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1.
Article in English | WPRIM | ID: wpr-913845

ABSTRACT

Purpose@#In 2017, the Children’s Hepatic Tumors International Collaboration-Hepatoblastoma Stratification (CHIC-HS) system was introduced. We aimed to evaluate the accuracy of CHIC-HS System for the prediction of event-free survival (EFS) in Korean pediatric patients with hepatoblastoma. @*Materials and Methods@#This two-center retrospective study included consecutive Korean pediatric patients with histopathologically confirmed hepatoblastoma from March 1988 through September 2019. We compared EFS among four risk groups according to the CHIC-HS system. Discriminatory ability of CHIC-HS system was also evaluated using optimism-corrected C-statistics. Factors associated with EFS were explored using multivariable Cox regression analysis. @*Results@#We included 129 patients (mean age, 2.6±3.3 years; female:male, 63:66). The 5-year EFS rates in the very low, low, intermediate, and high-risk groups, according to the CHIC-HS system were 90.0%, 82.8%, 73.5%, and 51.3%, respectively. The CHIC-HS system aligned significantly well with EFS outcomes (p=0.004). The optimism-corrected C index of CHIC-HS was 0.644 (95% confidence interval [CI], 0.561 to 0.727). Age ≥ 8 (vs. age ≤ 2; hazard ratio [HR], 2.781; 95% CI, 1.187 to 6.512; p=0.018), PRE-Treatment EXTent of tumor (PRETEXT) stage IV (vs. PRETEXT I or II; HR, 2.774; 95% CI, 1.228 to 5.974; p=0.009), and presence of metastasis (HR, 2.886; 95% CI, 1.457 to 5.719; p=0.002), which are incorporated as the first three nodes in the CHIC-HS system, were independently associated with EFS. @*Conclusion@#The CHIC-HS system aligned significantly well with EFS outcomes in Korean pediatric patients with hepatoblastoma. Age group, PRETEXT stage, and presence of metastasis were independently associated with EFS.

2.
Article in English | WPRIM | ID: wpr-897430

ABSTRACT

Purpose@#Atypical teratoid/rhabdoid tumor (ATRT) is a highly aggressive malignancy with peak incidence in children aged less than 3 years. Standard treatment for central nervous system ATRT in children under the age of 3 years have not been established yet. The objective of this study was to analyze characteristics and clinical outcomes of ATRT in children aged less than 3 years. @*Materials and Methods@#A search of medical records from seven centers was performed between January 2005 and December 2016. @*Results@#Forty-three patients were enrolled. With a median follow-up of 90 months, 27 patients (64.3%) showed at least one episode of disease progression (PD). The first date of PD was at 160 days after diagnosis. The 1- and 3-year progression-free survivals (PFS) were 51.2% and 28.5%, respectively. The 1- and 3-year overall survivals were 61.9% and 38.1%, respectively. The 3-year PFS was improved from 0% in pre-2011 to 47.4% in post-2011. Excluding one patient who did not receive any further therapy after surgery, 27 patients died due to PD (n=21), treatment-related toxicity (n=5), or unknown cause (n=1). In univariate analysis, factors associated with higher 3-year PFS were no metastases, diagnosis after 2011, early adjuvant radiotherapy, and high-dose chemotherapy (HDCT). In multivariate analysis, the use of HDCT and adjuvant radiotherapy remained significant prognostic factors for PFS (both p < 0.01). @*Conclusion@#Aggressive therapy including early adjuvant radiotherapy and HDCT could be considered to improve outcomes of ATRT in children under the age of 3 years.

3.
Article in English | WPRIM | ID: wpr-889726

ABSTRACT

Purpose@#Atypical teratoid/rhabdoid tumor (ATRT) is a highly aggressive malignancy with peak incidence in children aged less than 3 years. Standard treatment for central nervous system ATRT in children under the age of 3 years have not been established yet. The objective of this study was to analyze characteristics and clinical outcomes of ATRT in children aged less than 3 years. @*Materials and Methods@#A search of medical records from seven centers was performed between January 2005 and December 2016. @*Results@#Forty-three patients were enrolled. With a median follow-up of 90 months, 27 patients (64.3%) showed at least one episode of disease progression (PD). The first date of PD was at 160 days after diagnosis. The 1- and 3-year progression-free survivals (PFS) were 51.2% and 28.5%, respectively. The 1- and 3-year overall survivals were 61.9% and 38.1%, respectively. The 3-year PFS was improved from 0% in pre-2011 to 47.4% in post-2011. Excluding one patient who did not receive any further therapy after surgery, 27 patients died due to PD (n=21), treatment-related toxicity (n=5), or unknown cause (n=1). In univariate analysis, factors associated with higher 3-year PFS were no metastases, diagnosis after 2011, early adjuvant radiotherapy, and high-dose chemotherapy (HDCT). In multivariate analysis, the use of HDCT and adjuvant radiotherapy remained significant prognostic factors for PFS (both p < 0.01). @*Conclusion@#Aggressive therapy including early adjuvant radiotherapy and HDCT could be considered to improve outcomes of ATRT in children under the age of 3 years.

4.
Cancer Research and Treatment ; : 1184-1194, 2021.
Article in English | WPRIM | ID: wpr-913813

ABSTRACT

Purpose@#Effectiveness and safety of clofarabine (one of the treatment mainstays in pediatric patients with relapsed/refractory acute lymphoblastic leukemia [ALL]) was assessed in Korean pediatric patients with ALL to facilitate conditional coverage with evidence development. @*Materials and Methods@#In this multicenter, prospective, observational study, patients receiving clofarabine as mono/combination therapy were followed up every 4-6 weeks for 6 months or until hematopoietic stem cell transplantation (HSCT). Response rates, survival outcomes, and adverse events were assessed. @*Results@#Sixty patients (2-26 years old; 65% B-cell ALL, received prior ≥ 2 regimen, 68.3% refractory to previous regimen) were enrolled and treated with at least one dose of clofarabine; of whom 26 (43.3%) completed 6 months of follow-up after the last dose of clofarabine. Fifty-eight patients (96.7%) received clofarabine combination therapy. Overall remission rate (complete remission [CR] or CR without platelet recovery [CRp]) was 45.0% (27/60; 95% confidence interval [CI], 32.4 to 57.6) and the overall response rate (CR, CRp, or partial remission [PR]) was 46.7% (28/60; 95% CI, 34.0 to 59.3), with 11 (18.3%), 16 (26.7%), and one (1.7%) patients achieving CR, CRp, and PR, respectively. The median time to remission was 5.1 weeks (95% CI, 4.7 to 6.1). Median duration of remission was 16.6 weeks (range, 2.0 to 167.6 weeks). Sixteen patients (26.7%) proceeded to HSCT. There were 24 deaths; 14 due to treatment-emergent adverse events. @*Conclusion@#Remission with clofarabine was observed in approximately half of the study patients who had overall expected safety profile; however, there was no favorable long-term survival outcome in this study.

5.
Yonsei Medical Journal ; : 774-779, 2020.
Article | WPRIM | ID: wpr-833408

ABSTRACT

Purpose@#Histiocytic and dendritic cell neoplasms are rare hematologic tumors.This study aimed to describe the epidemiologic features of the entire spectrum of histiocytic and dendritic cell neoplasms, including clinicopathological variables and patient outcomes. @*Materials and Methods@#We comprehensively reviewed 274 patients who were diagnosed with histiocytic and dendritic neoplasms at Severance Hospital, Seoul, South Korea between 1995 and 2018. @*Results@#The most common neoplasm was Langerhans cell histiocytosis (LCH), followed by dermal xanthogranuloma. Among non-LCH sarcomas, histiocytic sarcoma (HS) showed a relatively high prevalence, followed by follicular dendritic cell sarcoma (FDCS). Disseminated juvenile xanthogranuloma (DJG), Erdheim-Chester disease (ECD), indeterminate dendritic cell tumor (IDCT), and interdigitating dendritic cell sarcoma (IDCS) rarely occurred. Generally, these tumors presented in childhood, although the non-LCH sarcoma (HS/FDCS/IDCS/IDCT) group of tumors and ECD occurred in late adulthood. Multiorgan involvement and advanced Ann-Arbor stage, as well as recurrence and death of disease, were not uncommon. The non-LCH sarcoma group had the worst overall survival, compared to the DJG, ECD, and LCH groups. @*Conclusion@#Our findings indicate that histiocytic and dendritic cell neoplasms exhibit heterogeneous epidemiologic characteristics and that some patients may have unfavorable outcomes, especially those with non-LCH sarcoma.

6.
Article | WPRIM | ID: wpr-832094

ABSTRACT

Background@#Approximately 8% of male patients presenting with primary mediastinal germ cell tumors (GCTs) have Klinefelter syndrome (KS), while patients diagnosed with retroperitoneal GCTs also exhibit a range of chromosomal abnormalities. The exact mechanism underlying the development of GCTs in Klinefelter syndrome is unknown, but KS frequently goes underdiagnosed as a result of its varied symptoms and a low general awareness of this condition. Thus, the Children’s Oncology Group recommends screening of Klinefelter syndrome in pediatric and adolescent male subjects who present with GCTs. @*Methods@#We retrospectively reviewed the medical records of extragonadal germ cell tumor patients treated at Severance hospital, department of pediatrics or division of pediatric hematology-oncology over the last ten years. @*Results@#A total of 95 patients with extragonadal germ cell tumors were included in this study. Karyotyping was done in eight patients out of 95 patients, three patients with KS and one patient with Down syndrome. Twelve of extragonadal GCT patients presented at mediastinum, with most common histology of mature teratoma, and three patients presented with chromosomal abnormalities, two with KS and one with Down syndrome. A total of nine patients were diagnosed with retroperitoneal GCTs and only one had KS. @*Conclusion@#We described the characteristics of 95 cases of extragonadal GCTs. Although the mechanism of extragonadal GCTs in KS is not clear, karyotyping in pediatric and adolescent extragonadal GCT patients could be helpful in figuring out chromosomal abnormalities including KS and their roles in GCT pathophysiology, which can contribute to improve one’s health.

7.
Article in English | WPRIM | ID: wpr-831569

ABSTRACT

Background@#Hodgkin's lymphoma (HL) constitutes 10%–20% of all malignant lymphomas and has a high cure rate (5-year survival, around 90%). Recently, interest has increased concerning preventing secondary complications (secondary cancer, endocrine disorders) in long-term survivors. We aimed to study the epidemiologic features and therapeutic outcomes of HL in children, adolescents, and young adults in Korea. @*Methods@#We performed a multicenter, retrospective study of 224 patients aged < 25 years diagnosed with HL at 22 participating institutes in Korea from January 2007 to August 2016. @*Results@#A higher percentage of males was diagnosed at a younger age. Nodular sclerosis histopathological HL subtype was most common, followed by mixed cellularity subtype.Eighty-one (36.2%), 101 (45.1%), and 42 (18.8%) patients were classified into low, intermediate, and high-risk groups, respectively. Doxorubicin, bleomycin, vinblastine, dacarbazine was the most common protocol (n = 102, 45.5%). Event-free survival rate was 86.0% ± 2.4%, while five-year overall survival (OS) rate was 96.1% ± 1.4%: 98.7% ± 1.3%, 97.7% ± 1.6%, and 86.5% ± 5.6% in the low, intermediate, and high-risk groups, respectively (P = 0.021). Five-year OS was worse in patients with B-symptoms, stage IV disease, highrisk, splenic involvement, extra-nodal lymphoma, and elevated lactate dehydrogenase level.In multivariate analysis, B-symptoms and extra-nodal involvement were prognostic factors for poor OS. Late complications of endocrine disorders and secondary malignancy were observed in 17 and 6 patients, respectively. @*Conclusion@#This is the first study on the epidemiology and treatment outcomes of HL in children, adolescents, and young adults in Korea. Future prospective studies are indicated to develop therapies that minimize treatment toxicity while maximizing cure rates in children, adolescents, and young adults with HL.

8.
Article | WPRIM | ID: wpr-831054

ABSTRACT

Purpose@#Loss of heterozygosity (LOH) at chromosomes 1p and 16q is a poor prognostic factor infavorable histology Wilms tumor (FHWT). This study investigated the prevalence of LOH at1p and 16q and evaluated its prognostic value in Korean children with FHWT. @*Materials and Methods@#We analyzed 101 FHWT patients who were diagnosed between 1996 and 2016 in KoreanSociety of Pediatric Hematology Oncology Group hospitals. Using paraffin-embedded kidneytissue samples sent from each center, we reviewed LOH at 1p and 16q in each patient andassessed the prognostic value of LOH status for clinical parameters affecting event-freesurvival (EFS). @*Results@#Of the 101 patients, 12 (11.9%) experienced recurrence; the 3-year EFS was 87.6%. LOHat 1p or 16q was detected in 19 patients (18.8%), with five having LOH at both 1q and 16q.The frequency of LOH at 1p was higher among younger patients (p=0.049), but there wasno difference in LOH prevalence according to tumor stage. In the multivariate analysis, LOHat 16q was a significant negative prognostic factor affecting EFS (3-year EFS, 73.7% vs.91.1%; hazard ratio, 3.95; p=0.037), whereas LOH at 1p was not (p=0.786). @*Conclusion@#LOH at 16q was a significant negative prognostic factor affecting outcome in Korean pediatricFHWT patients. Due to the small sample size of this study, large-scale multicenter trialsare warranted to investigate the prognostic value of LOH at 1p and 16q in Korean childrenwith FHWT.

9.
Article in Korean | WPRIM | ID: wpr-717646

ABSTRACT

BACKGROUND: Comprehensive clinical and radiologic follow-up is needed to preserve joint functions and quality of life in hemophilia using clinimetric tools such as Hemophilia joint health score (HJHS) or Pettersson score (PS). We investigated the joint health status evaluated using the tools in Korean hemophilia patients. METHODS: We reviewed retrospectively medical records to collect clinical parameters, HJHS and PS, who were followed up in Severance Hospital, Seoul, Korea. The correlation between HJHS and PS, and the effect of the prophylaxis for hemophilia on the outcomes measured with the scores were evaluated. The prophylaxis proportion (PP) was calculated as the proportion of prophylaxis duration to each patient's life time. RESULTS: Total of 28 patients with severe hemophilia were enrolled. Twelve patients (42.8%) were less than 20 years old. Total of 23 patients had experienced prophylaxis during their lives, and median PP was 39.7%. There was significant correlation between HJHS and PS (P 20 years old. CONCLUSION: HJHS and PS were positively correlated. Each score increased as the patient's age increased. The prophylaxis had protective effect on joint health. The prospective evaluation of HJHS and PS will be needed to prove the effect of proper management on the joint health status.


Subject(s)
Follow-Up Studies , Health Status , Hemophilia A , Humans , Joints , Korea , Medical Records , Patient Outcome Assessment , Prospective Studies , Quality of Life , Retrospective Studies , Seoul
10.
Article in English | WPRIM | ID: wpr-717644

ABSTRACT

BACKGROUND: The accurate and early diagnosis of acute myeloid leukemia (AML) is important to choose proper treatment option depending on the risk stratification. The delta neutrophil index (DNI) is a relatively new blood marker that indicates the proportion of immature granulocytes in peripheral blood circulation. This study aimed to evaluate the diagnostic value of the DNI for detecting AML in the early phase of acute leukemia. METHODS: We retrospectively analyzed laboratory tests and bone marrow study results of 163 pediatric patients with acute leukemia admitted to the emergency department, who were diagnosed with acute leukemia. An automatic analyzer (ADVIA 2120 Hematology System; Siemens Healthcare Diagnostics, Forchheim, Germany) was used to measure the DNI in the peripheral blood of each patient. RESULTS: The mean DNI was significantly different between the AML (N=39) and non-AML (N=124) groups (P < 0.05), and the DNI was the only significant marker for predicting AML in patients with acute leukemia (odds ratio, 1.328; P < 0.05). The DNI more than 4.4% has the highest predictability for distinguishing the patients with AML from the patients with acute leukemia. The mean DNI of the acute promyelocytic leukemia (APL, N=8) group was statistically higher than that of the non-APL group (N=31, P=0.019), but the DNI was not significant in the univariate logistic regression analysis. CONCLUSION: The DNI might be a promising peripheral blood marker for predicting AML in the early work-up of patients with acute leukemia.


Subject(s)
Blood Circulation , Bone Marrow , Child , Delivery of Health Care , Early Diagnosis , Emergency Service, Hospital , Granulocytes , Hematology , Humans , Leukemia , Leukemia, Myeloid, Acute , Leukemia, Promyelocytic, Acute , Logistic Models , Neutrophils , Retrospective Studies
11.
Article in English | WPRIM | ID: wpr-715965

ABSTRACT

PURPOSE: Although studies regarding dental developmental disturbances after childhood cancer treatment have increased, they have many limitations. Studies analyzing the significance of independent clinical risk factors with regard to the dental health status are also rare. We aimed to investigate the risk factors for dental developmental disturbances, particularly severe disturbances, in childhood cancer survivors (CCS). MATERIALS AND METHODS: Oral examinations and retrospective reviews of medical and panoramic radiographs were performed for 196 CCS (mean age, 15.6 years). Cancer type, age at diagnosis, treatment modality, type and accumulated dose of administered drugs, and dose and site of radiation were recorded. Dental developmental disturbances were diagnosed using panoramic radiographs and graded for severity according to the Modified Dental Defect Index (MDDI). Descriptive statistics and multivariate analyseswere performed to determine the association between dental abnormalities and clinical factors. RESULTS: In total, 109 CCS (55.6%) exhibited at least one dental anomaly, and the median value of MDDI was 2.5. Microdontia (30.6%) was the most prevalent anomaly, followed by tooth agenesis (20.4%), V-shaped roots (14.8%), and taurodontism (10.2%). Multivariate analysis revealed that a young age at diagnosis (≤ 3 years), a history of hematopoietic stem cell transplantation, the use of multiple classes of chemotherapeutic agents (≥ 4 classes), and the use of heavy metal agents were significant risk factors for severe dental disturbances. CONCLUSION: CCS with any of the above risk factors for severe developmental disturbances should be comprehensively followed up to minimize adverse consequences to their dental development and preserve their future dental health.


Subject(s)
Diagnosis , Diagnosis, Oral , Hematopoietic Stem Cell Transplantation , Humans , Multivariate Analysis , Odontogenesis , Retrospective Studies , Risk Factors , Survivors , Tooth , Tooth Abnormalities
12.
Article in English | WPRIM | ID: wpr-714195

ABSTRACT

Erdheim-Chester disease (ECD) is a rare non-Langerhan's cell histiocytosis disorder characterized by replacement of normal tissue by lipid-laden histiocytes affecting various organs. A few pediatric cases have been reported worldwide. Here we present a child with leukemia who was diagnosed as ECD. A 2-year and 11-month old boy diagnosed with high risk acute lymphoblastic leukemia (ALL) at the age of 17 months, received allogeneic hematopoietic stem cell transplantation (HSCT) at the age of 2 years old. Six months after the transplantation, the patient was admitted to the hospital with palpable left calf nodules. Bone marrow study suggested ECD without leukemia with complete chimerism status. Excisional biopsy of the left calf nodule showed ‘aggregation of non-Langerhan's cell type epitheloid histiocytes’; clinically suggestive of ECD. The patient was started on vinblastine and corticosteroid treatment.


Subject(s)
Biopsy , Bone Marrow , Child , Chimerism , Erdheim-Chester Disease , Hematopoietic Stem Cell Transplantation , Histiocytes , Histiocytosis , Humans , Leukemia , Male , Precursor Cell Lymphoblastic Leukemia-Lymphoma , Vinblastine
13.
Article in English | WPRIM | ID: wpr-714194

ABSTRACT

Cyclophosphamide-induced cardiotoxicity is an uncommon complication especially in patients who have never undergone mediastinal irradiation or cardiotoxic chemotherapy and do not have underlying cardiac diseases. Here, we describe the case of a 19-year-old female with chronic myeloid leukemia. She was previously treated with oral tyrosine kinase inhibitors and developed cardiomyopathy after receiving infusion of 60 mg/kg intravenous cyclophosphamide for two days with a conditioning regimen for allogenic hematopoietic stem cell transplantation. Severe thickening of the left ventricle and reduced ejection fraction without triggering agents were characteristic for cyclophosphamide-induced cardiomyopathy. Her NT-pro BNP and troponin T concentrations surged to >70,000 pg/mL (0=130 pg/mL) and 2,031 pg/mL (0-14 pg/mL), respectively, during the course of the therapy and multiple organ failure seemed imminent evidenced by unresponsive decline in blood pressure. However, with close monitoring and persistent conservative management which consisted of intravenous hydration, continuous hemodialysis, and mechanical ventilation, her condition recovered.


Subject(s)
Blood Pressure , Cardiomyopathies , Cardiotoxicity , Cyclophosphamide , Drug Therapy , Female , Heart Diseases , Heart Failure , Heart Ventricles , Heart , Hematopoietic Stem Cell Transplantation , Humans , Leukemia, Myelogenous, Chronic, BCR-ABL Positive , Multiple Organ Failure , Protein-Tyrosine Kinases , Renal Dialysis , Respiration, Artificial , Troponin T , Young Adult
14.
Article in English | WPRIM | ID: wpr-167296

ABSTRACT

PURPOSE: Malignant peripheral nerve sheath tumors (MPNSTs) are a rare subtype of sarcoma that occur spontaneously or in association with neurofibromatosis type 1 (NF-1). This study aimed to clinically differentiate these types of MPNSTs. MATERIALS AND METHODS: The study reviewed 95 patients diagnosed with and treated for MPNST at Yonsei University Health System, Seoul, Korea over a 27-year period. The clinical characteristics, prognostic factors, and treatment outcomes of sporadic MPNST (sMPNST) and NF-1 associated MPNST (NF-MPNST) cases were compared. RESULTS: Patients with NF-MPNST had a significantly lower median age (32 years vs. 45 years for sMPNST, p=0.012), significantly larger median tumor size (8.2 cm vs. 5.0 cm for sMPNST, p < 0.001), and significantly larger numbers of imaging studies and surgeries (p=0.004 and p < 0.001, respectively). The 10-year overall survival (OS) rate of the patients with MPNST was 52±6%. Among the patients with localized MPNST, patients with NF-MPNST had a significantly lower 10-year OS rate (45±11% vs. 60±8% for sMPNST, p=0.046). Univariate analysis revealed the resection margin, pathology grade, and metastasis to be significant factors affecting the OS (p=0.001, p=0.020, and p < 0.001, respectively). Multivariate analysis of the patients with localized MPNST identified R2 resection and G1 as significant prognostic factors for OS. CONCLUSION: NF-MPNST has different clinical features from sMPNST and requires more careful management. Further study will be needed to develop specific management plans for NF-MPNST.


Subject(s)
Humans , Korea , Multivariate Analysis , Neoplasm Metastasis , Neurilemmoma , Neurofibromatoses , Neurofibromatosis 1 , Pathology , Sarcoma , Seoul
15.
Article in English | WPRIM | ID: wpr-788610

ABSTRACT

BACKGROUND: Although combined chemotherapy has increased survival rates among children with cancer, such treatments can induce sensorineural hearing loss. Therefore, we aimed to identify risk factors for hearing impairments in patients with childhood cancer.METHODS: Audiograms were obtained from 115 patients with childhood cancer and survivors (age < 20 years). Pure tone audiometry (PTA) was performed at octave intervals within the range of 250-8000 Hz. We evaluated clinical risk factors associated with hearing impairments. Hearing loss was evaluated based on the maximal decibel (dB) loss in any frequency for each ear (RA(max) or LA(max)) and weighted mean dB loss for specific frequencies (RA(avg) or LA(avg)).RESULTS: Forty percent of patients (N=46) exhibited hearing loss >20 dB based on the weighted mean value in either ear. Severe hearing impairments were observed in 56% of patients with brain tumors. Although cisplatin or vinca alkaloids were significant risk factors for hearing impairment, the use of both cisplatin and vinca alkaloids exhibited the highest odds ratio for hearing impairment (P < 0.001, < 0.001 for R/LA(max); P=0.099, 0.039 for R/LA(avg)). Multivariate analysis revealed that the use of both cisplatin and vinca alkaloids was an independent risk factor for hearing impairment based on RA(max), LA(max), and LA(avg) (P < 0.001, < 0.001, 0.039, respectively).CONCLUSION: Our findings indicate that cisplatin and vinca alkaloids exert an additive effect on the risk of hearing impairment in survivors of childhood cancer. Further prospective studies are thus required to determine the most effective chemotherapeutic regimen for reducing ototoxicity.


Subject(s)
Audiometry , Brain Neoplasms , Child , Cisplatin , Drug Therapy , Ear , Hearing Loss , Hearing Loss, Sensorineural , Hearing , Humans , Multivariate Analysis , Odds Ratio , Prospective Studies , Risk Factors , Survival Rate , Survivors , Vinca Alkaloids , Vinca
16.
Yonsei Medical Journal ; : 816-822, 2017.
Article in English | WPRIM | ID: wpr-81890

ABSTRACT

PURPOSE: Children and adolescents diagnosed with cancer experience emotional distress, such as sadness, worrying, and irritability. However, there is little information about the psychological well-being of parents at the time of their child's diagnosis. We sought to identify factors that were associated with emotional distress in cancer patients as a basis for developing innovative psychological interventions. MATERIALS AND METHODS: A retrospective chart review was performed on patients newly diagnosed with cancer at a single center in Korea from 2014 to 2016. Eighty-five patients and their mothers completed psychological inventories. To determine factors associated with emotional distress in patients, we assessed the psychological inventory results using multiple linear regression after performing correlation analysis. RESULTS: The maternal Beck Depression Inventory-II (BDI-II) score was positively correlated with total problem scores and externalizing scores in patients aged less than 7 years. In patients aged 7–12 years, there was no significant association between the patient's emotional distress and other variables. In contrast, the maternal BDI-II score was the strongest factor associated with patient depression in adolescents. CONCLUSION: We suggest that the most important factor affecting emotional distress in children and adolescents with cancer is maternal depression, especially in patients aged 1–6 years and aged 13–17 years. Understanding the factors associated with emotional distress of cancer patients allows us to develop early psychiatric interventions for patients and their parents at the initial psychological crisis.


Subject(s)
Adolescent , Anxiety , Child , Depression , Diagnosis , Equipment and Supplies , Humans , Korea , Linear Models , Mother-Child Relations , Mothers , Parents , Retrospective Studies
17.
Article in English | WPRIM | ID: wpr-23108

ABSTRACT

BACKGROUND: Although combined chemotherapy has increased survival rates among children with cancer, such treatments can induce sensorineural hearing loss. Therefore, we aimed to identify risk factors for hearing impairments in patients with childhood cancer. METHODS: Audiograms were obtained from 115 patients with childhood cancer and survivors (age 20 dB based on the weighted mean value in either ear. Severe hearing impairments were observed in 56% of patients with brain tumors. Although cisplatin or vinca alkaloids were significant risk factors for hearing impairment, the use of both cisplatin and vinca alkaloids exhibited the highest odds ratio for hearing impairment (P < 0.001, < 0.001 for R/LA(max); P=0.099, 0.039 for R/LA(avg)). Multivariate analysis revealed that the use of both cisplatin and vinca alkaloids was an independent risk factor for hearing impairment based on RA(max), LA(max), and LA(avg) (P < 0.001, < 0.001, 0.039, respectively). CONCLUSION: Our findings indicate that cisplatin and vinca alkaloids exert an additive effect on the risk of hearing impairment in survivors of childhood cancer. Further prospective studies are thus required to determine the most effective chemotherapeutic regimen for reducing ototoxicity.


Subject(s)
Audiometry , Brain Neoplasms , Child , Cisplatin , Drug Therapy , Ear , Hearing Loss , Hearing Loss, Sensorineural , Hearing , Humans , Multivariate Analysis , Odds Ratio , Prospective Studies , Risk Factors , Survival Rate , Survivors , Vinca Alkaloids , Vinca
18.
Article in English | WPRIM | ID: wpr-788575

ABSTRACT

Ataxia-telangiectasia (AT) is characterized by cerebellar ataxia, progressive immunodeficiency, radiation sensitivity, telangiectasia, and predisposition to malignancy. AT patients have a 100-fold increased risk for the development of lymphoid malignancies. It is important to consider AT in a child with pre-existing ataxia, or lymphoid malignancy that was diagnosed at a younger age than expected. This consideration avoids the confusion between ataxia development and toxicity from chemotherapy. Hodgkin's lymphoma (HL) is usually treated with chemotherapy and/or radiotherapy. Unfortunately, when treated with conventional doses of radiotherapy, AT patients invariably experience devastating necrosis of their normal tissues. Therefore, a new treatment protocol for patients with HL in AT must be established. In this paper, we report the case of an 8-year-old female patient with HL in AT who was treated with chemotherapy. This patient was also treated with brentuximab (which targets CD30) for salvage therapy after the disease progressed.


Subject(s)
Ataxia , Ataxia Telangiectasia , Cerebellar Ataxia , Child , Clinical Protocols , Drug Therapy , Female , Hodgkin Disease , Humans , Necrosis , Radiation Tolerance , Radiotherapy , Salvage Therapy , Telangiectasis
19.
Yonsei Medical Journal ; : 572-579, 2016.
Article in English | WPRIM | ID: wpr-52544

ABSTRACT

PURPOSE: Unique features of adolescent cancer patients include cancer types, developmental stages, and psychosocial issues. In this study, we evaluated the relationship between diagnostic delay and survival to improve adolescent cancer care. MATERIALS AND METHODS: A total of 592 patients aged 0-18 years with eight common cancers were grouped according to age (adolescents, ≥10 years; children, <10 years). We retrospectively reviewed their symptom intervals (SIs, between first symptom/sign of disease and diagnosis), patient delay (PD, between first symptom/sign of disease and first contact with a physician), patient delay proportion (PDP), and overall survival (OS). RESULTS: Mean SI was significantly longer in adolescents than in children (66.4 days vs. 28.4 days; p<0.001), and OS rates were higher in patients with longer SIs (p=0.001). In children with long SIs, OS did not differ according to PDP (p=0.753). In adolescents with long SIs, OS was worse when PDP was ≥0.6 (67.2%) than <0.6 (95.5%, p=0.007). In a multivariate analysis, adolescents in the long SI/PDP ≥0.6 group tended to have a higher hazard ratio (HR, 6.483; p=0.069) than those in the long SI/PDP <0.6 group (HR=1, reference). CONCLUSION: Adolescents with a long SI/PDP ≥0.6 had lower survival rates than those with a short SI/all PDP or a long SI/PDP <0.6. They should be encouraged to seek prompt medical assistance by a physician or oncologist to lessen PDs.


Subject(s)
Adolescent , Child , Delayed Diagnosis , Female , Humans , Male , Multivariate Analysis , Neoplasms/classification , Patient Acceptance of Health Care/statistics & numerical data , Retrospective Studies , Survival Analysis , Survival Rate , Time Factors
20.
Yonsei Medical Journal ; : 915-922, 2016.
Article in English | WPRIM | ID: wpr-63332

ABSTRACT

PURPOSE: In childhood cancer survivors, the most common late effect is thyroid dysfunction, most notably subclinical hypothyroidism (SCH). Our study evaluated the risk factors for persistent SCH in survivors. MATERIALS AND METHODS: Survivors (n=423) were defined as patients who survived at least 2 years after cancer treatment completion. Thyroid function was assessed at this time and several years thereafter. Two groups of survivors with SCH were compared: those who regained normal thyroid function during the follow-up period (normalized group) and those who did not (persistent group). RESULTS: Overall, 104 of the 423 survivors had SCH. SCH was observed in 26% of brain or nasopharyngeal cancer survivors (11 of 43) and 21.6% of leukemia survivors (35 of 162). Sixty-two survivors regained normal thyroid function, 30 remained as persistent SCH, and 12 were lost to follow-up. The follow-up duration was 4.03 (2.15-5.78) years. Brain or nasopharyngeal cancer and Hodgkin disease were more common in the persistent group than in the normalized group (p=0.002). More patients in the persistent group received radiation (p=0.008). Radiation to the head region was higher in this group (2394±2469 cGy) than in the normalized group (894±1591 cGy; p=0.003). On multivariable analysis, lymphoma (p=0.011), brain or nasopharyngeal cancer (p=0.039), and head radiation dose ≥1800 cGy (p=0.039) were significant risk factors for persistent SCH. CONCLUSION: SCH was common in childhood cancer survivors. Brain or nasopharyngeal cancer, lymphoma, and head radiation ≥1800 cGy were significant risk factors for persistent SCH.


Subject(s)
Age Factors , Child , Child, Preschool , Female , Humans , Hypothyroidism/diagnosis , Infant , Male , Neoplasms/complications , Retrospective Studies , Risk Factors , Survivors
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