Clinical features and long-term outcomes of bilateral Wilms tumor treated with Taiwan Pediatric Oncology Group protocols: A single center report

2016 ◽  
Vol 12 (3) ◽  
pp. 300-307 ◽  
Author(s):  
Shih-Hsiang Chen ◽  
Iou-Jih Hung ◽  
Chao-Ping Yang ◽  
Tang-Her Jaing ◽  
Chao-Jan Wang ◽  
...  
2015 ◽  
Vol 122 (2) ◽  
pp. 392-399 ◽  
Author(s):  
Xing-ju Liu ◽  
Dong Zhang ◽  
Shuo Wang ◽  
Yuan-li Zhao ◽  
Mario Teo ◽  
...  

OBJECT The aim of this study was to describe the baseline clinical features and long-term outcomes of patients with moyamoya disease (MMD) based on a 25-year period at a single center in China. METHODS  Data obtained in 528 consecutive patients with MMD treated at the authors' hospital from 1984 to 2010 were reviewed retrospectively. Events of transient ischemic attack, new infarction, and hemorrhage were included. The Kaplan-Meier risk of stroke was calculated. RESULTS  The mean (± SD) patient age was 26 ± 13 years (range 2–67 years), and the female/male ratio was 0.9:1. There were 332 cases of ischemia and 196 hemorrhages. Adults had a higher rate of bleeding than children (50.7% vs 14.0%, respectively; p < 0.001). One hundred twenty-two patients were treated conservatively, and 406 patients underwent revascularization procedures. Of 528 patients, 331 (62.7%) had at least 1 year of follow-up (median 39.5 months) and data from these patients were analyzed. Rebleeding and mortality rates in patients with hemorrhagic MMD (n = 104) were higher than in those with ischemic MMD (n = 227) (26.9% vs 2.2% [p < 0.001] and 4.8% vs 0.4% [p < 0.05], respectively). Twenty-five of 60 (41.7%) conservatively treated patients and 8 of 271 (2.9%) surgically treated patients experienced rebleeding events, a difference that was significant in the Kaplan-Meier curve of rebleeding (p < 0.01). An improvement in perfusion was found in 164 of 224 (73.2%) surgically treated patients 1 month after discharge. However, there was no significant difference in the rate of ischemic events in the surgical and conservative groups (18.8% and 28.3%, respectively; p = 0.09). Among the 104 hemorrhagic cases, rebleeding attacks were observed in 25 patients in the nonsurgical group (n = 60) and 3 patients in the surgical group (n = 44) (41.7% and 6.8%, respectively; OR 9.7 [95% CI 2.7–35.0]; p < 0.01). CONCLUSIONS  There was no difference in the sex distribution of Chinese patients with MMD. Patients with hemorrhagic MMD had a much higher rate of rebleeding and poorer prognosis than those with the ischemic type. Surgical revascularization procedures can improve cerebral perfusion and have a positive impact in preventing rebleeding in patients with hemorrhagic MMD.


2010 ◽  
Vol 27 (3) ◽  
pp. 161-178 ◽  
Author(s):  
Canan Akyüz ◽  
Bilgehan Yalçın ◽  
İnci Yıldız ◽  
Volkan Hazar ◽  
Asım Yörük ◽  
...  

2001 ◽  
Vol 19 (4) ◽  
pp. 1047-1055 ◽  
Author(s):  
Howard M. Katzenstein ◽  
Paul M. Kent ◽  
Wendy B. London ◽  
Susan L. Cohn

PURPOSE: To investigate whether the rate of neurologic recovery or the incidence of long-term sequelae differed for children with neuroblastoma (NB) initially treated with chemotherapy versus surgical decompression with laminectomy, we reviewed the Pediatric Oncology Group (POG) experience. PATIENTS AND METHODS: A retrospective review of children diagnosed with intraspinal NB registered on POG NB Biology Protocol 9047 was performed. Survival, neurologic outcome, and orthopedic sequelae were evaluated according to age of the patient at diagnosis, stage of disease, duration and severity of neurologic symptoms, and therapeutic intervention. RESULTS: Between May 1990 and January 1998, 83 children with intraspinal NB were entered onto the study. Five-year survival for this cohort of patients was 71% ± 9%. Forty-three (52%) of the patients had neurologic symptoms at diagnosis. After treatment, six of 15 severely affected patients, who presented with paralysis, completely recovered neurologic function. Two of five patients with moderate deficits, consisting of paresis and bowel/bladder dysfunction, completely recovered neurologic function. Seventeen of 22 assessable children, who had mild symptoms comprised of paresis alone, fully recovered. Seven of 24 assessable patients who had undergone laminectomy developed scoliosis, whereas spinal deformities were only detected in one of 49 assessable patients managed without laminectomy (P = .001). CONCLUSION: The frequency of complete neurologic recovery in children with intraspinal NB inversely correlated with the severity of the presenting neurologic deficits. The rate of neurologic recovery was similar for patients treated with chemotherapy compared to those managed with laminectomy. Fewer orthopedic sequelae were observed in the children managed with chemotherapy than were seen in children managed with laminectomy.


2017 ◽  
Vol 135 (3) ◽  
pp. 513-519 ◽  
Author(s):  
Liang Wu ◽  
Ning Yao ◽  
Jingyi Fang ◽  
Jun Yang ◽  
Yulun Xu

Blood ◽  
1993 ◽  
Vol 81 (8) ◽  
pp. 2110-2117 ◽  
Author(s):  
RO Bash ◽  
WM Crist ◽  
JJ Shuster ◽  
MP Link ◽  
M Amylon ◽  
...  

Abstract Alteration of the TAL1 locus is the most common nonrandom genetic defect in childhood T-cell acute lymphoblastic leukemia (T-ALL). To determine if rearrangements of the TAL1 proto-oncogene confer a distinct leukemic phenotype, we studied leukemic peripheral blood or bone marrow samples from 182 children with newly diagnosed T-ALL enrolled on Pediatric Oncology Group treatment protocols. Forty-eight (26%) of the samples had a local rearrangement of the TAL1 locus. Demographic and clinical features were compared for patient subgroups with and without TAL1 rearrangements. The only clinical correlates that were significantly associated with TAL1 gene rearrangements were higher white blood cell count (P = .017) and higher hemoglobin (P = .007) at diagnosis. Immunophenotypically, samples with altered TAL1 were more likely to be CD2+ (P = .001) and lack CD10 (cALLa) expression (P = .007) than those without the rearrangement. There was a trend toward improved event-free survival (EFS) in patients with TAL1 rearrangements (4-year EFS was 44% +/- 7% for patients without the rearrangements v 59% +/- 11% for those with rearrangements), but the difference was not significant (P = .34). The role of TAL1 in leukemogenesis has yet to be clearly defined, and the prognostic significance of TAL1 gene rearrangements in T-ALL deserves further study.


1989 ◽  
Vol 17 (5-6) ◽  
pp. 401-408 ◽  
Author(s):  
Jeffrey P. Krischer ◽  
C. Philip Steuber ◽  
Teresa J. Vietti ◽  
Steven J. Culbert ◽  
Abdelsalam H. Ragab ◽  
...  

2010 ◽  
Vol 6 ◽  
pp. S59-S60 ◽  
Author(s):  
Canan Akyüz ◽  
Bilgehan Yalçin ◽  
İnci Yildiz ◽  
Volkan Hazar ◽  
Asım YörÜk ◽  
...  

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