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1.
Article in English | WPRIM | ID: wpr-897872

ABSTRACT

Synovial sarcoma occurring in the pleura and lung is extremely rare. We report a case of pleuropulmonary synovial sarcoma as a second malignant neoplasm. The patient had been diagnosed with acute myelomonocytic leukemia at 5 years of age, and received matched sibling donor allogeneic bone marrow transplantation, with total body irradiation and cyclophosphamide conditioning. At 22 years of age, he complained of worsening chest discomfort and exertional dyspnea. Chest CT revealed a huge mass in the right middle lobe, pleura, and diaphragm. The patient was initially diagnosed as sarcomatoid malignant mesothelioma, without any environmental or occupational asbestos exposure. Five months later, the patient presented with soft tissue metastasis and underwent needle biopsy. Pathological examination including SYT-SSX RT-PCR revealed synovial sarcoma, which led to a review of the original tumor findings and confirmed the diagnosis of pleuropulmonary synovial sarcoma.To the best of our knowledge, our patient is the first case of pleuropulmonary synovial sarcoma developed after allogeneic hematopoietic stem cell transplantation.

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-897416

ABSTRACT

At the end of 2019, the cause of pneumonia outbreaks in Wuhan, China, was identified as severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection. In February 2020, the World Health Organization named the disease cause by SARS-CoV-2 as coronavirus disease 2019 (COVID-19). In response to the pandemic, the Korean Cancer Association formed the COVID-19 task force to develop practice guidelines. This special article introduces the clinical practice guidelines for cancer patients which will help oncologists best manage cancer patients during the COVID-19 pandemic.

4.
Blood Research ; : S34-S38, 2021.
Article in English | WPRIM | ID: wpr-897353

ABSTRACT

Myelodysplastic syndrome (MDS) refers to a heterogeneous group of clonal blood disorders characterized by ineffective hematopoiesis, cytopenia, dysplasia, and an increased risk of acute myeloid leukemia (AML). A growing number of inherited genetic loci that contribute to MDS/AML development are rapidly being identified. As genetic sequencing has become increasingly integrated into clinical practice, clearly defined syndromes have emerged, known as the MDS/AML predisposition syndrome. With more patients and families being identified with predisposing conditions, knowledge of the approach of evaluating and managing MDS with genetic predisposition is increasingly essential. This article reviews MDS with genetic predisposition and the practical aspects of management in patients with predisposition syndrome.

5.
Article in English | WPRIM | ID: wpr-890168

ABSTRACT

Synovial sarcoma occurring in the pleura and lung is extremely rare. We report a case of pleuropulmonary synovial sarcoma as a second malignant neoplasm. The patient had been diagnosed with acute myelomonocytic leukemia at 5 years of age, and received matched sibling donor allogeneic bone marrow transplantation, with total body irradiation and cyclophosphamide conditioning. At 22 years of age, he complained of worsening chest discomfort and exertional dyspnea. Chest CT revealed a huge mass in the right middle lobe, pleura, and diaphragm. The patient was initially diagnosed as sarcomatoid malignant mesothelioma, without any environmental or occupational asbestos exposure. Five months later, the patient presented with soft tissue metastasis and underwent needle biopsy. Pathological examination including SYT-SSX RT-PCR revealed synovial sarcoma, which led to a review of the original tumor findings and confirmed the diagnosis of pleuropulmonary synovial sarcoma.To the best of our knowledge, our patient is the first case of pleuropulmonary synovial sarcoma developed after allogeneic hematopoietic stem cell transplantation.

6.
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.

7.
Article in English | WPRIM | ID: wpr-889712

ABSTRACT

At the end of 2019, the cause of pneumonia outbreaks in Wuhan, China, was identified as severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection. In February 2020, the World Health Organization named the disease cause by SARS-CoV-2 as coronavirus disease 2019 (COVID-19). In response to the pandemic, the Korean Cancer Association formed the COVID-19 task force to develop practice guidelines. This special article introduces the clinical practice guidelines for cancer patients which will help oncologists best manage cancer patients during the COVID-19 pandemic.

8.
Blood Research ; : S34-S38, 2021.
Article in English | WPRIM | ID: wpr-889649

ABSTRACT

Myelodysplastic syndrome (MDS) refers to a heterogeneous group of clonal blood disorders characterized by ineffective hematopoiesis, cytopenia, dysplasia, and an increased risk of acute myeloid leukemia (AML). A growing number of inherited genetic loci that contribute to MDS/AML development are rapidly being identified. As genetic sequencing has become increasingly integrated into clinical practice, clearly defined syndromes have emerged, known as the MDS/AML predisposition syndrome. With more patients and families being identified with predisposing conditions, knowledge of the approach of evaluating and managing MDS with genetic predisposition is increasingly essential. This article reviews MDS with genetic predisposition and the practical aspects of management in patients with predisposition syndrome.

9.
Article in English | WPRIM | ID: wpr-874357

ABSTRACT

Fibrolamellar hepatocellular carcinoma (FLHCC) is a rare liver cancer affecting adolescents and young adults without any pre existing liver disease. Hyperammonemic encephalopathy (HAE) is a serious paraneoplastic syndrome, and several cases of HAE have been reported in patients with FLHCC. This condition is rare; hence, there are currently no management guidelines for cancer-related HAE. Herein, we report a case of an 18-year-old man with advanced FLHCC who developed HAE during the first course of chemotherapy consisting of cisplatin, doxorubicin, 5-fluorouracil, and interferon-α. He was successfully treated with continuous venovenous hemofiltration, sodium benzoate, sodium phenylbutyrate, and amino acid supplementation for HAE. After the second course of chemotherapy, he underwent surgery, and thereafter, his ammonia levels were normal without any ammonia scavenger therapy. Treatments for HAE described here will be helpful for this rare, but serious metabolic complication of FLHCC and could partially applied to HAE related to any malignancies.

10.
Article in English | WPRIM | ID: wpr-874346

ABSTRACT

Purpose@#This study investigated the incidence and relative survival rates (RSRs) for cancers among adolescents and young adults (AYAs) aged 15-39 years between 1993 and 2016 in Korea @*Materials and Methods@#Data from the Korea Central Cancer Registry were used to calculate percent distributions, age-specific incidence rates, age-standardized incidence rates (ASRs) per million, annual percent changes (APCs), average APCs, and RSRs for cancers diagnosed in AYAs. @*Results@#ASR of all cancers among AYAs was 654.5 per million. The largest diagnosed group of cancers was carcinomas (almost 80%). Crude incidence increased with age, from 170.4 per million for those aged 15-19 years to 1,639.8 per million for those aged 35-39 years. ASR increased from 414.8 per million to 820.4 per million, with an APC of 9.0%. The incidence of thyroid carcinoma showed the most rapid increment (APC, 14.0%), followed by non-Hodgkin lymphoma (APC, 13.4%). The 5-year RSR among AYAs significantly improved from 62.1% to 90.8%. Survival improvement in AYAs was higher than that in children but lower than that in older adults (APC, 2.1% vs. 1.9% vs. 3.1%). The most marked survival improvement was found for leukemia and lymphoma. Astrocytoma, rhabdomyosarcoma, and carcinoma of the trachea, bronchus, and lung had a 5-year RSR of < 50%. @*Conclusion@#There was an improvement in cancer survivals in AYAs, comparable to that achieved in children. However, survivals in several cancer types do not appear to be improving. Further research focusing on the epidemiology and therapeutic strategies for cancers in AYAs is needed.

11.
Article | WPRIM | ID: wpr-831549

ABSTRACT

Background@#Hereditary hemolytic anemia (HHA) is a rare disease characterized by premature red blood cell (RBC) destruction due to intrinsic RBC defects. The RBC Disorder Working Party of the Korean Society of Hematology established and updated the standard operating procedure for making an accurate diagnosis of HHA since 2007. The aim of this study was to investigate a nationwide epidemiology of Korean HHA. @*Methods@#We collected the data of a newly diagnosed pediatric HHA cohort (2007–2016) and compared this cohort's characteristics with those of a previously surveyed pediatric HHA cohort (1997–2006) in Korea. Each participant's information was retrospectively collected by a questionnaire survey. @*Results@#A total of 369 children with HHA from 38 hospitals distributed in 16 of 17 districts of Korea were investigated. RBC membranopathies, hemoglobinopathies, RBC enzymopathies, and unknown etiologies accounted for 263 (71.3%), 59 (16.0%), 23 (6.2%), and 24 (6.5%) of the cases, respectively. Compared to the cohort from the previous decade, the proportions of hemoglobinopathies and RBC enzymopathies significantly increased (P < 0.001 and P = 0.008, respectively). Twenty-three of the 59 hemoglobinopathy patients had immigrant mothers, mostly from South-East Asia. @*Conclusion@#In Korea, thalassemia traits have increased over the past 10 years, reflecting both increased awareness of this disease and increased international marriages. The enhanced recognition of RBC enzymopathies is due to advances in diagnostic technique; however, 6.5% of HHA patients still do not have a clear diagnosis. It is necessary to improve accessibility of diagnosing HHA.

12.
Article in English | WPRIM | ID: wpr-719418

ABSTRACT

PURPOSE: Dexrazoxane has been used as an effective cardioprotector against anthracycline cardiotoxicity. This study intended to analyze cardioprotective efficacy and secondary malignancy development, and elucidate risk factors for secondary malignancies in dexrazoxane-treated pediatric patients. MATERIALS AND METHODS: Data was collected from 15 hospitals in Korea. Patients who received any anthracyclines, and completed treatment without stem cell transplantation were included. For efficacy evaluation, the incidence of cardiac events and cardiac event-free survival rates were compared. Data about risk factors of secondary malignancies were collected. RESULTS: Data of total 1,453 cases were analyzed; dexrazoxane with every anthracyclines group (D group, 1,035 patients) and no dexrazoxane group (non-D group, 418 patients). Incidence of the reported cardiac events was not statistically different between two groups; however, the cardiac event-free survival rate of patients with more than 400 mg/m2 of anthracyclines was significantly higher in D group (91.2% vs. 80.1%, p=0.04). The 6-year cumulative incidence of secondary malignancy was not different between both groups after considering follow-up duration difference (non-D, 0.52%±0.37%; D, 0.60%±0.28%; p=0.55). The most influential risk factor for secondary malignancy was the duration of anthracycline administration according to multivariate analysis. CONCLUSION: Dexrazoxane had an efficacy in lowering cardiac event-free survival rates in patients with higher cumulative anthracyclines. As a result of multivariate analysis for assessing risk factors of secondary malignancy, the occurrence of secondary malignancy was not related to dexrazoxane administration.


Subject(s)
Anthracyclines , Cardiotoxicity , Dexrazoxane , Disease-Free Survival , Follow-Up Studies , Humans , Incidence , Korea , Multivariate Analysis , Neoplasms, Second Primary , Risk Factors , Stem Cell Transplantation
13.
Article in English | WPRIM | ID: wpr-763509

ABSTRACT

Vascular anomalies comprise a heterogeneous group of disorders characterized by abnormal growth or development of blood vessels. Diagnosis of vascular anomalies is often challenging due to the large variety of conditions, which exhibit phenotypic heterogeneity and a wide range of symptomology and severity. Accurate diagnosis is crucial for appropriate evaluation and management, often requiring multidisciplinary specialists. Recent interdisciplinary collaboration has led to collaborative studies and their outcomes are being prospectively evaluated. While there is still a role for surgical intervention in various vascular anomalies, discoveries of pharmacologic agents effective in treating vascular anomalies have broadened our medical therapeutic options. This paper focuses on vascular anomaly issues often seen by the pediatricians and reviews the clinical pearls on infantile hemangiomas, lymphatic malformations, venous malformations, and arteriovenous malformations.


Subject(s)
Arteriovenous Malformations , Blood Vessels , Cooperative Behavior , Diagnosis , Hemangioma , Population Characteristics , Prospective Studies , Specialization
14.
Article in English | WPRIM | ID: wpr-760893

ABSTRACT

Bacillus cereus causes serious central nervous system infections, especially in immunocompromised patients. Successful treatment requires adequate antimicrobial concentrations in the cerebrospinal fluid; however, in some cases, achieving this with systemic treatment alone is difficult. We treated intractable B. cereus ventriculitis with intraventricular vancomycin, with no major adverse events.


Subject(s)
Bacillus cereus , Bacillus , Central Nervous System Infections , Cerebral Ventriculitis , Cerebrospinal Fluid , Immunocompromised Host , Injections, Intraventricular , Pharmacokinetics , Vancomycin
15.
Article in Korean | WPRIM | ID: wpr-125603

ABSTRACT

Infantile hemangioma (IH) is the most common type of benign tumor during infancy. Due to their propensity to spontaneously involute, most IHs can be left untreated. However, IHs located in particular anatomic areas and those with complications need rapid and active treatment. It is often challenging for clinicians to predict which IHs are in need of systemic treatment. Data from various studies have revealed further insights into IH. The treatment options include propranolol, steroids, interferon-α, vincristine, surgical excision, and laser therapy. More infants are now treated with propranolol than were previously treated with oral corticosteroids, and the full implications of this shift in practice are not yet clear. This paper summarizes the pathogenesis, clinical course and current recommendations for management of IHs.


Subject(s)
Adrenal Cortex Hormones , Hemangioma , Humans , Infant , Laser Therapy , Propranolol , Steroids , Vincristine
16.
Article in English | WPRIM | ID: wpr-143633

ABSTRACT

This retrospective study investigated the clinical characteristics and outcomes of second malignant neoplasms (SMNs) in survivors of childhood cancer from multiple institutions in Korea. A total of 102 patients from 11 institutions who developed SMN after childhood cancer treatment between 1998 and 2011 were retrospectively enrolled. The most common primary malignant neoplasms (PMNs) were central nervous system (CNS) tumors (n = 17), followed by acute lymphoblastic leukemia (n = 16), non-Hodgkin lymphoma (n = 13), and osteosarcoma (n = 12). The most common SMNs were therapy-related myeloid neoplasms (t-MNs; acute myeloid leukemia [AML], 29 cases; myelodysplastic syndrome [MDS], 12 cases), followed by thyroid carcinomas (n = 15) and CNS tumors (n = 10). The median latency period was 4.9 years (range, 0.5-18.5 years). Among 45 patients with solid tumors defined as an SMN, 15 (33%) developed the lesion in a field previously subjected to radiation. The 5-year overall survival (OS) rate of patients with an SMN was 45% with a median follow-up time of 8.6 years. Patients with AML, MDS, and CNS tumors exhibited the poorest outcomes with 5-year OS rates of 18%, 33%, and 32%, respectively, whereas those with second osteosarcoma showed comparable outcomes (64%) to patients with primary counterpart and those with second thyroid carcinoma had a 100% OS rate. Further therapeutic efforts are recommended to improve the survival outcomes in patients with SMNs, especially in cases with t-MNs and CNS tumors.


Subject(s)
Adolescent , Antineoplastic Agents/therapeutic use , Central Nervous System Neoplasms/diagnosis , Child , Child, Preschool , Disease-Free Survival , Hospitals , Humans , Infant , Leukemia, Myeloid, Acute/diagnosis , Myelodysplastic Syndromes/diagnosis , Neoplasms, Second Primary/diagnosis , Osteosarcoma/diagnosis , Retrospective Studies , Stem Cell Transplantation , Survival Rate , Transplantation, Autologous , Young Adult
17.
Article in English | WPRIM | ID: wpr-143624

ABSTRACT

This retrospective study investigated the clinical characteristics and outcomes of second malignant neoplasms (SMNs) in survivors of childhood cancer from multiple institutions in Korea. A total of 102 patients from 11 institutions who developed SMN after childhood cancer treatment between 1998 and 2011 were retrospectively enrolled. The most common primary malignant neoplasms (PMNs) were central nervous system (CNS) tumors (n = 17), followed by acute lymphoblastic leukemia (n = 16), non-Hodgkin lymphoma (n = 13), and osteosarcoma (n = 12). The most common SMNs were therapy-related myeloid neoplasms (t-MNs; acute myeloid leukemia [AML], 29 cases; myelodysplastic syndrome [MDS], 12 cases), followed by thyroid carcinomas (n = 15) and CNS tumors (n = 10). The median latency period was 4.9 years (range, 0.5-18.5 years). Among 45 patients with solid tumors defined as an SMN, 15 (33%) developed the lesion in a field previously subjected to radiation. The 5-year overall survival (OS) rate of patients with an SMN was 45% with a median follow-up time of 8.6 years. Patients with AML, MDS, and CNS tumors exhibited the poorest outcomes with 5-year OS rates of 18%, 33%, and 32%, respectively, whereas those with second osteosarcoma showed comparable outcomes (64%) to patients with primary counterpart and those with second thyroid carcinoma had a 100% OS rate. Further therapeutic efforts are recommended to improve the survival outcomes in patients with SMNs, especially in cases with t-MNs and CNS tumors.


Subject(s)
Adolescent , Antineoplastic Agents/therapeutic use , Central Nervous System Neoplasms/diagnosis , Child , Child, Preschool , Disease-Free Survival , Hospitals , Humans , Infant , Leukemia, Myeloid, Acute/diagnosis , Myelodysplastic Syndromes/diagnosis , Neoplasms, Second Primary/diagnosis , Osteosarcoma/diagnosis , Retrospective Studies , Stem Cell Transplantation , Survival Rate , Transplantation, Autologous , Young Adult
18.
Article in English | WPRIM | ID: wpr-13394

ABSTRACT

PURPOSE: This study aimed to evaluate the clinical course of childhood immune thrombocytopenia (ITP) and to assess the risk factors for developing chronic ITP. METHODS: The records of 64 children diagnosed with ITP from November 2005 and December 2014 at single center were retrospectively analyzed. RESULTS: The median age at diagnosis and the median platelet count were 1 year (range, 1 month to 15 years) and 9×10⁹/L (range, 0–84×10⁹/L), respectively. No patient experienced severe bleeding. Nineteen children (29.7%) spontaneously recovered their platelet count to ≥100×10⁹/L at a median of 10 days. In total 45 patients (70.3%) received intravenous immunoglobulin (IVIG) as first-line therapy, and showed platelet recovery at 1 week. The final diagnosis of 55 (85.9%) and 9 patients (14.1%) was acute and chronic ITP, respectively. Older age, absence of prior infection and insidious onset of symptoms were significantly associated with the development of chronic ITP. Among the patients who received IVIG, those with platelet count <45×10⁹/L at 1 month after IVIG showed a significantly higher incidence of chronic ITP compared to those with platelet count ≥45×10⁹/L (88.8% vs. 44.4%, P<0.01). CONCLUSION: In most patients, ITP runs a benign course and approximately 86% of them recover within 1 year of their initial diagnosis. The potential impact of the risk factors of chronic ITP on clinical practice needs to be explored and further studies are warranted to determine whether IVIG influences the course of ITP.


Subject(s)
Blood Platelets , Child , Diagnosis , Hemorrhage , Humans , Immunoglobulins , Immunoglobulins, Intravenous , Incidence , Platelet Count , Purpura, Thrombocytopenic, Idiopathic , Retrospective Studies , Risk Factors
19.
Article in Korean | WPRIM | ID: wpr-788538

ABSTRACT

Hepatoblastoma, the most common primary liver tumor of early childhood, is a rare neoplasm, accounting for 0.8-2.0% of all pediatric cancers. The etiology is unknown. The most common method of testing for hepatoblastoma is alpha-fetoprotein (AFP). AFP is used as a biomarker for presence of residual liver tumor, therefore indicator of the successful treatment. Multimodal therapy is composed of chemotherapy and surgical intervention. In general, the cure of hepatoblastoma in children depends on complete resection of the primary tumor. While > or =90% of patients who undergone primary complete resection of their tumors can be cured, patients with unresectable or metastatic tumors show survival rate of < or =65%. Liver transplantation (LT) is a feasible treatment option for carefully selected patients who are free of extrahepatic disease prior to LT. The still dismal outcome of multifocal disseminated tumors warrants the investigation of new cytotoxic drug and substances against specific molecular targets.


Subject(s)
alpha-Fetoproteins , Child , Drug Therapy , Hepatoblastoma , Humans , Liver , Liver Transplantation , Survival Rate
20.
Article in Korean | WPRIM | ID: wpr-13544

ABSTRACT

Hepatoblastoma, the most common primary liver tumor of early childhood, is a rare neoplasm, accounting for 0.8-2.0% of all pediatric cancers. The etiology is unknown. The most common method of testing for hepatoblastoma is alpha-fetoprotein (AFP). AFP is used as a biomarker for presence of residual liver tumor, therefore indicator of the successful treatment. Multimodal therapy is composed of chemotherapy and surgical intervention. In general, the cure of hepatoblastoma in children depends on complete resection of the primary tumor. While > or =90% of patients who undergone primary complete resection of their tumors can be cured, patients with unresectable or metastatic tumors show survival rate of < or =65%. Liver transplantation (LT) is a feasible treatment option for carefully selected patients who are free of extrahepatic disease prior to LT. The still dismal outcome of multifocal disseminated tumors warrants the investigation of new cytotoxic drug and substances against specific molecular targets.


Subject(s)
alpha-Fetoproteins , Child , Drug Therapy , Hepatoblastoma , Humans , Liver , Liver Transplantation , Survival Rate
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