BACKGROUND: To examine survival rates and renal function after partial nephrectomy (PN) and radical nephrectomy (RN) in patients with chronic kidney disease (CKD). METHODS: We studied 4,332 patients who underwent PN or RN for pathological T1a-T2N0M0 renal cell carcinoma from 1988 to 2014. Patients were divided into two subgroups of CKD stage I–II and stage III. Kidney function, and survival outcomes were compared between groups. RESULTS: We included 1,756 patients with CKD I–II and 276 patients with CKD III in the final pair-matched analysis. Kidney function was significantly better preserved in the PN than in the RN group among all patients. However, the beneficial effect of PN on kidney function gradually disappeared over time in CKD III patients. The 5-year overall survival (OS) rates after PN and RN differed in patients with CKD I–II disease (99.4% vs. 96.5%, respectively, P = 0.015). The 5-year OS rates after surgery were not affected by mode of nephrectomy in CKD III patients (97.8% vs. 93.5%, P = 0.103). The 5-year cancer-specific survival rates did not differ between treatment groups in all CKD stage. Cox hazard analysis showed that the operative method was a significant factor for OS in CKD I–II patients (hazard ratio [HR], 0.320; confidence interval [CI], 0.122–0.840; P = 0.021). However, PN was not beneficial in terms of OS in CKD III patients (HR, 0.395; CI, 0.086–1.172; P = 0.117). CONCLUSION: PN is associated with a higher OS rate and better kidney function in patients with preoperative CKD stage I and II, but not in those with CKD stage III.
Carcinoma, Renal Cell* ; Humans ; Kidney* ; Methods ; Nephrectomy* ; Renal Insufficiency ; Renal Insufficiency, Chronic* ; Survival Rate*

BACKGROUND: Although the overall survival of childhood acute lymphoblastic leukemia (ALL) approaches 85-90%, the prognosis of relapsed or refractory (R/R) ALL is grave. This study aimed to identify the treatment pattern, treatment response, and overall survival of these patients.METHODS: We reviewed data of 64 patients with R/R ALL whose initial diagnosis of ALL had been made between 1 and 21 years of age. Patients who received clofarabine as part of an induction regimen were excluded. Relapsed patients were limited to those who relapsed after ≥2 prior induction regimens. Treatment patterns, response rates, and overall survival were analyzed.RESULTS: Patients' median age was 15.0 years (range, 6.0-25.0) at the diagnosis of R/R ALL. The most frequently used agents other than steroid were vincristine (54.0%), cytarabine (44.6%), and idarubicin (36.5%), while L-asparaginase was used in only one patient. The complete remission (CR) and overall response (OR) rates were 38.1 and 42.9%, respectively. Sixteen patients (25.4%) underwent allogeneic hematopoietic stem cell transplantation (HSCT). The 5-year overall survival was 6.7%. The survival of patients with HSCT was significantly higher compared with those without HSCT (35.2% vs 0%, P=0.0097). Among 14 patients who achieved CR or CR without platelet recovery (CRp) before HSCT, the 3-year survival was 46.9%.CONCLUSION: The survival of Korean patients with R/R childhood ALL was dismal despite a reasonable CR rate, whereas that of those who received HSCT after CR or CRp was excellent. More treatment options are needed to improve the overall outcome of R/R childhood ALL.
Blood Platelets ; Cytarabine ; Diagnosis ; Hematopoietic Stem Cell Transplantation ; Humans ; Idarubicin ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Prognosis ; Retrospective Studies ; Vincristine

PURPOSE: To identify magnetic resonance imaging (MRI) findings of leukemic infiltration of optic nerve and optic neuritis in leukemic patients with emphasis of clinical findings as reference standard to differentiate them. MATERIALS AND METHODS: MRI and clinical findings of 7 patients diagnosed as leukemic infiltration of optic nerve (n = 5) and optic neuritis (n = 2) in our institution between July 2006 and August 2015were reviewed retrospectively. In particular, MR imaging findings involved perineural enhancement and thickening of optic nerve and its degree, signal intensity, laterality (unilateral/bilateral), intraconal fat infiltration and its degree, and associated central nervous system abnormalities. RESULTS: Of 5 cases of leukemic infiltration of optic nerve, 4 cases showed positive cerebrospinal fluid (CSF) study for leukemia relapse and 1 case was positive on bone marrow (BM) biopsy only. Moreover, of 5 leukemic infiltration of optic nerve, 2 cases showed the most specific MR findings for leukemic central nervous system involvement including 1 prominent leptomeningeal enhancement and 1 chloroma. However, other MR imaging findings of the patients with leukemic infiltration or optic neuritis such as thickening and perineural enhancement of optic nerves are overlapped. CONCLUSION: Enhancement and thickening of optic nerve were overlapped MR findings in leukemic infiltration of optic nerve and optic neuritis. Our findings suggest that enhancing optic nerve thickening with associated central nervous system MR abnormality favors the diagnosis of leukemic infiltration of optic nerve, especially in patients with history of acute lymphoblastic leukemia. However, CSF and BM study were required for differentiation between leukemic infiltration of optic nerve and optic neuritis.
Biopsy ; Bone Marrow ; Central Nervous System ; Cerebrospinal Fluid ; Diagnosis ; Humans ; Leukemia ; Leukemic Infiltration* ; Magnetic Resonance Imaging ; Optic Nerve Diseases ; Optic Nerve* ; Optic Neuritis* ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Recurrence ; Retrospective Studies ; Sarcoma, Myeloid

This study was conducted to investigate long-term neurocognitive outcomes and to determine associated risk factors in a cohort of Korean survivors of childhood acute lymphoblastic leukemia (ALL). Forty-two survivors of ALL were compared with 42 healthy controls on measures of a neurocognitive test battery. We analysed potential risk factors (cranial irradiation, sex, age at diagnosis, elapsed time from diagnosis, and ALL risk group) on neurocognitive outcomes. ALL patients had lower, but non-significant full-scale intelligence quotient (FSIQ, 107.2 +/- 12.2 vs. 111.7 +/- 10.2), verbal intelligence quotient (VIQ, 107.7 +/- 13.6 vs. 112.2 +/- 11.4), and performance intelligence quotient (PIQ, 106.3 +/- 14.2 vs. 110.1 +/- 10.7) scores than healthy controls. However, patients treated with cranial irradiation performed significantly lower on FSIQ (102.2 +/- 8.1), VIQ (103.3 +/- 11.7), and PIQ (101.4 +/- 13.2) compared to non-irradiated patients and healthy controls. ALL patients also had poor attention, concentration, and executive functions. Among ALL survivors, cranial irradiation was a risk factor for poor FSIQ, being male was a risk factor for poor PIQ, and younger age was a risk factor for poor attention. Therefore, the delayed cognitive effects of ALL treatment and its impact on quality of life require continuing monitoring and management.
Adolescent ; Age Factors ; Child ; *Cognition ; Female ; Humans ; Intelligence ; Male ; Precursor Cell Lymphoblastic Leukemia-Lymphoma/mortality/*psychology ; *Survivors ; Tertiary Healthcare

Alveolar soft part sarcoma (ASPS) is an extremely rare malignant soft tissue sarcoma primarily affecting young patients. It usually occurs in the lower extremities, although it can occur in soft tissue anywhere in the body. However, to our knowledge, there has been no case of primary ASPS originating from the kidney in the literature. We herein present the imaging and clinical features of an ASPS which occurred in a 16-year-old male presented as a palpable mass in the left side of the abdomen.
Adolescent ; Biopsy ; Diagnostic Imaging/methods ; Humans ; Kidney/pathology ; Kidney Neoplasms/*diagnosis/pathology ; Male ; Rare Diseases/*diagnosis/pathology ; Sarcoma, Alveolar Soft Part/*diagnosis/pathology

Langerhans cell sarcoma (LCS) is a neoplastic proliferation of Langerhans cells with malignant cytological features and multi-organ involvement that typically has a poor prognosis. We experienced 2 cases of LCS in children less than 2 years of age and report them based primarily on CT and MR findings. Both children had findings of hepatosplenomegaly with low-attenuation nodular lesions, had multiple lymphadenopathy, and had shown recurrent lesions invading the skull during follow-up after chemotherapy.
Female ; Hepatomegaly/diagnosis ; Humans ; Infant ; Langerhans Cell Sarcoma/*diagnosis ; Magnetic Resonance Imaging ; Mediastinal Neoplasms/*diagnosis ; Neoplasm Recurrence, Local ; Skull Neoplasms/*diagnosis ; Splenomegaly/diagnosis ; Tomography, X-Ray Computed

The aim of this study was to investigate the diphtheria-tetanus-pertussis antibody titers after antineoplastic treatment and to suggest an appropriate vaccination approach for pediatric hemato-oncologic patients. A total of 146 children with either malignancy in remission after cessation of therapy or bone marrow failure were recruited. All children had received routine immunization including diphtheria-tetanus-acellular pertussis vaccination before diagnosis of cancer. The serologic immunity to diphtheria, tetanus and pertussis was classified as: completely protective, partially protective, or non-protective. Non-protective serum antibody titer for diphtheria, tetanus and pertussis was detected in 6.2%, 11.6%, and 62.3% of patients, respectively, and partial protective serum antibody titer for diphtheria, tetanus and pertussis was seen in 37%, 28.1%, and 8.9% of patients. There was no significant correlation between the severity of immune defect and age, gender or underlying disease. Revaccination after antineoplastic therapy showed significantly higher levels of antibody for each vaccine antigen. Our data indicates that a large proportion of children lacked protective serum concentrations of antibodies against diphtheria, tetanus, and pertussis. This suggests that reimmunization of these patients is necessary after completion of antineoplastic treatment. Also, prospective studies should be undertaken with the aim of devising a common strategy of revaccination.
Adolescent ; Age Factors ; Antibodies, Bacterial/blood/immunology ; Antineoplastic Agents/therapeutic use ; Child ; Child, Preschool ; Diphtheria/immunology/prevention & control ; Diphtheria-Tetanus-acellular Pertussis Vaccines/*immunology ; Female ; Hematologic Neoplasms/*diagnosis/drug therapy ; Humans ; Immunization, Secondary ; Lymphoma/diagnosis/drug therapy ; Male ; Neuroblastoma/diagnosis/drug therapy ; Sex Factors ; Tetanus/immunology/prevention & control ; Whooping Cough/immunology/prevention & control

Respiratory syncytial virus (RSV) is the major cause of lower respiratory tract infection in infants. Life-threatening RSV infection is often reported in young children and immunocompromised hosts. Since there is no report on ribavirin therapy for RSV pneumonia in pediatric cancer patients in Korea, we report one case of RSV pneumonia that developed in an infant with acute lymphoblastic leukemia (ALL). Despite administration of oral ribavirin and intravenous immunoglobulin, the patient's respiratory distress worsened and admission to an intensive care unit was necessary. Chest x-ray showed multifocal consolidation, pneumothorax, and pneumomediastinum. Treatment with aerosolized ribavirin led to significant clinical improvement. The role of aerosolized ribavirin is still controversial, but it might have a therapeutic potential for severe RSV pneumonia in children with leukemia.
Child ; Humans ; Immunocompromised Host ; Immunoglobulins ; Infant ; Intensive Care Units ; Korea ; Leukemia ; Mediastinal Emphysema ; Pneumonia ; Pneumothorax ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Respiratory Syncytial Viruses ; Respiratory Tract Infections ; Ribavirin ; Thorax

PURPOSE: Acute appendicitis has been reported to be relatively rare in pediatric leukemia patients but there is no official data for this in Korea. And there is no consensus for its treatment in this population. MATERIALS AND METHODS: We conducted a retrospective study of 7 patients diagnosed with appendicitis among 1209 pediatric patients who were diagnosed with leukemia from 1996 to 2008 at a single institution in Korea. RESULTS: The median age at the time of the diagnosis of appendicitis was 12 years (range: 3-15 years), and 3 of the patients were male. The median absolute neutrophil count (ANC) at the time of diagnosis was 0.99x10(9)/L (range: 0-3x10(9)/L). The mean time from the onset of symptoms to the diagnosis was 4 days. All 7 leukemia patients with appendicitis underwent surgery and they demonstrated a survival of 100% without significant complications. CONCLUSION: The incidence of appendicitis in pediatric leukemia patients was 0.57% in our study. Early diagnosis with abdominal ultrasound or computed tomography and early surgical resection in leukemic patient with acute appendicitis may be a safer and more effective treatment option. Even when perforation has already occurred and when the patient has an ANC of 0x10(9)/L, surgical treatment may improve overall survival without incurring significant complications.
Acute Disease ; Adolescent ; Appendectomy/adverse effects ; Appendicitis/*diagnosis/surgery ; Child ; Child, Preschool ; Female ; Humans ; Infant ; Korea ; Leukemia/*physiopathology ; Male ; Retrospective Studies

BACKGROUND: Diamond Blackfan anemia (DBA), characterized by impaired red cell production, is a rare condition that is usually symptomatic in early infancy. The purpose of this study was to assess nationwide experiences of DBA encountered over a period of 20 years. METHODS: The medical records of 56 patients diagnosed with DBA were retrospectively reviewed from November 1984 to July 2010. Fifteen institutions, including 13 university hospitals, participated in this study. RESULTS: The male-to-female ratio of patients with DBA was 1.67:1. The median age of diagnosis was 4 months, and 74.1% were diagnosed before 1 year of age. From 2000 to 2009, annual incidence was 6.6 cases per million. Excluding growth retardation, 38.2% showed congenital defects: thumb deformities, ptosis, coarctation of aorta, ventricular septal defect, strabismus, etc. The mean hemoglobin concentration was 5.1+/-1.9 g/dL, mean corpuscular volume was 93.4+/-11.6 fL, and mean number of reticulocytes was 19,700/mm3. The mean cellularity of bone marrow was 75%, with myeloid:erythroid ratio of 20.4:1. After remission, 48.9% of patients did not need further steroids. Five patients with DBA who received hematopoietic transplantation have survived. Cancer developed in 2 cases (3.6%). CONCLUSION: The incidence of DBA is similar to data already published, but our study had a male predilection. Although all patients responded to initial treatment with steroids, about half needed further steroids after remission. It is necessary to collect further data, including information regarding management pathways, from nationwide DBA registries, along with data on molecular analyses.
Anemia ; Anemia, Diamond-Blackfan ; Aortic Coarctation ; Bone Marrow ; Congenital Abnormalities ; Diamond ; Erythrocyte Indices ; Heart Septal Defects, Ventricular ; Hemoglobins ; Hospitals, University ; Humans ; Incidence ; Korea ; Male ; Medical Records ; Registries ; Reticulocytes ; Retrospective Studies ; Steroids ; Strabismus ; Thumb ; Transplants