older than 70 years of age. 3) Laser therapy proved effective of those patients who received the laser treatment within one month of the onset of the disease. 4) For those patients who received treatment within one month of the disease and reflecting a 50% improvement rate, the average irradiation time was 5.7.
Jo, Kwang-Wook;Kong, Doo-Sik;Lim, Do-Hoon;Ahn, Yong-Chan;Nam, Do-Hyun;Lee, Jung-Il
Journal of Korean Neurosurgical Society
/
v.50
no.2
/
pp.99-102
/
2011
Objective : The purpose of this retrospective study was to evaluate the outcome of gamma knife radiosurgery (GKRS) and/or whole brain radiation therapy (WBRT) for the treatment of small cell lung carcinoma (SCLC) metastasis to the brain. Methods : From 2000 to 2010, 50 patients underwent GKRS for metastatic brain lesions originating from SCLC. Among these patients, 11 received prophylactic cranial irradiation (PCI) before the development of metastatic lesions (PCI group), and GKRS was performed as an initial treatment for newly diagnosed lesions in 12 patients who had not received PCI (primary GKRS group). In addition, GKRS was performed as a salvage treatment for progressive lesions after WBRT in 27 patients (salvage GKRS group). The medical records and imaging data of all patients were retrospectively analyzed. Results : The overall survival of the 50 patients was 20.8 months (range 1-53) after the diagnosis of primary tumor and 12.0 months (range 1-47) after the development of cerebral metastasis. Median survival after GKRS was 4.8 months (range 1-15) in the PCI group, 4.6 months (range 0-18) in the primary GKRS group, and 7.6 months (range 0-33) in the salvage GKRS group. Further treatment for progressive lesions after GKRS was necessary in 15 patients, after a mean interval of 3.8 months. Causes of death were systemic organ failure in 15 patients, deterioration of neurological state in 13 patients, and unknown or combined causes in 16 patients. The local control rate of the lesions treated with GKRS was 76.4% (decreased in 13 patients and stable in 16 patients at the final imaging follow-up (mean 5.60 months). Conclusion : GKRS is an effective local treatment for brain metastasis from SCLC both as an initial treatment for newly diagnosed lesions after PCI and as a salvage treatment for recurrent or progressive lesions. However, the survival benefit is not significant because most patients die of systemic multi-organ failure with a short life expectancy.
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