• 대한골관절종양학회지
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• 제5권3호
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• pp.183-189
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• 1999

Ewing's sarcoma is an uncommon malignant neoplasm of the long bone and it has a poor prognosis due to its early metastasis and aggressive local spread. It is mostly found before the age of 30 and it is rare in extraskeletal sites. Extraskeletal Ewing's sarcoma has been reported to occur in various sites including the larynx, scalp, nasal fossa, neck, chest wall, lung, pelvis, perineum, arm, finger, leg and toe, but it is extremely rare as a primary epidural tumor of the spine. We experienced a case of extraosseous epidural Ewing's sarcoma arising in the lumbar spinal canal at L3-L5 level in a 9-year-old boy. Following total laminectomy from L3 to L5 with a lumbar vertebrae and mass excision, he received chemotherapy with complete remission.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제23권2호
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• pp.180-187
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• 2020

Giant cell hepatitis with autoimmune hemolytic anemia (AHA) is a rare disease of infancy characterized by the presence of both Coombs-positive hemolytic anemia and progressive liver disease with giant cell transformation of hepatocytes. Here, we report a case involving a seven-month-old male infant who presented with AHA followed by cholestatic hepatitis. The clinical features included jaundice, pallor, and red urine. Physical examination showed generalized icterus and splenomegaly. The laboratory findings suggested warm-type AHA with cholestatic hepatitis. Liver biopsy revealed giant cell transformation of hepatocytes and moderate lobular inflammation. The patient was successfully treated with four doses of rituximab. Early relapse of hemolytic anemia and hepatitis was observed, which prompted the use of an additional salvage dose of rituximab. He is currently in clinical remission.

• BMB Reports
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• 제48권8호
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• pp.427-428
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• 2015

Despite high interests on microenvironmental regulation of leukemic cells, little is known for bone marrow (BM) niche in leukemia patients. Our recent study on BMs of acute myeloid leukemia (AML) patients showed that the mesenchymal stromal cells (MSCs) are altered during leukemic conditions in a clinical course-dependent manner. Leukemic blasts caused reprogramming of transcriptomes in MSCs and remodeling of niche cross-talk, selectively suppressing normal primitive hematopoietic cells while supporting leukemogenesis and chemo-resistance. Notably, differences in BM stromal remodeling were correlated to heterogeneity in subsequent clinical courses of AML, i.e., low numbers of mesenchymal progenitors at initial diagnosis were correlated to complete remission for 5-8 years, and high contents of mesenchymal progenitor or MSCs correlated to early or late relapse, respectively. Thus, stromal remodeling by leukemic cell is an intrinsic part of leukemogenesis that can contribute to the clonal dominance of leukemic cells over normal hematopoietic cells, and can serve as a biomarker for prediction of prognosis. [BMB Reports 2015; 48(8): 427-428]

• Asian Pacific Journal of Cancer Prevention
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• 제15권15호
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• pp.6099-6101
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• 2014

Purpose: To retrospectively analyze variability and clinical significance of serum ferritin levels in Chinese patients with hematologic malignancies. Materials and Methods: Serum ferritin were measured by radioimmunoassay, using a kit produced by the Beijing Institute of Atomic Energy. Patients with hematologic malignancies, and treated in the Department of Hematology in Nanjing First Hospital and fulfilled study criteria were recruited. Results: Of 473 patients with hematologic malignancies, 262 patients were diagnosed with acute leukemia, 131 with lymphoma and 80 with multiple myeloma. Serum ferritin levels of newly diagnosed and recurrent patients were significantly higher than those entering complete remission stage or in the control group (p<0.001). Conclusions: Serum ferritin lever in patients with hematologic malignancies at early stage and recurrent stage are significantly increased, so that detection and surveillance of changes of serum ferritin could be helpful in assessing conditions and prognosis of this patient cohort.

• Journal of Chest Surgery
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• 제16권4호
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• pp.571-583
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• 1983

Thymic tumor is the most common neoplasm originating in the anterior mediastinum. Histologically, thymic tumors and tumorlike conditions have been classified into thymic cyst, thymolipoma, true thymic hyperplasia, thymoma and carcinoid tumor of the thymus gland. We have experienced several tumors and a few tumorlike conditions of the thymus gland in 10 patients: thymoma 6, thymic hyperplasia 2, carcinoid tumor 1 and thymic cyst 1. The age distributions were ranging from 30 to 60 years except for one 3 year old child [malignant thymoma, lymphocytic type], and male to female ratio was 1:1.5. In 6 cases, thymic lesions were removed through lateral thoracic incision [right 2, left 4] because the mediastinal masses were growing far into the pleural space, meanwhile, medially located lesions [4 cases] through median sternotomy incision. Among these 10 patients, 3 were malignant thymomas, in which 2 were died of recurrence after tumor resection. Myasthenia gravis was all noted in 3 females [thymoma 2, thymic follicular hyperplasia 1], in which only two revealed remission in symptoms following thymectomy. The authors would like to recommend early radical thymectomy through median sternotomy incision whenever patients demonstrate suspicious thymic tumor lesions on the chest roentgenogram or generalized myasthenic symptoms.

• Clinical and Experimental Pediatrics
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• 제58권1호
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• pp.28-32
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• 2015

Purpose: Diencephalic syndrome is an uncommon cause of failure to thrive in early childhood that is associated with central nervous system neoplasms in the hypothalamic-optic chiasmatic region. It is characterized by complex signs and symptoms related to hypothalamic dysfunction; such nonspecific clinical features may delay diagnosis of the brain tumor. In this study, we analyzed a series of cases in order to define characteristic features of diencephalic syndrome. Methods: We performed a retrospective study of 8 patients with diencephalic syndrome (age, 5-38 months). All cases had presented to Seoul National University Children's Hospital between 1995 and 2013, with the chief complaint of poor weight gain. Results: Diencephalic syndrome with central nervous system (CNS) neoplasm was identified in 8 patients. The mean age at which symptoms were noted was $18{\pm}10.5$ months, and diagnosis after symptom onset was made at the mean age of $11{\pm}9.7$ months. The mean z score was $-3.15{\pm}1.14$ for weight, $-0.12{\pm}1.05$ for height, $1.01{\pm}1.58$ for head circumference, and $-1.76{\pm}1.97$ for weight-for-height. Clinical features included failure to thrive (n=8), hydrocephalus (n=5), recurrent vomiting (n=5), strabismus (n=2), developmental delay (n=2), hyperactivity (n=1), nystagmus (n=1), and diarrhea (n=1). On follow-up evaluation, 3 patients showed improvement and remained in stable remission, 2 patients were still receiving chemotherapy, and 3 patients were discharged for palliative care. Conclusion: Diencephalic syndrome is a rare cause of failure to thrive, and diagnosis is frequently delayed. Thus, it is important to consider the possibility of a CNS neoplasm as a cause of failure to thrive and to ensure early diagnosis.

• Radiation Oncology Journal
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• 제37권2호
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• pp.82-90
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• 2019

Purpose: To evaluate the results of hypofractionated radiotherapy (HFX) for early glottic cancer. Materials and Methods: Eighty-five patients with cT1-2N0M0 squamous cell carcinoma of the glottis who had undergone HFX, performed using intensity-modulated radiotherapy (IMRT, n = 66) and three-dimensional conformal radiotherapy (3D CRT, n = 19) were analyzed. For all patients, radiotherapy was administered at 60.75 Gy in 27 fractions. Forty-three patients received a simultaneous integrated boost (SIB) of 2.3-2.5 Gy per tumor fraction. Results: The median follow-up duration was 29.9 months (range, 5.5 to 76.5 months). All patients achieved complete remission at a median of 50 days after the end of radiotherapy (range, 14 to 206 days). The 5-year rates for locoregional recurrence-free survival was 88.1%, and the 5-year overall survival rate was 86.2%. T2 stage was a prognostic factor for locoregional recurrence-free survival after radiotherapy (p = 0.002). SIB for the tumor did not affect disease control and survival (p = 0.191 and p = 0.387, respectively). No patients experienced acute or chronic toxicities of ≥grade 3. IMRT significantly decreased the dose administered to the carotid artery as opposed to 3D CRT (V₃₅, p < 0.001; V₅₀, p < 0.001). Conclusions: Patients treated with HFX achieved acceptable locoregional disease control rates and overall survival rates compared with previous HFX studies. A fraction size of 2.25 Gy provided good disease control regardless of SIB administration.

• International Journal of Advanced Culture Technology
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• 제7권4호
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• pp.156-162
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• 2019

Rheumatoid arthritis (RA) is a chronic autoimmune disorder characterized by progressive joint deterioration; Furthermore, RA can also affect body tissues, including the skin, eyes, lungs, heart and blood vessels. The early stages of RA can be difficult to diagnose because the signs and symptoms mimic those of many other diseases. It is not known exactly what triggers the onset of RA and how to cure the disease. But recent discoveries indicate that remission of symptoms is more likely when treatment begins early with strong medications known as disease-modifying anti-rheumatic drugs (DMARDs). Tumor necrosis factor (TNF) inhibitors are typical examples of biotherapies that have been developed for RA. The substances may occur naturally in the body or may be made in the laboratory. Other biological therapies care biological response modifiers (BRMs)such as monoclonal antibodies, interferon, interleukin-2 (IL-2) and a protein binder using repeat units. These substances play significant anti-inflammatory roles. Proteins with recurrent, conserved amino acid stretches mediate interactions among proteins for essential biological functions; for example, ankyrin (ANK), Heat repeat protein (HEAT), armadillo repeat protein (ARM) and tetratricopeptide repeats (TPR). Here, we describe Leucine rich repeats (LRR) that ideally fold together to form a solenoid protein domain and is more applicable to our current study than the previously mentioned examples. Although BRMs have limitations in terms of immunogenicity and effector functions, among other factors, in the context therapeutic use and for proteomics research, We has become clear that repeat-unit-derived binding proteins will increasingly be used in biotechnology and medicine.

• Clinical Psychopharmacology and Neuroscience
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• 제16권4호
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• pp.469-480
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• 2018

Objective: Pharmacogenomic-based antidepressant treatment (PGATx) may result in more precise pharmacotherapy of major depressive disorder (MDD) with better drug therapy guidance. Methods: An 8-week, randomized, single-blind clinical trial was conducted to evaluate the effectiveness and tolerability of PGATx in 100 patients with MDD. All recruited patients were randomly allocated either to PGATx (n=52) or treatment as usual (TAU, n=48) groups. The primary endpoint was a change of total score of the Hamilton Depression Rating Scale-17 (HAMD-17) from baseline to end of treatment. Response rate (at least 50% reduction in HAMD-17 score from baseline), remission rate (HAMD-17 score ${\leq}7$ at the end of treatment) as well as the change of total score of Frequency, Intensity, and Burden of Side Effects Ratings (FIBSER) from baseline to end of treatment were also investigated. Results: The mean change of HAMD-17 score was significantly different between two groups favoring PGATx by -4.1 point of difference (p=0.010) at the end of treatment. The mean change in the FIBSER score from baseline was significantly different between two treatment groups favoring PGATx by -2.5 point of difference (p=0.028). The response rate (71.7 % vs. 43.6%, p=0.014) were also significantly higher in PGATx than in TAU at the end of treatment, while the remission rate was numerically higher in PGATx than in TAU groups without statistical difference (45.5% vs. 25.6%, p=0.071). The reason for early drop-out associated with adverse events was also numerically higher in TAU (n=9, 50.0%) than in PGATx (n=4, 30.8%). Conclusion: The present study clearly demonstrate that PGATx may be a better treatment option in the treatment of MDD in terms of effectiveness and tolerability; however, study shortcomings may limit a generalization. Adequately-powered, well-designed, subsequent studies should be mandatory to prove its practicability and clinical utility for routine practice.

• Journal of Digestive Cancer Research
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• 제3권2호
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• pp.82-88
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• 2015

위에 발생하는 암 중 위 림프종이 1-6% 가량을 차지하고 있고, 이 중 50% 가량이 MALT 림프종이다. MALT 림프종의 60-70% 가량은 조기, 국소병기에서 진단된다. 위 MALT 림프종은 일반적으로 그 진행이 매우 느리며 상복부 통증, 소화불량, 오심, 구토 등의 비특이적인 증상을 보인다. 진단은 조직학적으로 진단하며, 헬리코박터필로리 연관 만성 위염에서 보이는 염증성 병변과의 감별을 위해 Wotherspoon score를 이용한다. 여러 연구를 통해 위 MALT 림프종이 헬리코박터필로리 감염과 관련이 있다는 것이 밝혀져 있고, 3세염색체증, 18세염색체증, t(11;18), t(1;14), t(14;18), t(3;14) 등 여러 유전적 이상을 가질 수 있는 것으로 알려져 있다. 적절한 치료 방침을 결정하기 위해 병기를 결정하는 것은 매우 중요하며, Lugano International Conference classification을 주로 사용한다. 위 MALT 림프종의 1차 치료는 헬리코박터필로리 감염 유무 및 병기와 관계없이 헬리코박터필로리 제균치료이다. Stage I/II1 환자의 경우 제균치료로 60-90%의 완전 완화율을 보인다. 제균치료에 반응이 없는 경우에는 방사선치료, 항암화학요법, 면역치료 등을 시행해 볼 수 있고, 75-100%의 완전완화율을 보인다. 향후 헬리코박터필로리의 감염율이 감소하면서 위 MALT 림프종의 발생도 감소할 것으로 기대된다. 또한 향후 치료방침 확립을 위해 지속적인 연구가 필요할 것으로 생각한다.