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Decitabine in the Treatment of Acute Myeloid Leukemia and Myelodysplastic Syndromes, Which Combined with Complex Karyotype Respectively

  • Gao, Su;Li, Zheng;Fu, Jian-Hong;Hu, Xiao-Hui;Xu, Yang;Jin, Zheng-Ming;Tang, Xiao-Wen;Han, Yue;Chen, Su-Ning;Sun, Ai-Ning;Wu, De-Pei;Qiu, Hui-Ying
    • Asian Pacific Journal of Cancer Prevention
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    • v.16 no.15
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    • pp.6627-6632
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    • 2015
  • Background: We conducted a study exploring the clinical safety and efficacy of decitabine in patients with acute myeloid leukemia (AML) and myelodysplastic syndromes (MDS), combined with a complex karyotype. Materials and Methods: From April 2009 to September 2013, a total of 35 patients with AML/MDS combined with a complex karyotype diagnosed in the First Affiliated Hospital of Soochow University were included for retrospective analysis. All patients were treated with decitabine alone ($20mg/m^2$ daily for 5 days) or combination AAG chemotherapy (Acla 20mg qod*4d, Ara-C $10mg/m^2$ q12h*7d, G-CSF $300{\mu}g$ qd, the dose of G-CSF adjusted to the amount in blood routinely). Results: In 35 patients, 15 exhibited a complete response (CR), and 6 a partial response (PR), the overall response rate (CR+PR) being 60% (21 of 35). Median disease-free survival was 18 months and overall survival was 14 months. In the 15 MDS patients with a complex karyotype, the CR rate was 53.3% (8 of 15); in 20 AML patients with complex karyotype, the overall response rate was 65% (13 of 20). The response rate of decitabine alone (22 cases) was 56.5% (13 of 22), while in the combination chemotherapy group (13 cases), the effective rate was 61.5% (8 of 13)(P>0.05). There are 15 patients with chromosome 7 aberration, after treatment with decitabine, 7 CR, 3 PR, overall response rate was 66.7% (10 of 15). Of 18 patients with 3 to 5 kinds of chromosomal abnormalities, 66.7% demonstrated a response; of 17 with more than 5 chromosomal abnormalities, 52.9% had a response. In the total of 35 patients, with one course (23 patients) and ${\geq}$two courses (12 patients), the overall response rate was 40.9% and 92.3% (P<0.05). Grade III to IV hematological toxicity was observed in 27 cases (75%). Grade III to IV infections were clinically documented in 7 (20%). Grades I to II non-hematological toxicity were infections (18 patients), haematuria (2 patients), and bleeding (3 patients). With follow-up until September 2013, 7 patients were surviving, 18 had died and 10 were lost to follow-up. In the 6 cases who underwent allogeneic hematopoietic stem cell transplantation (HSCT) all were still relapse-free survivors. Conclusions: Decitabine alone or combination with AAG can improve outcome of AML/MDS with a complex karyotype, there being no significant difference decitabine in inducing remission rates in patients with different karyotype. Increasing the number of courses can improve efficiency. This approach with fewer treatment side effects in patients with a better tolerance should be employed in order to create an improved subsequent chance for HSCT.

Clinical Manifestations of Salmonellosis in Children during the last 12 Years: A Single Institution Experience (최근 12년간 발생한 살모넬라증 환아의 임상적 특성: 단일기관 연구)

  • Park, Jae-Hong;Lee, Taek-Jin
    • Pediatric Infection and Vaccine
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    • v.20 no.1
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    • pp.1-8
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    • 2013
  • Purpose : The purpose of this study is to investigate the clinical manifestations and antibiotic resistance of salmonellosis in children. Methods : We reviewed medical records and investigated the clinical characteristics of culture-proven childhood salmonellosis from January 2000 through December 2011 at the CHA Bundang Medical center. Results : We assessed 53 patients. The median age was 3-years-old (minimum 12 days, maximum 18-years-old) and the number of male patients was 33 (62.3%). It occurred most frequently in the summer (39%) and in 2001 (11 cases) however there was no case in 2009 and 2010. Salmonella typhi was isolated in 3 cases with septicemia. Antibiotic resistance to ampicillin was most frequently presented (30.2%) and 63.6% in serogroup B. No antibiotics resistance strains were cultured in patients with positive Salmonella typhi. Admitted patients from 2000 to 2011 were divided into 2 groups; group 1 from 2000 to 2005 and group 2 from 2006 to 2011. 40 cases belonged to group 1 and 13 cases were in the group 2. Group 2 showed more resistance to ampicillin than group 1 but without any statistical significance(25% vs. 38.5%, P=0.349). In group 1, the most common serotype was group D and in group 2, the most common serotype were group C and D. Conclusion : Salmonellosis in children was frequently occurred from 2000 to 2003 but decreased after 2004. There was no difference in clinical manifestations, serotypes and antibiotic resistances between the years.

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Bivalvation Valvuloplasty for Common Atrioventricular Valve Regurgitation in Functional Single Ventricle; Early and Mid-term Results (기능적 단심설에서 공통방실판 역류의 판막성형술; 판막 이분성형술(bivalvation)의 조기 및 중기 결과)

  • Chang, Yun-Hee;Sung, Si-Chan;Kim, Seon-Hee;Lee, Hyoung-Doo;Ban, Ji-Eun
    • Journal of Chest Surgery
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    • v.42 no.5
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    • pp.597-603
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    • 2009
  • Background: Atrioventricular valve regurgitation in pediatric patients with a functional single ventricles (FSV) - has been known as one of the important risk factors for death and unfavorable long-term results after a Fontan operation. We evaluated early and mid-term results of bivalvation valvuloplasty in FSV patients. Material and Method: We retrospectively evaluated 11 patients with a functional single ventricle who underwent bivalvationvalvuloplasty between 1999 and 2007. The degree of common atrioventricular valve regurgitation (CAVVR) was determined by color Doppler echocardiography (regurgitation grade scoring, trivial; 1, mild; 2, moderate; 3, severe; 4). Mean age at valve surgery was $6.9{\pm}7.0$ months (median 4 months, 24 days$\sim$21 months)and mean body weight was $6.2{\pm}2.8\;kg$ ($3.1{\sim}11.3\;kg$). Nine patients had isomerism heart and two of them had TAPVC. The concomitant procedures were performed in all but one patient. Additional commissural closure was performed in 3 patients and commissural annuloplasty in another 3 patients. Result: There was one hospital death after. surgery. A 32-day old patient who had been preoperatively dependent on a ventilator died of air way and lung problems 4.3 months after pulmonary artery banding and bivalvation valvuloplasty. Mean follow-up duration was 40 months ($4.3{\sim}114$ months). Mean preoperative CAVVR score was $3.3{\pm}0.6$, which decreased to $1.9{\pm}0.7$ postoperatively (p<0.0001). This residual regurgitation slightly increased to $2.2{\pm}0.4$ (no statistical significance) after a mean follow-up of 1.4.3 months. Six patients (60%) required re-operations for residual regurgitation at a subsequent bidirectionalcavopulmonary shunt or Fontan operation. One patient with Ebsteinoid malformation of the right sided atrioventricular valve required valve replacement due to stenoinsufficiency. Another patient required edge-to-edge repair at the right sided AV valve (between the right mural leaflet and the bridging leaflets). The remaining 4 patients required additional suture placements between bridging leaflets with or without commissural annuloplasty. All survivor had trivial or mild CAVVR at the latest follow-up. Conclusion: Bivalvation valvuloplasty for CAVVR in FSV patients is. an effective and safe procedure. However, significant numbers of the patients have small residual regurgitation and require additional valve procedures at subsequent operations. Long-term observations to monitor progression of the CAVVR is mandatory.

Clinical features of congenital muscular torticollis (선천성 근성 사경의 임상적 특징)

  • Jun, Ji Eun;Ryu, Hye Kyeong;Shim, Jae Won;Shim, Jung Yeon;Jung, Hye Lim;Park, Moon Soo;Kim, Deok-Soo
    • Clinical and Experimental Pediatrics
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    • v.50 no.3
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    • pp.241-247
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    • 2007
  • Purpose : Congenital muscular torticollis (CMT) is a common and benign congenital disorder of the musculoskeletal system in neonates and infants. The pathophysiology is that the sternocleidomastoid muscle (SCM) is shortened on the involved side by fibrosis, leading to ipsilateral tilt and contralateral rotation of the face and chin. In this study, we investigated the clinical features of CMT, the role of ultrasonography (USG) in prediction of prognoses and the clinical significance of early detection and treatment. Methods : Forty seven patients (M:F=31:16) were diagnosed as a CMT between March 2003 and May 2006. We reviewed age at diagnosis, physical findings, USG findings, treatment and therapeutic outcome from their medical records. Results : The median age at diagnosis was 90 days (18 days-9 years, 7 months) and the right side of neck was affected in more patients (right : left=26:21). Of 24 patients with a palpable neck mass, 21 had USG; 19 cases showed sternocleidomastoid tumor (SMT). In cases with no neck mass, USG was performed in 11 patients; seven had postural torticollis (POST), three had SMT and one had muscular torticollis (MT). Among 40 patients with follow-up, 36 had total resolution. There was negative correlation between the age at diagnosis and the recovery time, whereas the final outcome was not correlated with USG findings. However, the patients without positive findings in USG had earlier resolution (1 month vs 2.6 months, P=0.0008). The patients with SMT had earlier diagnosis and excellent outcomes. The patients with MT were delayed to diagnosis and had the longest time to resolve. Lastly, the patients with POST had delayed diagnoses, but they had excellent outcomes. Conclusion : Since the patients with delayed diagnoses, in despite of benign courses, may take a long time to resolve and rarely need surgical treatment, it is important to diagnose and treat early. This study showed that USG findings of the SCM may be used as predictive factors.

Optimization for the Physical Properties of Steamed Foam Cakes Prepared with Single-stage Method by Response Surface Methodology (반응표면 분석법에 의한 단단계법 거품형 찜 케이크의 물리적 특성의 최적화)

  • Kwhak, Sung-Ho;Jang, Myung-Sook
    • Journal of the Korean Society of Food Science and Nutrition
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    • v.34 no.4
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    • pp.557-566
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    • 2005
  • In preparation of steamed foam-cakes, effects of whipping time, amount of wheat flour, and amounts of emulsifier on physical properties of the steamed foam cakes were investigated using RSM (response surface methodology). The three independent variables selected for the RSM experiment were whipping time $(X_1)$, amount of wheat flour $(X_2)$, and amounts of wheat flour $(X_2)$, and concentration of emulsifier $(X_3)$ were set for single-stage mixing, respectively. A rotatable central composite design was used for treatment arrangement. The responses from the product for loaf volume, color values and textural properties were analysed. In the analysis of variance for the foam cakes prepared by single-stage method, significant interactions were observed between independent variables (experimental factors) and physical property like loaf volume (p<0.05); textural properties like hardness, gumminess, and chewiness (p<0.05). Among independent variables, concentration of emulsifier had the most effects on physical properties while whipping time. The ordinary points in surface response showed maximal points with physical property like colorimetric b value while other properties revealed saddle points. The 3-dimensional response surface graphs of the predicted regression models displayed decreasing loaf volumes with increasing whipping times and emulsifier concentrations beyond optimum levels. The optimum conditions for best loaf volume and textural property (hardness, gummimess and chewiness) of the products selected by extracting intersectional areas of the contour maps that commonly overlapped all characteristics were; $11\~13$ min whipping time, $470\~486\;g$ amount of wheat flour, and $19\~20\;g$ emulsifier concentration, in case of single-stage method. The median values extracted from the RSM experimental results for optimum manufacturing conditions for single-stage method, i.e., 12 min whipping time, 478 g amount of wheat flour, and 20 g emulsifier concentration were empirically proven to fit the predicted levels of physical properties from the final foam cakes.

The Role of Postoperative External Irradiation for the Incompletely Resected Meningiomas (불완전절제된 수막종에서 수술 후 방사선치료의 역할)

  • Kim Tae-Hyun;Yang Dae-Sik;Kim Chul-Young;Choi Myung-Sun
    • Radiation Oncology Journal
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    • v.18 no.2
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    • pp.85-91
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    • 2000
  • Purpose : The aim of this study is to look for the possible efficacy of postoperative external irradiation for incompletely resected meningiomas. Methods and Materials : From August 198: to January 1997, forty-four patients with intracranial meningioma were treated by postoperative external irradiation. Of the 44 meningiomas, 18 transitional, 13 meningotheliomatous, 6 hemangiopericytic, 4 atypical, 2 fibroblastic and 1 malignant meningioma were identified. We classified all patients into two groups by the histology. The benign group was consisted of the meningotheliomatous, transitional and fibroblastic types. The malignant group was consisted of the atypical, hemangiopericytic and malignat types. In the means of surgery, 37 patients were resected incompletely and 7 patients were managed by biopsy only. After surgery, all patients were received postoperative external irradiation. Radiotherapy was deliverd using Co-60 or 4 MV photon beam to a total dose of 50 to 65 Gy (mean dose 57.4 Gy) with a 1.8 to 2 Gy per fraction. The median follow-up was 48 months (range : 21 $\~$ 101 months). Multivariate analysis of the Influence by age, sex, location, histology and radiation dose on local control has been done using Cox's proportional hazard model. Results : 5-year local control rate was 93.8$\%$ for the benign histology and 51.8$\%$ for the malignant histology (p=0.0110) and overall local control rate at 5 years was 87.4$\%$. The analysis of the prognostic factors, such as age, sex, location, and radiation dose were not significant except for the histology. Conclusion : Adjuvant postoperative external irradiation appears to be significantly improved local control in the patients with incompletely resected meningiomas.

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Long-term Results of Breast-conserving Surgery and Radiation Therapy in Early Breast Cancer (초기 유방암의 유방보존수술과 방사선치료의 장기추적결과)

  • Kim, Jin-Hee;Byun, Sang-Jun
    • Radiation Oncology Journal
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    • v.27 no.3
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    • pp.153-162
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    • 2009
  • Purpose: To evaluate the long-term results after breast-conserving surgery and radiation therapy in early breast cancer in terms of failure, survival, and cosmesis. Materials and Methods: One hundred fifty-four patients with stage I and II breast cancer were treated with conservative surgery plus radiotherapy between January 1992 and December 2002 at the Keimyung University Dongsan Medical Center. According to TNM stage, 93 patients were stage I, 50 were IIa, and 11 were IIb. The affected breasts were irradiated with 6 MV photons to 50.4 Gy in 28 fractions over 5.5 weeks with a boost irradiation dose of 10~16 Gy to the excision site. Chemotherapy was administered in 75 patients and hormonal therapy in 92 patients with tamoxifen. Follow-up periods were 13~179 months, with a median of 92.5 months. Results: The 5- and 10-year overall survival rates were 97.3% and 94.5%, respectively. The 5- and 10-year disease-free survival (5YDFS and 10YDFS, respectively) rates were 92.5% and 88.9%, respectively; the ultimate 5YDFS and 10YDFS rates after salvage treatment were 93.9% and 90.2%, respectively. Based on multivariate analysis, only the interval between surgery and radiation therapy ($\leq$6 weeks vs. >6 weeks, p=0.017) was a statistically significant prognostic factor for DFS. The major type of treatment failure was distant failure (78.5%) and the most common distant metastatic site was the lungs. The cosmetic results were good-to-excellent in 96 patients (80.7%). Conclusion: Conservative surgery and radiation for early stage invasive breast cancer yielded excellent survival and cosmetic results. Radiation therapy should be started as soon as possible after breast-conserving surgery in patients with early breast cancer, ideally within 6 weeks.

Improvement of Fontan Circulatory Failure after Conversion to Total Cavopulmonary Connection (완전 대정맥-폐동맥 연결수술로 전환 후의 폰탄순환장애 개선)

  • Han Ki Park;Gijong Yi;Suk Won Song;Sak Lee;Bum Koo Cho;Young hwan Park
    • Journal of Chest Surgery
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    • v.36 no.8
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    • pp.559-565
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    • 2003
  • By improving the flow pattern in Fontan circuit, total cavopulmonary connection (TCPC) could result in a better outcome than atriopulmonary connection Fontan operation. For the patients with impaired hemodynamics after atriopulmonary Fontan connection, conversion to TCPC can be expected to bring hemodynamic and functional improvement. We studied the results of the revision of the previous Fontan connection to TCPC in patients with failed Fontan circulation. Material and method: From October1979 to June 2002, eight patients who had failed Fontan circulation, underwent revision of previous Fontan operation to TCPC at Yonsei University Hospital. Intracardiac anomalies of the patients were tricuspid atresia (n=4) and other functional single ventricles (n=4). Mean age at TCPC conversion was 14.0$\pm$7.0 years (range, 4.6~26.2 years) and median interval between initial Fontan operation and TCPC was 7.5 years (range, 2.4~14.3 years). All patients had various degree of symptoms and signs of right heart failure. NYHA functional class was 111 or IV in six patients. Paroxysmal atrial fibrillation (n:f), cyanosis (n=2), intraatrial thrombi (n=2), and protein losing enteropathy (PLE) (n=3) were also combined. The previous Fontan operation was revised to extracardiac conduit placement (n=7) and intraatrial lateral tunnel (n=1). Result: There was no operative death. Major morbidities included deep sternal infection (n=1), prolonged pleural effusion over two weeks (n=1), and temporary junctional lachyarrhythrnia (n=1). Postoperative central venous Pressure was lower than the preoperative value (17.9$\pm$3.5 vs. 14.9$\pm$1.0, p=0.049). Follow-up was complete in all patients and extended to 50,1 months (mean, 30.3$\pm$ 12.8 months). There was no late death. All patients were in NYHA class 1 or 11. Paroxysmal supraventricular tachycardia developed in a patient who underwent conversion to intraatrial lateral tunnel procedure, PLE was recurred in two patients among three patients who had had PLE before the convertsion. There was no newly developed PLE. Conclusion: Hemodynamic and functional improvement could be expected for the patients with Fontan circulatory failure after atriopulmonary connection by revision of their previous circulation to TCPC. The conversion could be performed with low risk of morbidity and mortality.

A Retrospective Analysis of Characteristics of Probiotics Associated with Invasive Bacterial Infections in Children (소아청소년에서 발생한 유산균 제제에 포함된 균주에 의한 침습성 감염증의 후향적 분석)

  • Koh, June Young;Seo, Euri;Lee, Jina
    • Pediatric Infection and Vaccine
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    • v.24 no.3
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    • pp.168-177
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    • 2017
  • Purpose: The purpose of this study was to analyze the clinical features and risk factors of invasive infections caused by Lactobacillus spp. and Saccharomyces spp., components of commercially available probiotics. Methods: We analyzed demographic and clinical data from children ${\leq}18$ years of age with an invasive infection caused by Lactobacillus spp. or Saccharomyces spp. at the Asan Medical Center Children's Hospital from January 1998 to June 2016. Probiotic consumption data were also analyzed. Results: During the study period, a total of 24 episodes of invasive infections were caused by Lactobacillus spp. (n=16) and Saccharomyces cerevisiae (n=8). Along with the increase of probiotic use (755,594 [days/1,000 patient-admission days] in 2001 to 2005, 1,444,066 in 2006 to 2010, and 6,904,736 in 2011 to 2016), the incidence of probiotic-associated invasive infection increased ($R^2=0.70$). The median age of the patients was 1.8 years (range, 2 months to 17 years), and most of them had underlying medical conditions. The 30-day mortality rate was 20.8% (5/24), and 11 (45.8%) of these patients resulted from a severe invasive infection. We determined the risk factors for invasive infection to be: previous intensive care unit stay (odds ratio [OR], 3.0; 95% confidence interval [CI], 1.5 to 6.1] and the presence of a central venous catheter (OR, 2.2; 95% CI, 1.2 to 4.3). Conclusions: Although the probiotic-associated invasive infections rarely occurred in children, the incidence has increased along with probiotic pressure. Judicious use of probiotics is mandatory, especially in young children with underlying medical conditions and continuous surveillance will be needed to minimize the safety concerns.

Results of Three-Dimensional Conformal Radiation Therapy for the Treatment of a Solitary Sternal Relapse of Breast Cancer (흉골에 단독 전이된 유방암의 삼차원 입체조형 방사선 치료 성적)

  • Kim, Hae-Young;Huh, Seung-Jae;Park, Won;Choi, Do-Ho;Kang, Min-Kyu;Yang, Jung-Hyun;Nam, Seok-Jin;Im, Young-Hyuck
    • Radiation Oncology Journal
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    • v.26 no.2
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    • pp.91-95
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    • 2008
  • Purpose: To evaluate the response and survival rate after three-dimensional conformal radiation therapy(3D-CRT) of patients with a solitary sternal relapse of breast cancer. Materials and Methods: Seventeen patients between May 1996 and June 2005 were evaluated with the salvage 3D-CRT treatment of a solitary sternal relapse of breast cancer. The treatment fields included the gross tumor volume with 2 cm margins. The total radiation dose was $35.0{\sim}61.5$ Gy(biologic effective dose of $43.7{\sim}76.9Gy_{10}$ using an $\alpha/\beta$ ratio of 10 Gy), with a daily dose of $1.8{\sim}3.0$ Gy. The tumor response was evaluated by the change in maximum tumor size via follow up CT scans $1{\sim}3$ months after the completion of treatment. Results: An objective tumor response was achieved in all patients, with a complete response in 5 patients and a partial response in 12 patients. The 5-year overall survival rate was 51.9%(median survival time: 27 months), and the most important factor affecting overall survival was the disease-free interval(interval from primary surgery of breast cancer to the development of sternal metastasis): The 5-year overall survival rate was 61.8% for patients with a disease-free interval ${\geq}12$ months and 0.0% for patients with disease-free interval <12 months(p=0.03). Conclusion: The response to 3D-CRT was good in patients with solitary sternal relapse of breast cancer. Particularly, patients with long disease-free interval from primary surgery survived significantly longer than patients with short disease-free interval from primary surgery.