• 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.2
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• pp.899-904
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• 2014

Concurrent chemo-radiation (CRT) has been established as the standard of care for non-metastatic loco-regionally advanced nasopharyngeal carcinoma (NPC) but recently the addition of induction chemotherapy in the already established regimen has presented an attractive multidisciplinary approach. This retrospective study was carried out to evaluate the efficacy of induction chemotherapy (IC) followed by CRT for the management of loco-regionally advanced NPC. Between July 2005 and September 2010, 99 patients were treated with cisplatin based IC followed by CRT. Induction chemotherapy included a 2 drug combination; intravenous gemcitabine $1000mg/m^2$ on day 1 and 8 and cisplatin $75mg/m^2$ on day 1 only. Radiotherapy (RT) was given as a phase treatment to a total dose of 70 Gy in 35 fractions. Concurrent cisplatin ($75mg/m^2$) was administered to all patients on days 1, 22 and 43. All patients were evaluated for tumor response and adverse effects after IC and 6 weeks after the completion of the treatment protocol. Statistical analysis was performed using SPSS version 17 and Kaplan Meier estimates were applied to project survival. Median follow-up duration was 20 months. The 5-year overall survival (OS), loco regional control (LRC) and relapse free survival (RFS) rates were 71%, 73% and 50%respectively. Acute grade 4 toxicity related to induction chemotherapy and concurrent chemo-radiation was 4% and 2% respectively, with only 3 toxicity-related hospital admissions. We conclude that induction gemcitabine and cisplatin followed by chemo-radiation is a safe and effective regimen in management of nasopharyngeal carcinoma, meriting further investigation in randomized clinical trials.

• Asian Pacific Journal of Cancer Prevention
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• v.15 no.2
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• pp.831-835
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• 2014

Background: Predictor factors determining complete response to treatment are still not clearly defined. We aimed to evaluate clinicopathological features, risk factors, treatment responses, and survival analysis of patient with advanced nonseminomatous GCTs (NSGCTs). Materials and Methods: Between November 1999 and September 2011, 140 patients with stage II and III NSGCTs were referred to our institutions and 125 patients with complete clinical data were included in this retrospective study. Four cycles of BEP regimen were applied as a first-line treatment. Salvage chemotherapy and/or high-dose chemotherapy (HDCT) with autologous stem cell transplantation were given in patients who progressed after BEP chemotherapy. Post-chemotherapy surgery was performed in selected patients with incomplete radiographic response and normal tumor markers. Results: The median age was 28 years. For the good, intermediate and poor risk groups, compete response rates (CRR) were, 84.6%, 67.9% and 59.4%, respectively. Extragonadal tumors, stage 3 disease, intermediate and poor risk factors, rete testis invasion were associated with worse outcomes. There were 32 patients (25.6%) with non-CR who were treated with salvage treatment. Thirty-one patients died from GCTs and 94% of them had stage III disease. Conclusions: Even though response rates are high, some patients with GCTs still need salvage treatment and cure cannot be achieved. Non-complete response to platinium-based first-line treatment is a negative prognostic factor. Our study confirmed the need for a prognostic and predictive model and more effective salvage approaches.

• Clinical and Experimental Pediatrics
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• v.55 no.5
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• pp.164-170
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• 2012

Purpose: Dramatic improvement of hemangioma to propranolol has been recently reported; however, details on dose and duration of treatment, potential risks, and monitoring have not been determined. The objective of this study is to describe and analyze the use of propranolol as a first-line treatment or as a single therapy in management of complicated hemangioma. Methods: A retrospective chart review of eight patients diagnosed with hemangioma and treated with propranolol in Kangbuk Samsung Hospital from February 2010 to April 2011 was performed. Results: Eight patients with hemangioma with functional impairment, cosmetic disfigurement, or rapid growth were treated with propranolol. Five patients had solitary facial hemangioma. The mean age of symptoms at onset was 5 weeks. The median age for starting propranolol treatment was 5.5 months. Propranolol at 2 mg/kg/day was finally administered in divided doses with a gradual increase. Significant regression was observed in seven patients, and shrinkage in size, softening in consistency, and decrease in redness were evident within 4 weeks. Among them, six patients were still taking propranolol, and one patient had stopped after 12 months. Other one patient did not show significant improvement with satisfactory result after 3 months of propranolol use. Treatment with propranolol was well tolerated and had few side effects. No rebound growth was observed in any of the patients. Conclusion: We observed that use of propranolol was very effective in treatment of hemangioma without obvious adverse effects or relapse.

• Tuberculosis and Respiratory Diseases
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• v.40 no.4
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• pp.367-377
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• 1993

Background: The benefits of combination chemotherapy in unresectable non-small cell lung cancer remain uncertain. But, according to the recent reports, the response rates of cisplatin-based polychemotherapy regimens are higher than those of single agent. Also, the response rates of high-dose cisplatin group are higher than those of low-dose cisplatin group. In attemp to answer the question whether treatments, combination chemotherapy (high VPP) and combination chemotherapy with radiation therapy, improve survival in advanced non-small cell lung cancer, we begin to study. Method: Thirty-five patients above stage III, diagnosed histologically as non-small cell lung cancer, were enrolled. Among them, nineteen received a combination chemotherapy consisting of VP-16 & high-dose cisplatin (100 $mg/m^2$) and/or radiation therapy. The other group (16 subjects) received no therapy. To investigate the differences of survival and response rates between two groups and the side effects related to therapy, we reviewed patients' records. Results: 1) The overall objective response rate was 47%(9/19) with one complete remission. 2) In patients who received polychemotherapy and radiation therapy, the response rate was 60%(6/10) with one complete remission and survival rates of 3 months, 6 months and 12 months were 100%, 70% and 40%. 3) In patients who received polychemotherapy, the response rate was 33% (3/9) with no complete remission and survival rates of 3 months, 6 months and 12 months were 78%, 67% and 33%. 4) Overall, treated patients survived significantly longer (p<0.05) than non-treated patients (median survival 307 days versus 95 days). 5) Analysis of the various prognostic factors disclosed that good performance status, stage III and squamous cell type showed the good response rates. 6) The toxicities were nausea and/or vomiting (100%), alopecia (90%), anemia (79%), leukopenia (69%), thrombocytopenia (2%), increased creatinine (16%) and neurotoxicity (5%). Conclusion: According to above results, there are relatively good results that high VPP combination chemotherapy in advanced non-small cell lung cancer improves survival in the treated group than in the non-treated group. Thus, it is considerd that we select the patients with proper indications and treat them with effective chemotherpy and radiation therapy. But, because improvement related to high VPP ploychemotherapy is not marked in this study, it is necessary that we should investigate follow-up studies in many cases.

• Journal of Korean Neurosurgical Society
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• v.30 no.sup2
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• pp.289-293
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• 2001

Objectives : The optimal treatment of craniopharyngioma is controversial. Despite recent advances in microsurgical management, complete surgical removal of craniopharyngioma remains very difficult. Radiation added to surgery is effective, but radiation therapy resulted in untoward side effect in young patient. Gamma knife radiosurgery offers the theoretical advantage of a reduced radiation dose to surrounding structures during the treatment of residual or recurrent craniopharyngioma compared with fractionated radiotheraphy. We described retrospective analysis of tumor size and clinical symptoms of patients after gamma knife radiosurgery in residual or recurrent craniopharyngioma were performed. Material and Methods : From September 1990 to January 2000, 18 patients of craniopharyngioma were treated by gamma knife radiosurgery. All patient had undergone surgery, but residual or recurrent tumor was found and all of them treated postoperative gamma knife radiosurgery. The mean age was 19(from 6 to 66) and male to female ratio was 10 to 8 and 8 patients were below 15 years old. In young age group(below age 15), the average volume of the tumor was $2904.8mm^3$ and mean maximal gamma knife dose was 34.9Gy. In old age group(older than 15), the average volume of the tumor was $2590.4mm^3$ and mean maximal gamma knife dose was 45.2Gy. The size of the tumor was average $2730.1mm^3$($88-12000mm^3$), mean average radiation dose was 40.7Gy and the mean prescription dose was 17.6 Gy(4-35Gy) delivered to a median prescription 50.7% isodose. Results : The follow up was from 1 year to 9 years(mean 59.1 months) after gamma knife radiosurgery. The tumor was controlled in 13(72.2%) patients. The tumor decreased in 9 patients and not changed in 4 patients. The tumor size increased in 4(22.2%) patients during follow up period. In two cases the tumor size increased because of its cystic portion was increased, but their solid portion of the tumor was not changed. In another two patients, the solid portion of the tumor was increased. So, one patient underwent reoperation and the other patient underwent operation and repeated gamma knife radiosurgery. The tumor recurred in one case(5.6%) that is a outside of irradiated site. The presenting symptoms were improved in 4 patients(improved visual acuity in 1, controlled increased intracranial presure sign in 3 patients). In one case, visual acuity decreased after gamma knife radiosurgery. The endocrine symptoms were not influenced by gamma knife radiosurgery. Conclusion : Craniopharyngioma can be treated successfully by gamma knife radiosurgery. Causes of the tumor regrowth are inadequate dose planning because of postoperatively poor margination of the tumor, close approximation of optic nerve and residual tumors outside the target lesion. Recurrence can develop 4 years after gamma knife radiosurgery. Volume is important, but the accurate targeting is more important to prevent tumor recurrence. If the tumor definition is not clear during planning gamma knife surgery, long-term image follow up is required.

• Radiation Oncology Journal
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• v.27 no.1
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• pp.10-14
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• 2009

Purpose: This study was designed to evaluate the effectiveness and prognostic factors for patients treated with postoperative radiation therapy following surgery or with radiation therapy alone for squamous cell carcinoma of the esophagus. Materials and Methods: We retrospectively analyzed 132 esophageal cancer patients treated with postoperative radiation therapy following surgery or patients who were treated with radiation therapy alone at our institution from 1989 to 2006. Thirty-five patients had stage II disease, 88 patients had stage III disease and nine patients had stage IV disease. Tumors were located at the upper esophagus in 18 patients, the mid esophagus in 81 patients and the distal esophagus in 33 patients. Sixty patients were treated with radiation therapy alone and 72 patients were treated with postoperative radiation therapy following surgery. Eight patients received a dose less than 40 Gy and 78 patients received a dose of 40 to 50 Gy. The remaining 46 patients received a dose of 50 to 60 Gy. The majority of patients who underwent postoperative radiation therapy received a dose of 45 Gy. Results: Actuarial survival rates for all of the patients at two years and five years were 24% and 5%, respectively. The median survival time was 11 months. Survival rates for patients who underwent postoperative RT at two years and five years were 29% and 8%, respectively. The corresponding survival rates for patients who received radiation alone were 18% and 2%, respectively. Survival rates at two years and five years were 43% and 15% for stage II disease, 22% and 2% for stage III disease and 0% and 0% for stage IV disease, respectively; these findings were statistically significant. Two-year survival rates for patients with upper, middle and distal esophageal cancer were 19, 29% and 22%, respectively. Although there was a trend of slightly better survival for middle esophageal tumors, this finding was not statistically significant. Complete response to radiation was achieved in 13 patients (22%) and partial response to radiation was achieved in 40 patients (67%) who received radiation alone. No response to radiation was noted in seven patients (12%). A statistically significant difference in survival rates was seen between patients that had a complete response and patients that had a partial response. Two-year survival rates for patients that had a complete response versus patients that had a partial response were 31% and 17%, respectively. There were no survivors for patients with no response as determined at two-year follow-up. Conclusion: We conclude that radiation therapy is an effective treatment for esophageal cancer. Stage and response to radiation therapy were noted to be prognostic factors. A more effective treatment modality is needed to improve long term survival because of the relatively dismal prognosis for this tumor.

• Radiation Oncology Journal
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• v.24 no.4
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• pp.223-229
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• 2006

$\underline{Purpose}$: Combined modality therapy including chemotherapy, surgery and radiotherapy is considered the standard of care for the treatment of stage III non-small cell lung cancer (NSCLC). This study was conducted to evaluate the efficacy of paclitaxel and cisplatin with induction chemotherapy followed by concurrent chemoradiotherapy for stage IIIB NSCLC. $\underline{Materials\;and\;Methods}$: Between July 2000 and October 2005, thirty-nine patients with stage IIIB NSCLC were treated with two cycles of induction chemotherapy followed by concurrent chemoradiotherapy. The induction chemotherapy included the administration of paclitaxel ($175\;mg/m^2$) by intravenous infusion on day 1 and treatment with cisplatin ($75\;mg/m^2$) by intravenous infusion on day 1 every 3 weeks. Concurrent chemoradiotherapy included the use of paclitaxel ($60\;mg/m^2$) plus cisplatin ($25\;mg/m^2$) given intravenously for 6 weeks on day 43, 50, 57, 71, 78 and 85. Thoracic radiotherapy was delivered with 1.8 Gy daily fractions to a total dose of $54{\sim}59.4\;Gy$ in $6{\sim}7$ weeks (median: 59.4 Gy). $\underline{Results}$: The follow up period was $6{\sim}63$ months (median: 21 months). After the induction of chemotherapy, 41.0% (16 patients) showed a partial response and 59.0% (23 patients) had stable disease. After concurrent chemoradiotherapy, 10.3% (4 patients) had a complete response, 41.0% (16 patients) had a partial response, and the overall response rate was 51.3% (20 patients). The 1-, 2-, 3-year overall survival rates were 66.7%, 40.6%, and 27.4% respectively, with a median survival time of 20 months. The 1-, 2-, 3-year progression free survival rates were 43.6%, 24.6%, and 24.6%, respectively, with median progression free survival time of 10.7 months. Induction chemotherapy was well tolerated. Among 39 patients who completed the entire treatment including chemoradiotherapy, 46.3% (18 patients) had esophagitis greater than grade 3 and 28.2% (11 patients) had radiation pneumonitis greater than grade 3. $\underline{Conclusion}$: Paclitaxel and cisplatin with induction chemotherapy followed by concurrent chemoradiotherapy for stage IIIB NSCLC seems to be an effective treatment. Occurrence of esophagitis and pneumonitis represents a significant morbidity and suggests a modification of the treatment regimen, either with the chemotherapy schedule or with radiotherapy treatment planning.

• Radiation Oncology Journal
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• v.14 no.2
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• pp.137-147
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• 1996

Purpose : This report is the result f retrospective analysis for children who received prophylactic cranial irradiation combined with intrathecal chemotherapy. Materials and Methods : Ninety children with ALL who had got bone marrow remission after induction chemotherapy received PCI. All but 3 children were treated with a dose of 1800 cGy as a standard regimen. While the PCI was given, all patients received intrathecal chemotherapy. Results : Nine of 90 patients experienced CNS relapse during the duration of follow-up ranged from 36 to 96 months (median 60 months). Three children experienced BM relapse prior to CNS relapse. Therefore, CNS relapse rate as the first adverse event was $6.7\%$. Median time interval of CNS relapse was 16 months from the first day of hematologic complete remission. Eighty-nine percent of patients who had CNS relapse were associated with hematologic relapse. and $78\%$ of CNS relpase occurred during maintenance chemotherapy (on-therapy relapse). The CNS RFS at 2 and 5 years are $68\%$ and $42\%$, respectively with median of 43 months. The Prognostic factors affecting CNS RFS are initial WBC count (cut-off point of 50,000/ul), FAB subtype and CALGB risk criteria. The DFS at 2 and 5 years are 61 and $39\%$, respectively with median of 34 months. The prognostic factors affecting DFS are initial WBC count (cut-off point of 50,000/ul), FAB subtype, POG and CALGB risk criteria. Conclusions : In our study, $6.7\%$ of CNS relapse rate as a first adverse event was comparable with other studies. Various risk criteria was based on age at diagnosis and initial WBC count such as POG and CALGB criteria, had prognostic significance for CNS RFS and DFS. Prospective randomized trial according to prognostic subgroup based on risk criteria and systematic study about neuropsychologic function for long term survivors, are essential to determine the most effective and least toxic form of CNS prophylaxis.

• Pediatric Infection and Vaccine
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• v.26 no.3
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• pp.179-187
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• 2019

Purpose: Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome is a leading cause of periodic fever in children. This study describes the clinical characteristics of PFAPA syndrome in patients from a single center. Methods: Thirteen children diagnosed with PFAPA syndrome at Seoul National University Children's Hospital were included in this study. Retrospective medical chart reviews were performed. Results: Among the 13 patients, 8 (61.5%) were male. The median follow-up duration was 3.3 years (range, 10 months-8.3 years). The median age of periodic fever onset was 3 years (range, 1-6 years). All patients had at least 5 episodes of periodic fever and pharyngitis, managed with oral antibiotics, before diagnosis. The median occurrence of fever was every 3.9 weeks and lasted for 4.2 days. All patients had pharyngitis and 12 (92.3%) had cervical lymphadenitis. Blood tests were performed for 12 patients, and no patients had neutropenia. Both the C-reactive protein and erythrocyte sedimentation rate were elevated at medians of 4.5 mg/dL (range, 0.4-13.2 mg/dL) and 29 mm/hr (range, 16-49 mm/hr), respectively. Throat swab cultures and rapid streptococcal antigen tests were negative. Nine (69.2%) patients received oral prednisolone at a median dose of 0.8 mg/kg, and in 6 (66.7%) patients, fever resolved within a few hours. Three (23.1%) patients received tonsillectomy and adenoidectomy. Conclusions: PFAPA syndrome should be considered when a child presents with periodic fever along with aphthous stomatitis, pharyngitis, or cervical lymphadenitis. Glucocorticoid administration is effective for fever resolution and can reduce unnecessary use of antibiotics.