• Journal of Korean Neurosurgical Society
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• v.39 no.6
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• pp.409-412
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• 2006

Objective : Primary central nervous system lymphoma[PCNSL] is a non-Hodgkin's lymphoma arising in the central nervous system. Combined chemotherapy and radiation therapy is the standard treatment for PCNSL. However, treatment induced neurotoxicity is a major problem especially in elderly patients. Methods : From May 2001 to April 2004, elderly five patients with PCNSL confirmed via pathological examination were underwent Novalis radiosurgery in authors' institution, who were investigated retrospectively. Of these patients, 2 were male and 3 were female, with a mean age 68 years old [range $65{\sim}73$]. The number of lesions was 1 in 2 patients, 2 in 2, and 3 in 1. The mean follow up period was 12.6 months [range $8{\sim}16$]. Results : Clinical symptoms and signs in all patients markedly improved within 1 weeks after Novalis radiosurgery. The Karnofsky performance status score was also improved from a pre-radiosurgery average of 68 to a post-radiosurgery one of 82. All of treated lesions showed a partial or complete regression of the original mass. There have been no complication following Novalis radiosurgery. Conclusion : The preliminary results of our experience indicate that radiosurgery with Novalis provides a safe and effective therapeutic alternative treatment of PCNSL in elderly patients.

• Radiation Oncology Journal
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• v.12 no.1
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• pp.33-42
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• 1994

From 1982 to 1991, sixteen Patients with primary non-Hodgkin's lymphoma of the central nervous system(CNS) were seen at Kyung Hee University Hospital. The most common subtypes were large, noncleaved cell lymphoma and immunoblastic lymphoma of B cells. Lesions most commonly involved were the parietal lobes and/or deep nuclei. Positive cerebrospinal fluid cytology was rare at initial presentation. Sixteen patients were treated with surgical biopsy or resection followed by whole brain radiotherapy at a median dose of 40 Gy(range=30-50 Gy) with variable boost doses. Of 16 patients who underwent surgery and postoperative radiotherapy, fourteen patients died between 2 and 49 months following treatment, and two are alive with no evidence of disease at 8 and 22 months. The 1-and 2-year survival rates were 55.6$ \% $ and 34.7$ \% $, respectively with 12 months of median survival. Patterns of failure were analyzed in eleven patients of total 16 patients. Failure at the original site of involvement was uncommon after radiotherapy treatment. In contrast, failure in the brain at sites other than those originally invovled was common in spite of the use of whole brain irradiation. Failure occurred in the brain 11/16(68.7$ \% $), in spinal axis 4/16(25.0$ \% $). The age, sex, location of involvement within CNS, numbers of lesion, or radiation dose did not influence on survival. The authors conclude that Primary CNS lymphoma is a locally aggressive disease that is poorly controlled with conventional radiation therapy. The limitation of current therapy for this disease are discussed, and certain promising modality should be made in regarding the management of future patients with this disease.

• Journal of Yeungnam Medical Science
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• v.38 no.3
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• pp.258-263
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• 2021

Neurolymphomatosis (NL) is defined as the involvement of the peripheral nervous system in lymphocytic invasion. It is a very rare form of lymphoma that may occur as an initial presentation or recurrence. It affects various peripheral nervous structures and can therefore mimic disc-related nerve root pathology or compressive mononeuropathy. NL often occurs in malignant B-cell non-Hodgkin lymphomas. Notwithstanding its aggressiveness or intractability, NL should be discriminated from other neurologic complications of lymphoma. Herein, we present a case of primary NL as the initial presentation of diffuse large B-cell lymphoma (DLBCL) of the sciatic nerve. The patient presented with weakness and pain in his left leg but had no obvious lesion explaining the neurologic deficit on initial lumbosacral and knee magnetic resonance imaging (MRI). NL of the left sciatic nerve at the greater sciatic foramen was diagnosed based on subsequent hip MRI, electrodiagnostic test, positron emission tomography/computed tomography, and nerve biopsy findings. Leg weakness slightly improved after chemotherapy and radiotherapy. We report a case wherein NL, a rare cause of leg weakness, manifested as the initial presentation of primary DLBCL involving the sciatic nerve at the greater sciatic foramen.

• Journal of Korean Neurosurgical Society
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• v.29 no.12
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• pp.1628-1633
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• 2000

Objective : The incidence of primary CNS lymphoma(PCNSL) has been increasing recently. The purpose of this study is to establish of prognostic factors and treatment options for PCNSL. Methods : Thirty-one PCNSL patients were treated in our institute between 1985 and 1997. All patients were histologically confirmed via stereotactic biopsy or open biopsy. The authors retrospectively analyzed clinical characteristics of PCNSL and prognostic factors, including histological cell types, immunohistological cell types and treatment options of PCNSL. Our data were statistically analyzed using Kaplan Meier survival curve and multivariated ANOVA test. Results : The clinical and radiological characteristics of PCNSL were resembled to those of other reports. The most common histological subtype was diffuse large cell type(55.5%). In immunohistolgical study, the incidence of T-cell lymphoma(35.7%) was very higher than that of others. The radiotherapy could prolonged patients' survival(p=0.021). One-year and 3-year survival rate of PCNSL were 66.9% and 45.9%, respectively. One-year survival rate of B cell and T cell lymphoma were 72.7% and 50.0%, respectively. The patients with B-cell lymphoma showed better prognosis than patients with T-cell lymphoma(p=0.049). Conclusion : On the basis of our data, active radiotherapy could prolong patients' survival. the T-cell lymphoma revealed higher incidence than those of other reports and had poor prognosis than that of B cell lymphoma.

• The Korean Journal of Medicine
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• v.99 no.1
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• pp.37-49
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• 2024

Background/Aims: In Korea, the incidence of primary diffuse large B-cell lymphoma of the central nervous system (PCNSL) is increasing and autologous stem cell transplantation (ASCT) has improved the survival of younger patients. We explored our real-world experience with PCNSL at Asan Medical Center (AMC). Methods: We used the AMC lymphoma registry to collect patient data prospectively. We analyzed 279 patients diagnosed from 2002 until August 2019. Results: The PCNSL incidence at AMC increased progressively and comprised 7.4-8.9% of new non-Hodgkin lymphoma patients annually during the most recent 4 years. The median age was 60 years (range, 17-85) and males comprised 55%. Patients under 65 years of age (n = 183) had no significant differences in characteristics compared to those aged 65 years or over, with the exception of less occipital lobe involvement and lower beta-2 microglobulin levels. Rituximab, methotrexate, procarbazine, and vincristine (R-MPV) combination induction had the best overall response, of 95%. The median overall survival was 3.8 years with 5- and 10-year survival rates of 41.5% and 30.2%, respectively. Survival was better in younger patients and those treated with ASCT. Thiotepa, busulfan, and cytoxan (TBC) conditioning chemotherapy had better survival than other combinations. The International Extranodal Lymphoma Study Group and Memorial Sloan Kettering Cancer Center prognostic score systems were valid in this cohort. Age and performance status were independent prognostic factors. Exclusive extra-central nervous system failure occurred in six patients (5.6%) among 107 failures. Conclusions: The incidence of PCNSL is rising. R-MPV induction therapy followed by ASCT with TBC has improved the survival of young, fit PCNSL patients.

• Journal of Korean Neurosurgical Society
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• v.30 no.3
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• pp.334-341
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• 2001

Objective : We have currently changed treatment strategies to methotrexate(MTX)-based preirradiation chemotherapy with subsequent planned radiation for the initial therapy of primary central nervous system lymphoma (PCNSL). The aim of this study was to evaluate the results of treating PCNSL with chemotherapy plus radiotherapy (CRT) or radiotherapy(RT) alone. Method and Material : This study involved 10 females and 3 males patients with a mean age of 54.2 years. All patients underwent surgery, open(8 cases) or stereotactic biopsy(5 cases) for histological diagnosis. Eleven tumors were diffuse large B-cell lymphomas. Tumor volume change in the follow-up images and survival time were evaluated in patients treated with CRT and RT alone. In the beginning, two patients received ProMACE-Cytabom chemotherapeutic regimen, but did not complete the course and died of progressive tumor 8 and 9 months after diagnosis, respectively. One patient died at 6 months before chemotherapy. These three were excluded from the survival analysis. Five patients(RT group) completed full courses of cranial irradiation with or without boost. For the current combined modality treatment, high-dose MTXbased chemotherapy(systemic and intrathecal MTX, IV vincristine, and oral procarbazine) followed by whole brain irrdiation to 45Gy to tumor was introduced in 5 patients of CRT group. Result : A complete response was achieved in three of five who received RT only and in all of five who received CRT. All patients in CRT groups are in disease free status at a mean 23 months following therapy. The RT group patients refused any additional salvage therapy at tumor relapse and survived at mean 20 months from diagnosis. The Karnofsky performance status improved in eight of ten patients with treatment. The treatment toxicity included leukoencephalopathy in RT group and severe leukopenia, transient hepatitis, avascular necrosis of femoral head, hearing loss, and amenorrhea in CRT group, respectively. Conclusion : The combined modality therapy of MTX-based chemotherapy plus radiotherapy for PCNSL may enhance tumor response and improve patient survival. The patients who received CRT should be carefully followed up because of the higher risk of treatment-induced late neurotoxicity.

• Investigative Magnetic Resonance Imaging
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• v.21 no.1
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• pp.9-19
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• 2017

Background: Normalized cerebral blood volume (nCBV) can be measured using manual or semiautomatic segmentation method. However, the difference in diagnostic performance on brain tumor differentiation between differently measured nCBV has not been evaluated. Purpose: To compare the diagnostic performance of manually obtained nCBV to that of semiautomatically obtained nCBV on glioblastoma (GBM) and primary central nervous system lymphoma (PCNSL) differentiation. Materials and Methods: Histopathologically confirmed forty GBM and eleven PCNSL patients underwent 3T MR imaging with dynamic susceptibility contrast-enhanced perfusion MR imaging before any treatment or biopsy. Based on the contrast-enhanced T1-weighted imaging, the mean nCBV (mCBV) was measured using the manual method (manual mCBV), random regions of interest (ROIs) placement by the observer, or the semiautomatic segmentation method (semiautomatic mCBV). The volume of enhancing portion of the tumor was also measured during semiautomatic segmentation process. T-test, ROC curve analysis, Fisher's exact test and multivariate regression analysis were performed to compare the value and evaluate the diagnostic performance of each parameter. Results: GBM showed a higher enhancing volume (P = 0.0307), a higher manual mCBV (P = 0.018) and a higher semiautomatic mCBV (P = 0.0111) than that of the PCNSL. Semiautomatic mCBV had the highest value (0.815) for the area under the curve (AUC), however, the AUCs of the three parameters were not significantly different from each other. The semiautomatic mCBV was the best independent predictor for the GBM and PCNSL differential diagnosis according to the stepwise multiple regression analysis. Conclusion: We found that the semiautomatic mCBV could be a better predictor than the manual mCBV for the GBM and PCNSL differentiation. We believe that the semiautomatic segmentation method can contribute to the advancement of perfusion based brain tumor evaluation.

• Radiation Oncology Journal
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• v.38 no.1
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• pp.35-43
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• 2020

Purpose: This retrospective study compares higher-dose whole-brain radiotherapy (hdWBRT) with reduced-dose WBRT (rdWBRT) in terms of clinical efficacy and toxicity profile in patients treated for primary central nervous system lymphoma (PCNSL). Materials and Methods: Radiotherapy followed by high-dose methotrexate (HD-MTX)-based chemotherapy was administered to immunocompetent patients with histologically confirmed PCNSL between 2000 and 2016. Response to chemotherapy was taken into account when prescribing the radiation dose to the whole brain and primary tumor bed. The whole brain dose was ≤23.4 Gy for rdWBRT (n = 20) and >23.4 Gy for hdWBRT (n = 68). Patients manifesting cognitive disturbance, memory impairment and dysarthria were considered to have neurotoxicity. A median follow-up was 3.62 years. Results: The 3-year overall survival (OS) and progression-free survival (PFS) were 70.0% and 48.9% with rdWBRT, and 63.2% and 43.2% with hdWBRT. The 3-year OS and PFS among patients with partial response (n = 45) after chemotherapy were 77.8% and 53.3% with rdWBRT, and 58.3% and 45.8% with hdWBRT (p > 0.05). Among patients with complete response achieved during follow-up, the 3-year freedom from neurotoxicity (FFNT) rate was 94.1% with rdWBRT and 62.4% with hdWBRT. Among patients aged ≥60 years, the 3-year FFNT rate was 87.5% with rdWBRT and 39.1% with hdWBRT (p = 0.49). Neurotoxicity was not observed after rdWBRT in patients aged below 60 years. Conclusion: rdWBRT with tumor bed boost combined with upfront HD-MTX is less neurotoxic and results in effective survival as higher-dose radiotherapy even in partial response after chemotherapy.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• v.36 no.6
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• pp.497-501
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• 2010

Primary nasal type natural killer (NK)/T cell (NKTC) lymphoma, a specific form of malignant lymphoma, has a higher geographic incidence in Oriental, Mexican, and South American populations than the Western population. In Koreans, it comprises 9-12% of all cases of non-Hodgkin's lymphoma. This type of lymphoma has also been named as angiocentic lymphoma and lethal midline granuloma because the most common site is the upper airway area and its clinical aggressiveness presents with a necrotic and destructive pattern. NKTC lymphoma can also be detected in different organs (testis, spleen, parotid gland, skin, gastroinstinal tract, central nervous system, lungs, bone marrow, etc.) other than the upper airway including the oral cavity. The lymphoma detected in the oral cavity shows various destructive and inflammatory changes, similar to the signs of inflammation and infection from periodontitis and pulpal disease, making a diagnosis difficult with just the clinical signs. For early detection, clinical, radiological, and pathological examinations are required. This report describes the clinical, radiological and histological characteristics with a case report for the early detection of NKTC lymphoma in the oral cavity.

• Asian Pacific Journal of Cancer Prevention
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• v.15 no.11
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• pp.4733-4738
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• 2014

Purpose: We developed and evaluated a regimen including fotemustine, teniposide and dexamethasone (FTD) for treating patients with central nervous system (CNS) lymphoma based on pharmacokinetic properties of individual agents and in combination. Patients and Methods: In a comparison study, 8 patients with primary CNS lymphoma (PCNSL) and 8 with secondary CNS lymphoma (SCNSL) were treated with FTD (comprising fotemustine 100 mg/m2, 1h infusion, day 1; teniposide 60 mg/m2, >0.5 h infusion, on day 2, 3, 4; dexamethasone 40 mg, 1h infusion, on day 1, 2, 3, 4 and 5; and methotrexate 12 mg, cytosine arabinoside 50 mg plus dexamethasone 5 mg intrathecally, on day 2 and 7). Cycles were repeated every 3 weeks. After response assessment, patients received whole brain radiotherapy. Results: Of the 8 PCNSL patients, 4 (50%) achieved CR and 3 (38%) PR, an overall response rate of 88%. Four patients (50%) were in continuing remission at the end of this study after a median follow-up of 30 months (range 10 to 56 months). Of the 8 SCNSL patients the overall response rate was 63% (CR+PR: 38%+25%). All responses were achievable with predictable toxicity mainly reflecting reversible myelosuppression. Conclusion: This study suggests that FTD could be an effective treatment for CNS lymphoma, and is worthy of further evaluation.