• The Plant Pathology Journal
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• v.27 no.1
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• pp.20-25
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• 2011

Gibberella zeae (anamorph: Fusarium graminearum), a homothallic (self-ferile) ascomycete with ubiquitous geographic distribution, causes serious diseases in several cereal crops. Ascospores (sexual spores) produced by this fungal pathogen have been suggested as the main source of primary inoculum in disease development. Here, we report the function of a gene designated GzRUM1, which is essential for ascospore formation in G. zeae. The deduced product of GzRUM1 showed significant similarities to the human retinoblastoma (tumor suppressor) binding protein 2 and a transcriptional repressor, Rum1 in the corn smut fungus (Ustilago maydis). The transcript of GzRUM1 was detected during the both vegetative and sexual stages, but was more highly accumulated during the latter stage. In addition, no GzRUM1 transcript was detected in a G. zeae strain lacking a mating-type gene (MAT1-2), a master regulator for sexual development in G. zeae. Targeted deletion of GzRUM1 caused no dramatic changes in several traits except ascospore formation. The ${\Delta}$GzRUM1 strain produced perithecia (sexual fruit bodies) but not asci nor ascospores within them. This specific defect leading to an arrest in ascospore development suggests that GzRUM1, as Rum1 in U. maydis, functions as a transcriptional regulator during sexual reproduction in G. zeae.

• Asian Pacific Journal of Cancer Prevention
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• v.16 no.10
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• pp.4193-4198
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• 2015

Background: Globally, retinoblastoma is the most common primary intraocular malignancy occurring in children. This paper documents the recent incidence rates of retinoblastoma by age and sex groups from the Population Based Cancer Registries (PBCRs) of Bangalore, Mumbai, Chennai, Delhi and Kolkata using the data from the National Cancer Registry Programme. Materials and Methods: Relative proportions, sex ratio, method of diagnosis, and incidence rates (crude and age standardized) for each PBCR and pooled rates of the five PBCRs were calculated for the years 2005/06 to 2009/10. Standard errors and 95% confidence limits of ASIRs by sex group in each PBCR were calculated using the Poisson distribution. Standardised rate ratios of ASIR by sex group and rate ratios at risk were also calculated. Results: The maximum retinoblastoma cases were in the 0-4 age group, accounting for 78% (females) and 81% (males) of pooled cases from five PBCRs. The pooled crude incidence rate in the 0-14 age group was 3.5 and the pooled ASIR was 4.4 per million. The pooled ASIR in the 0-4, 5-9 and 10-14 age group were 9.6, 2.0 and 0.1 respectively. The M/F ratio in Chennai (1.9) and Bangalore PBCRs (2.0) was much higher than the other PBCRs. Among the PBCRs, the highest incidence rate in 0-4 age group was found in males in Chennai (21.7 per million), and females in Kolkata (18.9 per million). There was a distinct variation in incidence rates in the PBCRs in different geographic regions of India.

• Clinical and Experimental Pediatrics
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• v.51 no.1
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• pp.84-88
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• 2008

Purpose : Radiotherapy is effective in local treatment for retinoblastoma. However, asymmetric facial hypoplasia after radiation is a serious late effect. This study was performed to investigate the effects of enucleation and chemotherapy with or without radiotherapy in advanced intraocular and intraorbital retinoblastoma. Methods : Between 1985 October and 2006 December, the records of thirty five patients who were diagnosed as retinoblastoma at Yeungnam University Hospital were reviewed. Advanced intraocular and intraorbital retinoblastoma patients classified as Reese-Ellsworth group III, IV, and V and Grabowski- Abramson class II were selected for the study. Results : Eighteen patients were enrolled in this study. All patients were enucleated and had received chemotherapy. Nine patients received radiotherapy and nine patients didn't receive radiotherapy. Tumor cells were found on resection margin of optic nerve in five of nine patients who received radiotherapy, but none of nine who didn't receive radiotherapy. Chemotherapy included vincristine, adriamycin, cyclophosphamide, VM-26, cisplatin before 2001, and vincristine, etoposide, and carboplatin after 2001. There were no recurrences or metastases in nine patients who didn't receive radiotherapy. But two of nine patients who received radiotherapy had metastases to brain. However, all survivors who received radiotherapy had significant facial asymmetry. Conclusion : In advanced intraocular and intraorbital retinoblastoma without tumor cell on resection margin of optic nerve, enucleation and chemotherapy without local radiotherapy appears to be safe for long-term survival. However, in those with tumor cells on resection margin of optic nerve, enucleation and chemotherapy with local radiotherapy seems to be necessary to improve survival.

• Proceedings of the Korean Biophysical Society Conference
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• 2001.06a
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• pp.60-60
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• 2001

We have investigated the effect of environmental acidity on the induction of apoptosis caused by heat and carboplatin alone or combined. The degree of apoptosis after heating at 42.5$^{\circ}C$ for 1h in pH 6.6 medium was greater than that in pH 7.5 medium in WERI human retinobalstoma cells. When heated in the same pH medium, more apoptosis occurred in the WERI cells than in the Y79 human retinoblastoma cells.(omitted)

• Clinical and Experimental Pediatrics
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• v.56 no.6
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• pp.254-259
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• 2013

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 Children's 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.

• Asian Pacific Journal of Cancer Prevention
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• v.15 no.18
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• pp.7805-7809
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• 2014

Objective: To explore the clinical efficacy and prognostic factors of chemoreduction combined with topical treatment of advanced intraocular retinoblastoma (RB). Materials and Methods: A total of 22 eyes from 17 children with RB were selected for the study and treated with chemoreduction combined with topical cryotherapy, transpupillary thermotherapy (TTT) or episcleral plaque brachytherapy. Clinical and follow-up data were retrospectively analyzed. Results: All children received 2~6 courses of chemoreduction treatment, ($4.5{\pm}0.8$ courses on average); 17 eyes from 13 children were treated by chemoreduction combined with cryotherapy or TTT and 5 eyes from 4 children with chemoreduction combined with $^{125}I$ episcleral plaque brachytherapy. The eye retention rate was 81.8% (18/22), among which 38.9% (7/18) featured restored or maintained good vision. Postoperative follow-up period was 7 to 34 months, ($18.6{\pm}5.2$ months on average). The recurrence rate was 41.2% (7/17), among which 57.1% (4/7) were controlled by supplementing or appending cryotherapy or TTT treatment during the follow-up period. The tumor basal diameter and thickness were significantly reduced (P<0.05 or P<0.01) after treatment. All children demonstrated different degrees of hair loss, 70.6% (12/17) with different degrees of gastrointestinal reactions, 5.88% (1/17) with neutropenia and 11.8% (2/17) being seriously infected during the chemotherapeutic treatment. Univariate and logistic regression analysis showed that tumor basal diameter before treatment had a significant effect on the prognosis (P<0.01). Conclusions: Chemoreduction combined with topical therapy can effectively control RB in the short term, and tumor basal diameter before treatment is an independent risk factor for prognosis.

• The Korean Journal of Physiology and Pharmacology
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• v.23 no.6
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• pp.449-458
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• 2019

Retinoblastoma (Rb) is one of the most common eye malignancies occur in childhood. The crucial roles of non-coding RNAs, particularly long non-coding RNAs (lncRNAs) and microRNAs (miRNAs), have been widely reported in Rb progression. In the present study, we found the expression of lncRNA T-cell leukemia/lymphoma 6 (TCL6) was significantly downregulated in Rb tissues and cell lines. Knockdown of lncRNA TCL6 promoted cell proliferation while reduced cell apoptosis in Rb cells. Moreover, lncRNA TCL6 serves as a sponge for miR-21, a previously-reported oncogenic miRNA in Rb, by direct targeting to negatively regulated miR-21 expression, therefore modulating Rb proliferation through miR-21. TCL6 overexpression inhibited Rb cell proliferation while miR-21 overexpression exerted an opposing effect; the effect of TCL6 overexpression was partially attenuated by miR-21 overexpression. PTEN/PI3K/AKT signaling pathway was involved in lncRNA TCL6/miR-21 axis modulating Rb cell proliferation. Taken together, lncRNA TCL6 serves as a tumor suppressor by acting as a sponge for miR-21 to counteract miR-21-mediated PTEN repression.

• Radiation Oncology Journal
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• v.8 no.2
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• pp.169-176
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• 1990

Radiotherapy result was analyzed in 23 children with retinoblastoma treated in Seoul National University Hospital from 1980 to 1987. Three ($17\%$) had bilateral tumor at diagnosis. Among 20 children with unilateral retinoblastoma 13 children got radiotherapy after enucleation, 2 were treated with radiotherapy alone, and 5 were delivered with radiotherapy after relapse. Of 15 non-recurrent unilateral tumors, there were 5 stage II children, 8 stage III, and 2 stage IV by staging system proposed by St. Jude Children's Research Hospital. Chemotherapy was combined when resection margin of the optic nerve was positive or when malignant cell was found in CSF. Of 12 children who completed radiotherapy, local or distant failure was not found but 2 cases of relapse at the contralateral retina were observed. Their 5 year survival rate was $82.2\%$. Another case of contralateral relapse was detected in children who was treated with radiotherapy alone. Thus overall frequency of the bilateral disease was $33\%$. Prognosis of recurrent tumors were so poor that no cases of CR was obtained and that 3 year survival rate was $20\%$. Two of 3 bilateral cases at diagnosis were in NED status. Complication were sunken orbit only. Result of radiotherapy was so good in early stage or small bulk tumor that treatment delay after diagnosis must not be allowed.

• Archives of Plastic Surgery
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• v.41 no.6
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• pp.785-787
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• 2014

• Asian Pacific Journal of Cancer Prevention
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• v.17 no.1
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• pp.81-83
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• 2016

Background: Cancer is a subject of continuing concern, more common in adults than in children, but often with a poor outcome in the latter. Our study set itself the objective to describe the epidemiological and histological aspects of solid cancers in children in Togo. Materials and Methods: This descriptive, cross-sectional study focused on cases of solid cancers in children diagnosed from 2010 to 2014 (5 years) at the pathology laboratory of the Tokoin teaching hospital. Data were collected from the records of that laboratory. Results: We collected 66 cases of childhood cancer representing 5% of all solid cancers. The annual incidence was 13.2 cases. The sex ratio (M/F) was 1.4; mean age was of $7.2{\pm}1.6years$. The age group most affected was that of 5-9 years (40.9%). Four histological groups of solid childhood cancers were listed: lymphoma (n=34 cases; 51.5%), embryonic cancer (n=17 cases; 25.8%), sarcomas (n=13 cases; 19.7%) and carcinoma (n=2 cases; 3%). The most common histological types were Burkitt lymphoma (36.4%), nephroblastoma (10.6%) and retinoblastoma (10.6%). Conclusions: This study shows that solid cancers in children are relatively frequent in Togo with a male predominance. They are still largely dominated by Burkitt lymphoma, followed by retinoblastoma and nephroblastoma.