Jung Woo Han

Posterior reversible encephalopathy syndrome in childhood with hematologic/oncologic diseases.

Posterior reversible encephalopathy syndrome (PRES) is a clinico-neuroradiologic disease entity represented by characteristic magnetic resonance image (MRI) findings of subcortical/cortical hyperintensity in T2-weighted sequences, more often observed in parieto-occipital lobes, accompanied by clinical neurologic alterations. PRES is a rare central nervous system complication in childhood hematologic-oncologic patients and shows very different neurologic symptoms between patients, from numbness on extremities to generalized seizure. The etiology of PRES was not well known until these days. In this study, 8 patients with PRES were reviewed, retrospectively. There were 4 patients with acute lymphocytic leukemia, 1 with aplastic anemia, and 3 with solid tumors (1 patient each for neuroblastoma, Ewing sarcoma, and osteosarcoma). Allogeneic stem cell transplantation was performed in 2 patients. Immunosuppressive agents such as tacrolimus and cyclosporine A were used in 3 patients. One neuroblastoma patient was in immediate postoperative status. All patients experienced seizure attacks of different types and showed typical MRI findings. Follow-up MRIs revealed significant improvements. From this review, we might consider chemotherapy and surgery as additive causes for PRES other than immunosuppressive agents. Therefore, careful examination of the patients receiving chemotherapy and surgery was needed to find out this uncommon but good prognostic complication.

Impact of psychological and cancer-related factors on HRQoL for Korean childhood cancer survivors

AbstractPurposeThe purposes of this study were (1) to compare health-related quality of life (HRQoL) between Korean childhood cancer survivors and healthy controls and (2) to examine the impact of demographic, diagnosis/treatment, and psychological variables on physical and psychosocial health in survivors.MethodsThe HRQoL (PedsQL), Self-Concept Inventory, and Child Behavior Checklist were administered to childhood cancer survivors, age/gender-matched healthy counterparts, and their parents. Independent-samples t tests and hierarchical multiple regression analyses were conducted.ResultsCompared with healthy controls, childhood cancer survivors reported significantly lower scores across physical and psychosocial HRQoL. For survivors, demographic, diagnosis/treatment, current health status, and psychological variables explained more than 50xa0% of the variance in both subscales of HRQoL. Especially, self-concept, a psychological variable, explained a significant portion of the variance in physical and psychosocial HRQoL after controlling for cancer-related factors. Several cancer-related factors including time since treatment completion, having a history of allogeneic hematopoietic stem cell transplantation, multiple treatment modalities, and suffering from severe late effects also associated with specific dimension of HRQoL.ConclusionsChildhood cancer survivors do experience lower level of QoL and psychological factors, especially self-concept, should be considered when supporting the well-being of childhood cancer survivors.n

Combined chemotherapy and intra-arterial chemotherapy of retinoblastoma

Purpose Retinoblastoma (RB) is the most common primary malignant intraocular tumor in children. Although systemic chemotherapy has been the primary treatment, intra-arterial chemotherapy (IAC) represents a new treatment option. Here, we performed alternate systemic chemotherapy and IAC and retrospectively reviewed the efficacy and safety of this approach. Methods Patients diagnosed with intraocular RB between January 2000 and December 2011 at Severance Childrens Hospital, Yonsei University, were reviewed. Before February 2010, the primary treatment for RB was chemotherapy (non-IAC/CTX). Since February 2010, the primary treatment for RB has been IAC (IAC/CTX). External beam radiotherapy or high-dose chemotherapy were used as last resort treatments just prior to enucleation at the time of progression or recurrence during primary treatment. Enucleation-free survival (EFS) and progression-free survival were assessed. Results We examined 19 patients (median age, 11.9 months; range, 1.4 to 75.6 months) with a sum of 25 eyes, of which, 60.0% were at advanced Reese Ellsworth (RE) stages. The enucleation rate was 33.3% at early RE stages and 81.8% at advanced RE stages (P=0.028). At 36 months, EFS was significantly higher in the IAC/CTX group than in the non-IAC/CTX group (100% vs. 40.0%, P=0.016). All 5 patients treated with IAC achieved eye preservation, although most patients were at advanced RE stages (IV-V). Conclusion Despite the limitation of a small sample size, our work shows that an alternative combined approach using IAC and CTX may be safe and effective for eye preservation in advanced RB.

Increasing and Worsening Late Effects in Childhood Cancer Survivors during Follow-up

Recent advances in childhood cancer treatment have increased survival rates to 80%. Two out of three survivors experience late effects (LEs). From a group of 241 survivors previously described, 193 were followed at the long-term follow-up clinic (LTFC) of Severance Hospital in Korea; the presence of LEs was confirmed by oncologists. We reported the change in LEs during 3 yr of follow-up. The median follow-up from diagnosis was 10.4 yr (5.1-26.2 yr). Among 193 survivors, the percentage of patients with at least one LE increased from 63.2% at the initial visit to 75.1% at the most recent visit (P = 0.011). The proportion of patients having multiple LEs and grade 2 or higher LEs increased from the initial visit (P = 0.001 respectively). Forty-eight non-responders to the LTFC were older and had less frequent and severe LEs than responders at initial visit (all P < 0.05). In multivariate analysis, younger age at diagnosis, older age at initial visit, a diagnosis of a brain tumor or lymphoma, and use of radiotherapy were significant risk factors for LEs (all P < 0.05). Adverse changes in LEs were seen among the survivors, regardless of most clinical risk factors. They need to receive comprehensive, long-term follow up.

Poor bone health at the end of puberty in childhood cancer survivors.

Although the survival rate following childhood cancer is >80%, late effects are a major concern. We aimed to determine the clinical factors affecting bone health after puberty in childhood cancer survivors at risk for low bone mineral density (BMD).

FEASIBILITY OF SEQUENTIAL HIGH-DOSE CHEMOTHERAPY IN ADVANCED PEDIATRIC SOLID TUMORS

The purpose of this study was to evaluate the feasibility and tumor response of 3 cycles of sequential high-dose chemotherapy (HDCT) in advanced pediatric solid tumor patients. Medical records of 11 children who underwent 2 consequent courses of reduced conditioning HDCT followed by final HDCT with autologous HSC infusion were reviewed in a retrospective manner. Each median time to an absolute neutrophil count > 0.5 × 109/L was 12, 13, and 12 days. Major toxic reactions were fever, infection, and vomiting. One patient experienced transplantation-related mortality. Nine patients showed complete and partial responses to the therapy at 6 months follow-up after final HDCT. Finally, 6 patients are alive without evidence of disease at median follow-up of 24 months. Even though it is a preliminary result, the authors think that this treatment could be a feasible treatment option for advanced pediatric solid tumor patients.

Symptom Interval and Patient Delay Affect Survival Outcomes in Adolescent Cancer Patients

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.

Subclinical Hypothyroidism in Childhood Cancer Survivors

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.

Characteristics and Outcomes of Second Malignant Neoplasms after Childhood Cancer Treatment: Multi-Center Retrospective Survey

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.

Huge retroperitoneal complicated pseudotumour in haemophilia B with inhibitor

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