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Purpose@#Renal tumors account for approximately 7% of all childhood cancers. These include Wilms tumor (WT), clear cell sarcoma of the kidney (CCSK), malignant rhabdoid tumor of the kidney (MRTK), renal cell carcinoma (RCC), congenital mesoblastic nephroma (CMN) and other rare tumors. We investigated the epidemiology of pediatric renal tumors in Korea. @*Materials and Methods@#From January 2001 to December 2015, data of pediatric patients (0–18 years) newly-diagnosed with renal tumors at 26 hospitals were retrospectively analyzed. @*Results@#Among 439 patients (male, 240), the most common tumor was WT (n=342, 77.9%), followed by RCC (n=36, 8.2%), CCSK (n=24, 5.5%), MRTK (n=16, 3.6%), CMN (n=12, 2.7%), and others (n=9, 2.1%). Median age at diagnosis was 27.1 months (range 0-225.5) and median follow-up duration was 88.5 months (range 0-211.6). Overall, 32 patients died, of whom 17, 11, 1, and 3 died of relapse, progressive disease, second malignant neoplasm, and treatment-related mortality. Five-year overall survival and event free survival were 97.2% and 84.8% in WT, 90.6% and 82.1% in RCC, 81.1% and 63.6% in CCSK, 60.3% and 56.2% in MRTK, and 100% and 91.7% in CMN, respectively (p < 0.001). @*Conclusion@#The pediatric renal tumor types in Korea are similar to those previously reported in other countries. WT accounted for a large proportion and survival was excellent. Non-Wilms renal tumors included a variety of tumors and showed inferior outcome, especially MRTK. Further efforts are necessary to optimize the treatment and analyze the genetic characteristics of pediatric renal tumors in Korea.
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Purpose@#The advances in the treatment of retinoblastoma have enabled salvaging the globe in advanced stages with intra-arterial chemotherapy (IAC). We developed a strategy of alternate application of systemic intravenous chemotherapy (IVC) and IAC (referred to as alternate systemic IVC and IAC; ASIAC) to reduce central nervous metastases during IAC and examined its efficacy and safety in eye globe salvage in this study. @*Materials and Methods@#Between January 2010 and February 2021, 43 eyes of 40 patients received ASIAC treatment for retinoblastoma at the Yonsei Cancer Center, Yonsei University Health System. Their medical records were reviewed retrospectively to evaluate the eye salvage rate (ESR), defined from diagnosis to enucleation. High-risk retinoblastoma was defined as group D or E by the International Classification of Retinoblastoma. @*Results@#The study enrolled 38 and five cases of high-risk and low-risk retinoblastoma, respectively. In total, 178 IAC and 410 IVC courses were administered, with a median of 4 (interquartile range [IQR], 3.0 to 5.0) IAC and 9 (IQR, 6.0 to 11) IVC courses per eye, respectively. The 5-year ESR was 60.4%±8.7% for the whole cohort, 100% for low-risk retinoblastoma, and 53.6%±9.8% for high-risk retinoblastoma. Among those diagnosed since 2015, the 5-year ESR for high-risk retinoblastoma was 63.5%±14.0%. Fifteen eyes underwent enucleation; no viable tumor was found in three enucleated eyes. There were no deaths in this cohort. @*Conclusion@#Primary IAC-IVC (i.e., ASIAC) for patients with retinoblastoma was tolerable and effective in salvaging the eye and maintaining survival.
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BACKGROUND: von Willebrand disease (VWD) is one of the most common inherited bleeding disorders. However, the number of patients who register to the Korea Hemophilia Foundation (KHF) is much lower than the expected prevalence rate and only few hospitals perform tests for diagnosis autonomously. Thus, we surveyed practical realities of VWD in Yeungnam region. METHODS: Patients with VWD (N=267) who were diagnosed at eleven university hospitals from March 1995 to March 2018 were enrolled in this study. We evaluated the medical records from each hospital retrospectively. RESULTS: Two hundred and twenty-eight children and 39 adults met the diagnostic criteria for VWD. Seventy-eight (57.4%) patients had the blood type O. Fifty-eight patients were definite type 1 (21.7%), 151 were possible type 1 (56.6%), and the others were type 2. Abnormal laboratory findings were the most common factor for the diagnosis in children. VWF mutations were detected in 17 patients. Patients with a family history showed age of diagnosis of 9 y, which is higher than in those with no family history (6 yr), and also showed a higher rate of significant bleeding (32.1% vs. 14.2%). VWF:RCo and VWF:Ag tests were performed in-hospital at only 1 of 11 hospitals. Twelve of 267 patients were enrolled at the KHF (4.5%). CONCLUSION: A high rate of out-sourcing studies may result in inaccurate diagnosis. The registration rate to the KHF is still lower than the prevalence rate. A comprehensive nationwide registration system is necessary in order to identify the actual prevalence rate and promote the diagnosis of VWD in Korea.
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Adulto , Criança , Humanos , Diagnóstico , Hemofilia A , Hemorragia , Hospitais Universitários , Coreia (Geográfico) , Prontuários Médicos , Prevalência , Estudos Retrospectivos , Doenças de von WillebrandRESUMO
OBJECTIVES: To investigate if vitamin D receptor (VDR) gene polymorphisms and circulating vitamin D levels are associated with pelvic floor disorders (PFDs). METHODS: In this case-control study, 25-hydroxy-vitamin D (25[OH]D) serum levels were analyzed in 47 females with PFDs and 87 healthy females (controls), respectively. The VDR gene polymorphisms were determined by using polymerase chain reaction and performing digestions with 4 restriction enzymes i.e., ApaI, TaqI, FokI, and BsmI. Vitamin D levels of patients were divided into 0.05). However, there was a significant difference in the distribution of vitamin D levels between study group and controls using Pearson's χ2 test (30 ng/mL: 87.2%, 12.8%, and 0% in the study group and 75.9%, 16.1%, and 8.0% in controls, respectively, P < 0.05). Taken together, our observations suggest that vitamin D levels could be associated with PFDs and that 2 polymorphisms (i.e., ApaI and BsmI) in the VDR gene may contribute to an increased prevalence of PFDs in women with insufficient levels of vitamin D. CONCLUSIONS: Examining vitamin D levels and performing a VDR genotype analysis may be helpful for assessing PFD risk.
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Feminino , Humanos , Estudos de Casos e Controles , Genótipo , Distúrbios do Assoalho Pélvico , Diafragma da Pelve , Reação em Cadeia da Polimerase , Prevalência , Receptores de Calcitriol , Deficiência de Vitamina D , Vitamina D , VitaminasRESUMO
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.
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Humanos , Diagnóstico , Diagnóstico Bucal , Transplante de Células-Tronco Hematopoéticas , Análise Multivariada , Odontogênese , Estudos Retrospectivos , Fatores de Risco , Sobreviventes , Dente , Anormalidades DentáriasRESUMO
PURPOSE: Pulmonary toxicities, including infectious pneumonia (IP) and idiopathic pneumonia syndrome (IPS), are serious side effects of total body irradiation (TBI) used for myeloablative conditioning. This study aimed to evaluate clinical factors associated with IP and IPS following TBI. MATERIALS AND METHODS: Fifty-eight patients with hematologic malignancies who underwent TBI before allogeneic hematopoietic stem cell transplantation between 2005 and 2014 were reviewed. Most patients (91%) received 12 Gy in 1.5 Gy fractions twice a day. Pulmonary toxicities were diagnosed based on either radiographic evidence or reduced pulmonary function, and were subdivided into IP and IPS based on the presence or absence of concurrent infection. RESULTS: Pulmonary toxicities developed in 36 patients (62%); 16 (28%) had IP and 20 (34%) had IPS. IP was significantly associated with increased treatment-related mortality (p = 0.028) and decreased survival (p = 0.039). Multivariate analysis revealed that the risk of developing IPS was significantly higher in patients who received stem cells from a matched unrelated donor than from a matched sibling donor (p = 0.021; hazard ratio [HR] = 12.67; 95% confidence interval [CI], 1.46–110.30). Combining other conditioning agents with cyclophosphamide produced a higher tendency to develop IP (p = 0.064; HR = 6.19; 95% CI, 0.90–42.56). CONCLUSION: IP and IPS involve different risk factors and distinct pathogeneses that should be considered when planning treatments before and after TBI.
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Humanos , Ciclofosfamida , Neoplasias Hematológicas , Transplante de Células-Tronco Hematopoéticas , Mortalidade , Análise Multivariada , Pneumonia , Fatores de Risco , Irmãos , Transplante de Células-Tronco , Células-Tronco , Doadores de Tecidos , Doadores não Relacionados , Irradiação Corporal TotalRESUMO
PURPOSE: Pulmonary toxicities, including infectious pneumonia (IP) and idiopathic pneumonia syndrome (IPS), are serious side effects of total body irradiation (TBI) used for myeloablative conditioning. This study aimed to evaluate clinical factors associated with IP and IPS following TBI. MATERIALS AND METHODS: Fifty-eight patients with hematologic malignancies who underwent TBI before allogeneic hematopoietic stem cell transplantation between 2005 and 2014 were reviewed. Most patients (91%) received 12 Gy in 1.5 Gy fractions twice a day. Pulmonary toxicities were diagnosed based on either radiographic evidence or reduced pulmonary function, and were subdivided into IP and IPS based on the presence or absence of concurrent infection. RESULTS: Pulmonary toxicities developed in 36 patients (62%); 16 (28%) had IP and 20 (34%) had IPS. IP was significantly associated with increased treatment-related mortality (p = 0.028) and decreased survival (p = 0.039). Multivariate analysis revealed that the risk of developing IPS was significantly higher in patients who received stem cells from a matched unrelated donor than from a matched sibling donor (p = 0.021; hazard ratio [HR] = 12.67; 95% confidence interval [CI], 1.46–110.30). Combining other conditioning agents with cyclophosphamide produced a higher tendency to develop IP (p = 0.064; HR = 6.19; 95% CI, 0.90–42.56). CONCLUSION: IP and IPS involve different risk factors and distinct pathogeneses that should be considered when planning treatments before and after TBI.
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Humanos , Ciclofosfamida , Neoplasias Hematológicas , Transplante de Células-Tronco Hematopoéticas , Mortalidade , Análise Multivariada , Pneumonia , Fatores de Risco , Irmãos , Transplante de Células-Tronco , Células-Tronco , Doadores de Tecidos , Doadores não Relacionados , Irradiação Corporal TotalRESUMO
OBJECTIVES: We investigated the utility of regular radiological follow-up on the early detection of contralateral breast cancer(CBC) and prognosis in patients with metachronous bilateral breast cancer. METHODS: Between 1983 and 2010, 49(2.1%) metachronous bilateral breast cancer patients were identified among a total of 2,343 cases of invasive or in situ breast carcinomas. We reviewed the patients' medical records including age, stage, duration between the first and second breast cancer diagnosis, operation method, recurrence, and breast cancer-specific survival. RESULTS: The mean ages at the first and second breast cancer diagnosis were 43.8 and 49.2 years, respectively. The mean duration between the first and second breast cancer diagnosis was 68.9 months (range, 7–266 months). Regular radiological follow-up with annual mammography(MMG) with or without ultrasonography was conducted in 28 patients (63.6%, Group 1), and no regular follow-up was performed in 12 patients (27.3%, Group 2). The median follow-up duration was 150 months. In a comparative analysis, Group 1 patients exhibited more stage 0 and stage 1 malignancies (82.1% vs. 25%, P =0.006) as second cancer and the same or an improved stage (71.4% vs. 33.3%, P =0.042) of second cancer compared to Group 2 patients. Breast cancer-specific survival rates between the two groups after the first cancer occurrence were higher in Group 1 patients compared to Group 2 patients, although this did not reach statistical significance. CONCLUSION: Screening for CBC with regular radiological follow-up could result in early detection of CBC, less invasive surgical procedures, and enhanced breast cancer-specific survival outcomes.
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Humanos , Neoplasias da Mama , Mama , Diagnóstico , Seguimentos , Programas de Rastreamento , Prontuários Médicos , Métodos , Segunda Neoplasia Primária , Prognóstico , Recidiva , Taxa de Sobrevida , UltrassonografiaRESUMO
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.
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Humanos , Coreia (Geográfico) , Análise Multivariada , Metástase Neoplásica , Neurilemoma , Neurofibromatoses , Neurofibromatose 1 , Patologia , Sarcoma , SeulRESUMO
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.
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Adolescente , Criança , Pré-Escolar , Humanos , Lactente , Adulto Jovem , Antineoplásicos/uso terapêutico , Neoplasias do Sistema Nervoso Central/diagnóstico , Intervalo Livre de Doença , Hospitais , Leucemia Mieloide Aguda/diagnóstico , Síndromes Mielodisplásicas/diagnóstico , Segunda Neoplasia Primária/diagnóstico , Osteossarcoma/diagnóstico , Estudos Retrospectivos , Transplante de Células-Tronco , Taxa de Sobrevida , Transplante AutólogoRESUMO
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.
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Adolescente , Criança , Pré-Escolar , Humanos , Lactente , Adulto Jovem , Antineoplásicos/uso terapêutico , Neoplasias do Sistema Nervoso Central/diagnóstico , Intervalo Livre de Doença , Hospitais , Leucemia Mieloide Aguda/diagnóstico , Síndromes Mielodisplásicas/diagnóstico , Segunda Neoplasia Primária/diagnóstico , Osteossarcoma/diagnóstico , Estudos Retrospectivos , Transplante de Células-Tronco , Taxa de Sobrevida , Transplante AutólogoRESUMO
No abstract available.
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Pré-Escolar , Feminino , Humanos , Sequência de Bases , Medula Óssea/patologia , Cromossomos Humanos Par 12 , Cromossomos Humanos Par 17 , Rearranjo Gênico , Cariótipo , Proteínas de Fusão Oncogênica/genética , Leucemia-Linfoma Linfoblástico de Células Precursoras/diagnóstico , Análise de Sequência de DNA , Fatores Associados à Proteína de Ligação a TATA/genética , Transativadores/genética , Translocação GenéticaRESUMO
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.
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Criança , Feminino , Humanos , Ataxia , Ataxia Telangiectasia , Ataxia Cerebelar , Protocolos Clínicos , Tratamento Farmacológico , Doença de Hodgkin , Necrose , Tolerância a Radiação , Radioterapia , Terapia de Salvação , TelangiectasiaRESUMO
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.
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Criança , Feminino , Humanos , Ataxia , Ataxia Telangiectasia , Ataxia Cerebelar , Protocolos Clínicos , Tratamento Farmacológico , Doença de Hodgkin , Necrose , Tolerância a Radiação , Radioterapia , Terapia de Salvação , TelangiectasiaRESUMO
Intravesical bacillus Calmette-Guérin (BCG) immunotherapy is a common treatment modality for bladder cancer after transurethral resection of a bladder tumor. This therapy is generally safe, and development of a prostatic abscess with a prostatorectal fistula after intravesical BCG immunotherapy is a very rare complication. This finding was incidentally obtained by the authors, who examined a patient with colonoscopy for evaluation of abdominal pain. The patient was successfully treated with antitubercular drugs. To the authors’ knowledge, this is the first report of a patient with a tuberculous prostatic abscess with prostatorectal fistula after BCG immunotherapy in South Korea.
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Humanos , Dor Abdominal , Abscesso , Antituberculosos , Bacillus , Colonoscopia , Fístula , Imunoterapia , Coreia (Geográfico) , Mycobacterium bovis , Próstata , Tuberculose , Neoplasias da Bexiga UrináriaRESUMO
A 50-year-old woman complained of jaundice and dyspepsia that started 2 weeks prior to consultation. Abdomen-pelvic computed tomography showed a 3 cm mass in the right hepatic duct with central calcification, which was spreading into the second branch. Repeated biopsies through endoscopic retrograde cholangiopancreatography were needed for pathology, which was consistent with an adenocarcinoma. Imaging studies including positron emission tomography showed no evidence of distant metastasis. The patient underwent right lobectomy with bile duct resection. The final diagnosis was intrahepatic cholangiocarcinoma with central calcification. We reported a very rare case of centrally calcified mass growing in the second branch of the right hepatic duct. The possibility of intrahepatic cholangiocarcinoma with central calcification should be considered for differential diagnosis of intrahepatic calcification.
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Feminino , Humanos , Pessoa de Meia-Idade , Adenocarcinoma , Ductos Biliares , Biópsia , Colangiocarcinoma , Colangiopancreatografia Retrógrada Endoscópica , Diagnóstico , Diagnóstico Diferencial , Dispepsia , Ducto Hepático Comum , Icterícia , Metástase Neoplásica , Patologia , Tomografia por Emissão de PósitronsRESUMO
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.
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Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Fatores Etários , Hipotireoidismo/diagnóstico , Neoplasias/complicações , Estudos Retrospectivos , Fatores de Risco , SobreviventesRESUMO
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.
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Adolescente , Criança , Feminino , Humanos , Masculino , Diagnóstico Tardio , Análise Multivariada , Neoplasias/classificação , Aceitação pelo Paciente de Cuidados de Saúde/estatística & dados numéricos , Estudos Retrospectivos , Análise de Sobrevida , Taxa de Sobrevida , Fatores de TempoRESUMO
BACKGROUND: Hypermethylation of tumor suppressor gene has been reported in various types of leukemia with potential involvement in the inactivation of regulatory cell cycle and apoptosis genes. METHODS: To evaluate the methylation status at initial diagnosis and morphologic complete remission (CR) period in childhood acute lymphoblastic leukemia (ALL), we analyzed the methylation status of three key genes (CDH1, p16 and DAPK) in 43 childhood ALL patients and 7 healthy bone marrow (BM) donors. RESULTS: CDH1 was methylated in 26 (60.4%) patients, p16 in two (4.6%) patients and DAPK in six (13.9%) patients at the time of diagnosis. Twenty nine (67.4%) patients had methylation of at least one gene. None of the healthy BM donors showed methylation of the above genes. Age was the only factor which showed significant association with the presence of DNA methylation (P=0.03). None of the other clinicopathological factors showed association with initial methylation status. At the time of morphologic CR, all patients who had aberrant DNA methylation at the time of diagnosis had no detectable residual methylation. CONCLUSION: Since hypermethylation was found in around two thirds of pretreatment ALL patients and none in healthy BM donor, we suggest hypermethylation of some important genes is a biologic marker of childhood ALL. We recommend that further studies with a large number of patients should be conducted.
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Humanos , Apoptose , Biomarcadores , Medula Óssea , Ciclo Celular , Diagnóstico , Metilação de DNA , Genes Supressores de Tumor , Leucemia , Metilação , Leucemia-Linfoma Linfoblástico de Células Precursoras , Doadores de TecidosRESUMO
BACKGROUND: Hypermethylation of tumor suppressor gene has been reported in various types of leukemia with potential involvement in the inactivation of regulatory cell cycle and apoptosis genes.METHODS: To evaluate the methylation status at initial diagnosis and morphologic complete remission (CR) period in childhood acute lymphoblastic leukemia (ALL), we analyzed the methylation status of three key genes (CDH1, p16 and DAPK) in 43 childhood ALL patients and 7 healthy bone marrow (BM) donors.RESULTS: CDH1 was methylated in 26 (60.4%) patients, p16 in two (4.6%) patients and DAPK in six (13.9%) patients at the time of diagnosis. Twenty nine (67.4%) patients had methylation of at least one gene. None of the healthy BM donors showed methylation of the above genes. Age was the only factor which showed significant association with the presence of DNA methylation (P=0.03). None of the other clinicopathological factors showed association with initial methylation status. At the time of morphologic CR, all patients who had aberrant DNA methylation at the time of diagnosis had no detectable residual methylation.CONCLUSION: Since hypermethylation was found in around two thirds of pretreatment ALL patients and none in healthy BM donor, we suggest hypermethylation of some important genes is a biologic marker of childhood ALL. We recommend that further studies with a large number of patients should be conducted.