• Journal of Korean Neurosurgical Society
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• 제61권3호
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• pp.292-301
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• 2018

Medulloblastoma is the most common malignant brain tumor of childhood and remains a major cause of cancer related mortality in children. Significant scientific advancements have transformed the understanding of medulloblastoma, leading to the recognition of four distinct clinical and molecular subgroups, namely wingless (WNT), sonic hedgehog, group 3, and group 4. Subgroup classification combined with the recognition of subgroup specific molecular alterations has also led to major changes in risk stratification of medulloblastoma patients and these changes have begun to alter clinical trial design, in which the newly recognized subgroups are being incorporated as individualized treatment arms. Despite these recent advancements, identification of effective targeted therapies remains a challenge for several reasons. First, significant molecular heterogeneity exists within the four subgroups, meaning this classification system alone may not be sufficient to predict response to a particular therapy. Second, the majority of novel agents are currently tested at the time of recurrence, after which significant selective pressures have been exerted by radiation and chemotherapy. Recent studies demonstrate selection of tumor sub-clones that exhibit genetic divergence from the primary tumor, exist within metastatic and recurrent tumor populations. Therefore, tumor resampling at the time of recurrence may become necessary to accurately select patients for personalized therapy.

• Journal of Korean Neurosurgical Society
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• 제45권2호
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• pp.107-111
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• 2009

Meningiomas are usually benign neoplasms in which extracranial metastases occur very rarely. We report a case of multiple extracranial metastases of an atypical meningioma following a local recurrence. A 68-year-old man presented with left-side motor weakness and dysarthria for two weeks. A computed tomography (CT) scan and magnetic resonance imaging (MRI) showed an intraventricular tumor. We performed a total mass removal, and the histopathologic findings were consistent with benign meningioma. Eight months later, the meningioma recurred. We performed a reoperation and whole brain radiation therapy postoperatively. The histopathologic findings showed atypical meningioma. Six months later, CT and MRI revealed metastases to multiple vertebrae, lung, ribs and perirenal soft tissue so a decompressive laminectomy with mass removal was performed. The histopathologic findings of the spinal tumors showed atypical meningioma. The results from perirenal biopsies were consistent with metastatic meningioma. In conclusion, extracranial metastasis as well as local recurrence must be considered in atypical or anaplastic meningioma. There must be regular follow-ups. Finally, an evaluation of the chest, abdomen and bone is necessary, especially when related symptoms or signs develop.

• Journal of Korean Neurosurgical Society
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• 제30권10호
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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 Chest Surgery
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• 제36권8호
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• pp.614-618
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• 2003

흉막폐아세포종은 소아에 국한하여 생기는 매우 드문 원발성 악성종양이며 극히 나쁜 예후를 보인다. 주 증상은 흉부불쾌감, 호흡곤란, 반복적인 상기도 감염, 발열, 마른기침, 그리고 흉통 등이다. 흉막폐아세포종은 매우 빠른 진행양상을 보이며, 폐문 및 종격 림프절에 전이될 수 있다. 원격전이는 뇌, 골조직, 그리고 복강 내 장기들에서 보인다 흉막폐아세포종의 치료는 다각적 접근을 요한다 수술에 의한 종괴의 일차적 제거가 우선적인 치료법이나, 종양의 크기나 침범 범위로 인해 일차적으로 수술적 제거가 힘든 경우 수술 전 신보조항암요법으로 종양의 크기를 줄일 수 있으며, 이후 수술적인 완전절제를 고려할 수 있다. 본원에서는 소아에서 발생한 흉막폐아세포종에 대해 신보조항암요법, 수술적 절제 및 보조항암요법을 통해 성공적으로 치료한 2예를 경험하고 이를 보고하고자 한다.

• Journal of Korean Neurosurgical Society
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• 제62권4호
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• pp.476-486
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• 2019

Objective : The functional information of $^{11}C$-methionine positron emission tomography (MET-PET) images can be applied for Gamma knife radiosurgery (GKR) and its image quality may affect defining the tumor. This study conducted the phantom-based evaluation for geometric accuracy and functional characteristic of diagnostic MET-PET image co-registered with stereotactic image in Leksell $GammaPlan^{(R)}$ (LGP) and also investigated clinical application of these images in metastatic brain tumors. Methods : Two types of cylindrical acrylic phantoms fabricated in-house were used for this study : the phantom with an array-shaped axial rod insert and the phantom with different sized tube indicators. The phantoms were mounted on the stereotactic frame and scanned using computed tomography (CT), magnetic resonance imaging (MRI), and PET system. Three-dimensional coordinate values on co-registered MET-PET images were compared with those on stereotactic CT image in LGP. MET uptake values of different sized indicators inside phantom were evaluated. We also evaluated the CT and MRI co-registered stereotactic MET-PET images with MR-enhancing volume and PET-metabolic tumor volume (MTV) in 14 metastatic brain tumors. Results : Imaging distortion of MET-PET was maintained stable at less than approximately 3% on mean value. There was no statistical difference in the geometric accuracy according to co-registered reference stereotactic images. In functional characteristic study for MET-PET image, the indicator on the lateral side of the phantom exhibited higher uptake than that on the medial side. This effect decreased as the size of the object increased. In 14 metastatic tumors, the median matching percentage between MR-enhancing volume and PET-MTV was 36.8% on PET/MR fusion images and 39.9% on PET/CT fusion images. Conclusion : The geometric accuracy of the diagnostic MET-PET co-registered with stereotactic MR in LGP is acceptable on phantom-based study. However, the MET-PET images could the limitations in providing exact stereotactic information in clinical study.

• Tuberculosis and Respiratory Diseases
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• 제73권5호
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• pp.273-277
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• 2012

Paraneoplastic limbic encephalitis (PLE) is a rare syndrome characterized by memory impairment, affective and behavioral disturbances and seizures. Among many different neoplasms known to cause PLE, small cell lung cancer (SCLC) is the most frequently reported. The pathogenesis is not fully understood but is believed to be autoimmune-related. We experienced a patient with typical clinical features of PLE. A 67-year-old man presented with seizure and disorientation. Brain magnetic resonance imaging demonstrated high signal intensity in the bilateral amygdala and hippocampus in flair and T2-weighted images suggestive of limbic encephalitis. Cerebrospinal fluid tapping revealed no evidence of malignant cells or infection. Positron emission tomography/computed tomography showed a lung mass with pleural effusion and a consequent biopsy confirmed the diagnosis of PLE associated with SCLC. The patient was subsequently treated with chemotherapy and neurologic symptoms gradually improved.

• Asian Pacific Journal of Cancer Prevention
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• 제17권3호
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• pp.1565-1570
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• 2016

Background: Central neurocytomas are rare neuronal neoplasms with a favorable prognosis. They are typically located in the lateral ventricles of the brain and mostly histologically correspond to WHO grade II with a Mib 1 labelling index of <2%. Similar tumors located in the cerebral hemispheres and spinal cord, for example, are called "extraventricular neurocytomas". A few tumors histologically show atypia, mitoses, vascular proliferation and/or necrosis and a Mib 1 index >2 % and are designated as "atypical neurocytomas. Aim: The aim of our study was to describe the common as well as unusual morphologic features and the role of various immunohistochemical stains in the diagnosis of these rare tumors. Materials and Methods: We retrieved and reviewed 35 cases diagnosed between 2001 and 2015. Results: Sixty percent of patients were males, and the mean age was 26 years. 31 cases (88.6%) were intraventricular and 4(11.4%) were extraventricular. Histologically, 6 cases (17.1%) were compatible with "atypical neurocytomas". All cases showed the classic morphology comprising nests and sheets of uniform, round cells with uniform round to oval nuclei with finely speckled chromatin and perinuclear cytoplasmic clearing (halos). All cases also showed delicate, fibrillary, neuropil-like matrices. Other common histologic features included capillary-sized blood vessels in a branching pattern in 57.1%, foci of calcification in 34.3% and perivascular pseudorosettes in 20%. Rare findings included Homer-Wright or true rosettes in 8.6% and ganglioid cells in 2.9%. Synaptophysin was the most consistent and valuable marker, being positive in almost all cases. GFAP positivity in tumor cells was seen in 25.7% of cases. Follow up was available in 13 patients. Of these 9 had histologically typical and 4 had atypical tumors. Only 1 (with an atypical neurocytoma) died, probably due to complications of surgery within one month, while 12 (including 3 with atypical neurocytomas) remained alive. Recurrence developed in 1 of these 12 patients (histologically consistent with typical morphology) almost 9 years after surgery. Only 4 patients, including 2 with atypical tumors, received postoperative radiotherapy, all with surgery in 2010 or later. Overall, prognosis was excellent with prolonged, recurrence free survival and most patients, even without receiving radiation therapy, were alive and well for many years, even a decade or more after surgery, without developing any recurrence, indicating the benign nature of these neoplasms.

• 대한영상의학회지
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• 제84권4호
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• pp.970-976
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• 2023

29세 남성에서의 미만성 연수막성 신경교종을 증례 보고한다. 이 질환은 드문 중추신경계 종양으로, 대부분 소아에서 발견되며 성인에서는 소수만 보고되어 있다. 본 환자는 만성 두통으로 내원하여 MRI를 시행하였다. 뇌 MRI에서 경도의 수두증과 다수의 테두리 조영증강을 보이는 병변이 안장위 수조에서 보였으며, FLAIR에서 신호가 억제되지 않는 다수의 비조영증강 낭종성 병변이 양측 기저핵, 시상 및 대뇌에서 관찰되었다. 척추 MRI에서는 요추 및 천추부위의 미만성 연수막 조영증강이 보였다. 생식세포종양의 연수막 파종을 의심하였고 경접형골 종양제거술을 시행 받았다. 병리학 검사에서 미만성 연수막성 신경교종으로 확진되었고, 차세대 염기서열 검사에서 FGFR1 유전자의 돌연변이가 발견되었다. 결론적으로 연수막 결절성 조영증강과 FLAIR에서 신호가 억제되지 않는 다수의 비조영증강 낭종성 뇌 병변이 함께 관찰될 경우 연수막 조영증강을 보이는 여러 다른 질환들과의 감별 진단에 도움이 된다.

• Safety and Health at Work
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• 제8권3호
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• pp.258-266
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• 2017

Background: Welders are exposed to many known and suspected carcinogens. An excess lung cancer risk among welders is well established, but whether this is attributable to welding fumes is unclear. Excess risks of other cancers have been suggested, but not established. We investigated welding cancer risks in the population-based Canadian Census Health and Environmental Cohort. Methods: Among 1.1 million male workers, 12,845 welders were identified using Standard Occupational Classification codes and followed through retrospective linkage of 1991 Canadian Long Form Census and Canadian Cancer Registry (1992-2010) records. Hazard ratios (HRs) were calculated using Cox proportional hazards models based on estimated risks of lung cancer, mesothelioma, and nasal, brain, stomach, kidney, and bladder cancers, and ocular melanoma. Lung cancer histological subtypes and risks by industry group and for occasional welders were examined. Some analyses restricted comparisons to blue-collar workers to minimize effects of potential confounders. Results: Among welders, elevated risks were observed for lung cancer [HR: 1.16, 95% confidence interval (CI): 1.03-1.31], mesothelioma (HR: 1.78, 95% CI: 1.01-3.18), bladder cancer (HR: 1.40, 95% CI: 1.15-1.70), and kidney cancer (HR: 1.30, 95% CI: 1.01-1.67). When restricted to blue-collar workers, lung cancer and mesothelioma risks were attenuated, while bladder and kidney cancer risks increased. Conclusion: Excess risks of lung cancer and mesothelioma may be partly attributable to factors including smoking and asbestos. Welding-specific exposures may increase bladder and kidney cancer risks, and particular sources of exposure should be investigated. Studies that are able to disentangle welding effects from smoking and asbestos exposure are needed.

• Radiation Oncology Journal
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• 제33권4호
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• pp.301-309
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• 2015

Purpose: To identify prognostic factors for disease progression and survival of patients with extracranial oligometastatic breast cancer (EOMBC), and to investigate the role of radiation therapy (RT) for metastatic lesions. Materials and Methods: We retrospectively reviewed the medical records of 50 patients who had been diagnosed with EOMBC following standard treatment for primary breast cancer initially, and received RT for metastatic lesions, with or without other systemic therapy between January 2004 and December 2008. EOMBC was defined as breast cancer with five or less metastases involving any organs except the brain. All patients had bone metastasis (BM) and seven patients had pulmonary, hepatic, or lymph node metastasis. Median RT dose applied to metastatic lesions was 30 Gy (range, 20 to 60 Gy). Results: The 5-year tumor local control (LC) and 3-year distant progression-free survival (DPFS) rate were 66.1% and 36.8%, respectively. High RT dose (${\geq}50Gy_{10}$) was significantly associated with improved LC. The 5-year overall survival (OS) rate was 49%. Positive hormone receptor status, pathologic nodal stage of primary cancer, solitary BM, and whole-lesion RT (WLRT), defined as RT whose field encompassed entire extent of disease, were associated with better survival. On analysis for subgroup of solitary BM, high RT dose was significantly associated with improved LC and DPFS, shorter metastasis-to-RT interval (${\leq}1month$) with improved DPFS, and WLRT with improved DPFS and OS, respectively. Conclusion: High-dose RT in solitary BM status and WLRT have the potential to improve the progression-free survival and OS of patients with EOMBC.