• Journal of Korean Neurosurgical Society
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• v.29 no.10
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• pp.1316-1321
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• 2000

Objectives : The authors analyzed the surgical series of Cushing's disease to evaluate the proper treatment policy and to verify the possible prognostic factors. Material and Methods : Of 50 patients diagnosed as Cushing's disease and operated at Department of Neurosurgery of our institute between 1988 and 1999, 48 patients with available medical records were analyzed retrospectively. Mean follow-up period was 48 months(3 to 109 months). Preoperative diagnosis was made after evaluating the patients with multiple-stage endocrinological studies and 31 selective patients were evaluated with inferior petrosal sinus sampling(IPSS). Magnetic resonance imaging(MRI) and/or high resolution computerized tomography(CT) was done in all patients. A total of 51 transsphenoidal adenomectomy(TSA) were performed including 3 revision for initial surgical failure cases. Remission was decided on the basis of both endocrinological criteria and clinical status. Radiation and/or ketoconazole therapy were applied to failed cases. For the verification of prognostic factors, the authors evaluated the statistical significance of multiple variables over remission rate by chi-square test. Result : Sensitivity of IPSS for central localization was 93.5% which was better than that of MRI(87.5%). But for lateralization, it was 72.4% for IPSS versus MRI 90.5%. Success rate of TSA was 82%(42/51) and recurrence rate was 9%(4/48). When including adjuvant treatments for surgically failed cases, overall success rate was 89.6% and all of 3 reoperated cases(TSA) due to recurrence were successful. Significant complication occurred in 7.8%(4/51) after TSA including hypopituitarism, diabetes insipidus, and visual loss. Non-existence of tumor in MRI and prolonged symptom duration(>3 years) were significant prognostic factors. Conclusion : TSA can be considered as initial treatment for Cushing's disease. In surgically failed cases, multiple treatment modality may improve the overall outcome and repeated TSA for recurrent cases seem to provide similar success.

• Journal of Korean Neurosurgical Society
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• v.61 no.5
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• pp.633-639
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• 2018

Objective : We investigated the outcomes of repeat stereotactic radiosurgery (SRS) for metastatic brain tumors that locally recurred despite previous SRS, focusing on the tumor control. Methods : A total of 114 patients with 176 locally recurring metastatic brain tumors underwent repeat SRS after previous SRS. The mean age was 59.4 years (range, 33 to 85), and there were 68 male and 46 female patients. The primary cancer types were non-small cell lung cancer (n=67), small cell lung cancer (n=12), gastrointestinal tract cancer (n=15), breast cancer (n=10), and others (n=10). The number of patients with a single recurring metastasis was 95 (79.8%), and another 19 had multiple recurrences. At the time of the repeat SRS, the mean volume of the locally recurring tumors was 5.94 mL (range, 0.42 to 29.94). We prescribed a mean margin dose of 17.04 Gy (range, 12 to 24) to the isodose line at the tumor border primarily using a 50% isodose line. Results : After the repeat SRS, we obtained clinical and magnetic resonance imaging follow-up data for 84 patients (73.7%) with a total of 108 tumors. The tumor control rate was 53.5% (58 of the 108), and the median and mean progression-free survival (PFS) periods were 246 and 383 days, respectively. The prognostic factors that were significantly related to better tumor control were prescription radiation dose of 16 Gy (p=0.000) and tumor volume less than both 4 mL (p=0.001) and 10 mL at the repeat SRS (p=0.008). The overall survival (OS) periods for all 114 patients after repeat SRS varied from 1 to 56 months, and median and mean OS periods were 229 and 404 days after the repeat SRS, respectively. The main cause of death was systemic problems including pulmonary dysfunction (n=58, 51%), and the identified direct or suspected brain-related death rate was around 20%. Conclusion : The tumor control following repeat SRS for locally recurring metastatic brain tumors after a previous SRS is relatively lower than that for primary SRS. However, both low tumor volume and high prescription radiation dose were significantly related to the tumor control following repeat SRS for these tumors after previous SRS, which is a general understanding of primary SRS for metastatic brain tumors.

• Radiation Oncology Journal
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• v.29 no.3
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• pp.199-205
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• 2011

Purpose: The present study compared the difference between intraoperative transrectal ultrasound (iTRUS)-based prostate volume and preplan computed tomography (CT), preplan magnetic resonance imaging (MRI)-based prostate volume to estimate the number of seeds needed for appropriate dose coverage in permanent brachytherapy for prostate cancer. Materials and Methods: Between March 2007 and March 2011, among 112 patients who underwent permanent brachytherapy with $^{125}I$, 60 image scans of 56 patients who underwent preplan CT (pCT) or preplan MRI (pMRI) within 2 months before brachytherapy were retrospectively reviewed. Twenty-four cases among 30 cases with pCT and 26 cases among 30 cases with pMRI received neoadjuvant hormone therapy (NHT). In 34 cases, NHT started after acquisition of preplan image. The median duration of NHT after preplan image acquisition was 17 and 21 days for cases with pCT and pMRI, respectively. The prostate volume calculated by different modalities was compared. And retrospective planning with iTRUS image was performed to estimate the number of $^{125}I$ seed required to obtain recommended dose distribution according to prostate volume. Results: The mean difference in prostate volume was 9.05 mL between the pCT and iTRUS and 6.84 mL between the pMRI and iTRUS. The prostate volume was roughly overestimated by 1.36 times with pCT and by 1.33 times with pMRI. For 34 cases which received NHT after image acquisition, the prostate volume was roughly overestimated by 1.45 times with pCT and by 1.37 times with pMRI. A statistically significant difference was found between preplan image-based volume and iTRUS-based volume (p<0.001). The median number of wasted seeds is approximately 13, when the pCT or pMRI volume was accepted without modification to assess the required number of seeds for brachytherapy. Conclusion: pCT-based volume and pMRI-based volume tended to overestimate prostate volume in comparison to iTRUS-based volume. To reduce wasted seeds and cost of the brachytherapy, we should take the volume discrepancy into account when we estimate the number of $^{125}I$ seeds for permanent brachytherapy.

• Journal of Korean Neurosurgical Society
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• v.50 no.4
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• pp.299-303
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• 2011

Objective : Brainstem metastases are rarely operable and generally unresponsive to conventional radiation therapy or chemotherapy. Recently, Gamma Knife Radiosurgery (GKRS) was used as feasible treatment option for brainstem metastasis. The present study evaluated our experience of brainstem metastasis which was treated with GKRS. Methods : Between November 1992 and June 2010, 32 patients (23 men and 9 women, mean age 56.1 years, range 39-73) were treated with GKRS for brainstem metastases. There were metastatic lesions in pons in 23, the midbrain in 6, and the medulla oblongata in 3 patients, respectively. The primary tumor site was lung in 21, breast in 3, kidney in 2 and other locations in 6 patients. The mean tumor volume was $1,517mm^3$ (range, 9-6,000), and the mean marginal dose was 15.9 Gy (range, 6-23). Magnetic Resonance Imaging (MRI) was obtained every 2-3 months following GKRS. Follow-up MRI was possible in 24 patients at a mean follow-up duration of 12.0 months (range, 1-45). Kaplan-Meier survival analysis was used to evaluate the prognostic factors. Results : Follow-up MRI showed tumor disappearance in 6, tumor shrinkage in 14, no change in tumor size in 1, and tumor growth in 3 patients, which translated into a local tumor control rate of 87.5% (21 of 24 tumors). The mean progression free survival was 12.2 months (range, 2-45) after GKRS. Nine patients were alive at the completion of the study, and the overall mean survival time after GKRS was 7.7 months (range, 1-22). One patient with metastatic melanoma experienced intratumoral hemorrhage during the follow-up period. Survival was found to be associated with score of more than 70 on Karnofsky performance status and low recursive partitioning analysis class (class 1 or 2), in terms of favorable prognostic factors. Conclusion : GKRS was found to be safe and effective for management of brainstem metastasis. The integral clinical status of patient seems to be important in determining the overall survival time.

• Progress in Medical Physics
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• v.19 no.3
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• pp.178-185
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• 2008

Living donor liver transplantation is increasingly performed as an alternative to cadaveric transplantation. Preoperative screening of the donor candidates is very important. The quality, size, and vascular and biliary anatomy of the liver are best assessed with magnetic resonance (MR) imaging or computed tomography (CT). In particular, the volume of the potential graft must be measured to ensure sufficient liver function after surgery. Preoperative liver segmentation has proved useful for measuring the graft volume before living donor liver transplantations in previous studies. In these studies, the liver segments were manually delineated on each image section. The delineated areas were multiplied by the section thickness to obtain volumes and summed to obtain the total volume of the liver segments. This process is tedious and time consuming. To compensate for this problem, automatic segmentation techniques have been proposed with multiplanar CT images. These methods involve the use of sequences of thresholding, morphologic operations (ie, mathematic operations, such as image dilation, erosion, opening, and closing, that are based on shape), and 3D region growing methods. These techniques are complex but require a few computation times. We made a phantom for volume measurement with pig and evaluated actual volume of spleen and liver of phantom. The results represent that our semiautomatic volume measurement algorithm shows a good accuracy and repeatability with actual volume of phantom and possibility for clinical use to assist physician as a measuring tool.

• Nuclear Medicine and Molecular Imaging
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• v.42 no.2
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• pp.153-163
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• 2008

Medical imaging modalities to image either anatomical structure or functional processes have developed along somewhat independent paths. Functional images with single photon emission computed tomography (SPECT) and positron emission tomography (PET) are playing an increasingly important role in the diagnosis and staging of malignant disease, image-guided therapy planning, and treatment monitoring. SPECT and PET complement the more conventional anatomic imaging modalities of computed tomography (CT) and magnetic resonance (MR) imaging. When the functional imaging modality was combined with the anatomic imaging modality, the multimodality can help both identify and localize functional abnormalities. Combining PET with a high-resolution anatomical imaging modality such as CT can resolve the localization issue as long as the images from the two modalities are accurately coregistered. Software-based registration techniques have difficulty accounting for differences in patient positioning and involuntary movement of internal organs, often necessitating labor-intensive nonlinear mapping that may not converge to a satisfactory result. These challenges have recently been addressed by the introduction of the combined PET/CT scanner and SPECT/CT scanner, a hardware-oriented approach to image fusion. Combined PET/CT and SPECT/CT devices are playing an increasingly important role in the diagnosis and staging of human disease. The paper will review the development of multi modality instrumentations for clinical use from conception to present-day technology and the application software.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.15 no.2
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• pp.87-92
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• 2015

Mucopolysaccharidosis (MPS) is an inherited disease entity associated with lysosomal enzyme deficiencies. MPS type 2, also known as Hunter syndrome, has a characteristic morphology primarily involving x-l inked recessive defects and iduronate-2-sulfatase gene mutation. The purpose of this case report is to provide important clues to help pediatricians identify Hunter syndrome patients earlier (i.e., before the disease progresses). A 30-month-old boy showed developmental delay and decreased speech ability. Physical examinations revealed a flat nose and extensive Mongolian spots. Brain magnetic resonance images (MRIs) showed bilateral multiple patchy T2 hyperintense lesions in the periventricular and deep white matter, several cyst-like lesions in the body of the corpus callosum, and diffuse brain atrophy, which were in keeping with the diagnosis. Based on these findings, the patient was suspected of having MPS. In the laboratory findings, although the genetic analysis of IDS (Iduronate-2-sulfatase) did not show any pathogenic variant, the enzymatic activity of IDS was not detected. We could confirm the diagnosis of MPS, because other sulfatases, such as ${\alpha}$-L-iduronidase, were detected in the normal range. Early enzymatic replacement therapy is essential and has a relatively good prognosis. Therefore, early diagnosis should be made before organ damage becomes irreversible, and brain MRIs can provide additional diagnostic clues to help distinguish the disorder.

• Asian Pacific Journal of Cancer Prevention
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• v.16 no.12
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• pp.5019-5024
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• 2015

Background: The purpose of this study was to assess the dosimetric and clinical feasibility of volumetric modulated arc based hypofractionated stereotactic radiotherapy (RapidArc) treatment for large acoustic schwannoma (AS >10cc). Materials and Methods: Ten AS patients were immobilized using BrainLab mask. They were subject to multimodality imaging (magnetic resonance and computed tomography) to contour target and organs at risk (brainstem and cochlea). Volumetric modulated arc therapy (VMAT) based stereotactic plans were optimized in Eclipse (V11) treatment planning system (TPS) using progressive resolution optimizer-III and final dose calculations were performed using analytical anisotropic algorithm with 1.5 mm grid resolution. All AS presented in this study were treated with VMAT based HSRT to a total dose of 25Gy in 5 fractions (5fractions/week). VMAT plan contains 2-4 non-coplanar arcs. Treatment planning was performed to achieve at least 99% of PTV volume (D99) receives 100% of prescription dose (25Gy), while dose to OAR's were kept below the tolerance limits. Dose-volume histograms (DVH) were analyzed to assess plan quality. Treatments were delivered using upgraded 6 MV un-flattened photon beam (FFF) from Clinac-iX machine. Extensive pretreatment quality assurance measurements were carried out to report on quality of delivery. Point dosimetry was performed using three different detectors, which includes CC13 ion-chamber, Exradin A14 ion-chamber and Exradin W1 plastic scintillator detector (PSD) which have measuring volume of $0.13cm^3$, $0.009cm^3$ and $0.002cm^3$ respectively. Results: Average PTV volume of AS was 11.3cc (${\pm}4.8$), and located in eloquent areas. VMAT plans provided complete PTV coverage with average conformity index of 1.06 (${\pm}0.05$). OAR's dose were kept below tolerance limit recommend by American Association of Physicist in Medicine task group-101(brainstem $V_{0.5cc}$ < 23Gy, cochlea maximum < 25Gy and Optic pathway <25Gy). PSD resulted in superior dosimetric accuracy compared with other two detectors (p=0.021 for PSD.

• Clinical and Experimental Pediatrics
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• v.59 no.sup1
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• pp.25-28
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• 2016

Phelan-McDermid syndrome is a rare genetic disorder caused by the terminal or interstitial deletion of the chromosome 22q13.3. Patients with this syndrome usually have global developmental delay, hypotonia, and speech delays. Several putative genes such as the SHANK3, RAB, RABL2B, and IB2 are responsible for the neurological features. This study describes the clinical features and outcomes of Korean patients with Phelan-McDermid syndrome. Two patients showing global developmental delay, hypotonia, and speech delay were diagnosed with Phelan-McDermid syndrome via chromosome analysis, fluorescent in situ hybridization, and multiplex ligation-dependent probe amplification analysis. Brain magnetic resonance imaging of Patients 1 and 2 showed delayed myelination and severe communicating hydrocephalus, respectively. Electroencephalography in patient 2 showed high amplitude spike discharges from the left frontotemporoparietal area, but neither patient developed seizures. Kidney ultrasonography of both the patients revealed multicystic kidney disease and pelviectasis, respectively. Patient 2 experienced recurrent respiratory infections, and chest computed tomography findings demonstrated laryngotracheomalacia and bronchial narrowing. He subsequently died because of heart failure after a ventriculoperitoneal shunt operation at 5 months of age. Patient 1, who is currently 20 months old, has been undergoing rehabilitation therapy. However, global developmental delay was noted, as determines using the Korean Infant and Child Development test, the Denver developmental test, and the Bayley developmental test. This report describes the clinical features, outcomes, and molecular genetic characteristics of two Korean patients with Phelan-McDermid syndrome.

• Journal of Chest Surgery
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• v.40 no.8
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• pp.574-577
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• 2007

Primary malignant neoplasm of the pericardium is very rare, Neoplastic involvement of the pericardium may result in rapidly developing hemorrhagic effusion. A 30-year-old male who occasionally suffered from chest tightness was referred to our hospital under the diagnosis of unstable angina. He presented with acute chest pain and severe dyspnea that had developed one day previously. The diagnostic investigations such as echocardiography, chest CT and magnetic resonance image suggested cardiac tamponade that was caused by rupture of the pericardial teratoma. An operation to remove the tumor and effusion was performed. The pericardial mass was completely excised, and the result of the frozen biopsy favored malignancy. The final pathologic report was malignant fibrosarcoma of the pericardium and no malignant cells were found on the cytology of the pericardial effusion. The patient had a smooth postoperative course and was referred to another hospital for additional radiation therapy. We report here on this case of cardiac tamponade that was caused by primary pericardial fibrosarcoma, and this required urgent diagnosis and surgical management.