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Uterine Sarcoma: Clinical Presentation, Treatment and Survival Outcomes in Thailand

  • Potikul, Chalermrat;Tangjitgamol, Siriwan;Khunnarong, Jakkapan;Srijaipracharoen, Sunamchok;Thavaramara, Thaovalai;Pataradool, Kamol
    • Asian Pacific Journal of Cancer Prevention
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    • v.17 no.4
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    • pp.1759-1767
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    • 2016
  • Background: Uterine sarcoma is a group of rare gynecologic tumors with various natures, and different lines of treatment. Most have a poor treatment outcome. This study targeted clinical characteristics, treatment, overall survival (OS), progression-free survival (PFS), and prognostic factors in uterine sarcoma patients in one tertiary center for cancer care. Materials and Methods: Uterine sarcoma patients who were treated at the Department of Obstetrics and Gynecology, Faculty of Medicine Vajira Hospital between January 1994 and December 2014 were identified. Clinico-pathological data were analyzed. Prognostic outcomes were examined by Kaplan-Meier curves and Cox regression analysis. Results: We identified 46 uterine sarcoma patients: 25 carcinosarcoma (CS) (54.3%), 15 leiomyosarcoma (LMS) (32.6%), and 6 undifferentiated uterine sarcoma (UUS) (13.1%) cases. Mean age was $54.0{\pm}11.9years$ (range 25-82 years). Abnormal uterine bleeding was the most common presenting symptom (63.0%). Among 33 patients (71.7%) who had pre-operative tissue collected, diagnosis of malignancy was correct in 29 (87.9%). All patients received primary surgery and retroperitoneal lymph nodes were resected in 34 (73.9%). After surgery, 5 (10.9%) had gross residual tumors. Stage I disease was most commonly found (56.5%). Adjuvant treatment was given to 27 (58.7%), most commonly chemotherapy. After a median follow-up of 16.0 months (range 0.8-187.4 months), recurrence was encountered in 22 patients (47.8%). Median time to recurrence was 5.8 months (range1.0-105.5 months). Distant metastasis was more common than local or locoregional failure. The 2-year PFS was 45.2% (95% confidence interval [CI], 30.6%-59.7%) and the 2-year OS was 48.3% (95% CI, 33.3%-60.7%). Multivariable analyses found residual disease after surgery as a significant factor only for PFS. Conclusions: Uterine sarcoma is a rare tumor entity. Even with multimodalities of treatment, the prognosis is still poor. Successful cytoreductive surgery is a key factor for a good survival outcome.

Prognosis and Management for Gallbladder Cancer with Hepatic Invasion: Long-term Results of 139 Patients from a Single Center in China

  • Qu, Kai;Chang, Hu-Lin;Liu, Si-Nan;Liu, Chang;Xu, Xin-Sen;Wang, Rui-Tao;Zhou, Lei;Tian, Feng;Wei, Ji-Chao;Tai, Ming-Hui;Meng, Fan-Di
    • Asian Pacific Journal of Cancer Prevention
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    • v.13 no.3
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    • pp.1015-1018
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    • 2012
  • Objective: To improve the diagnosis of primary gallbladder carcinoma (GBC) with/without hepatic metastases by analyzing our experience of different GBC treatment in our patients. Methods: A retrospective study was carried out to analyze the clinical data of the 139 patients with GBC who underwent hepatic resection in our unit from January 2003 to December 2007. Patients were divided into two groups according to whether they demonstrated hepatic invasion. Tumor presentation, surgical modes, and prognosis of each patient were retrospectively reviewed. Kaplan-Meier curves and log-rank tests were employed to compare the survival rates of those patients undergoing different surgical procedures. Results: Of the 139 patients, 46 were men and 93 were women with the male to female ratio of 1:2.0. Their ages were ranged from 35 to 86 years with a mean age of $62.8{\pm}10.4$ years. There were 73 patients complicated with hepatic invasion (group A), and no hepatic invasion occurred in the other 66 patients (group B). Compared with the group B, the patients with hepatic invasion suffered lower differentiation of tumor (p=0.000), more advanced Nevin staging (p=0.008) and poorer prognosis (p=0.013). Radical resection were more frequently performed in group B (75.76%) than in group A (45.20%) with better outcomes (p=0.000). Conclusion: GBC patients complicated with hepatic invasion had poorer prognosis than those without invasion in long-term follow-ups. Radical resection might result in a satisfied prognosis in patients without hepatic invasion, but appears less favorable than palliative resection in those who were complicated with hepatic invasion.

Face to Face with the Past: Memorizing the Plague of Athens through the Exhibition (과거와의 대면 : ${\ll}$미르티스${\gg}$ 전시를 통해 기억된 아테네 대 역병)

  • Cho, Eun-Jung
    • The Journal of Art Theory & Practice
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    • no.14
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    • pp.7-32
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    • 2012
  • The exhibition was started in 2010 in the New Acropolis Museum of Athens and embarked a journey since 2011 as a travelling exhibition inside Greece and abroad. The main purpose of the exhibition was to draw attention of the general public to the value of the 'rescue excavation' and of cultural heritage of Greece, by presenting the reconstruction bust of a girl whose skull was found in Kerameikos cemetery of ancient Athens. The new Kerameikos excavation was initiated by the construction of Metropolitan Railway lines in the center of Athens between 1992 to 1998. It revealed a pit of a mass burial where about 150 people were inhumed in a very hasty way without proper funeral rites or offerings. These bodies are identified as the victims of the infamous plague of Athens in the first years of the Peloponnesian War(430-426 BC). The epidemic disease killed almost one third of the city population including Pericles, and brought extreme fear and panic to the Athens society. The traditional funerary rites were totally disrupted, and the social decorum and the morality among the citizens became enfeebled. The plague and the civil war were the decisive factors to end the Golden Age of Democratic Athens. However, the exhibition organizers did not focus on the tragic aspect of this disaster and its casualties. Their main concern was to simplify the scholarly works of archaeological excavation and microchemistry analysis so that the exhibition viewers will easily understand and empathize the living value of the scholarly works of ancient Greek civilization. The centripetal element of the exhibition was the vivid face of an 11 years old ancient girl 'Myrtis', which was carefully reconstructed based on both the scientific data and artistic imagination. Also the set up of the exhibition was structured in order to stimuli cognitive and emotional experience of the visitors who witnessed the rebirth of a vibrant human being from an ancient debris. The museologists' continuous efforts to promote projects of contemporary artists, publications, and school programs related to the exhibition indicate that the ulterior motive of this exhibition is the cultural education of the present and future generation through the intimate experiences of ancient Greek life. Also this is the reason why the various museums that held the travelling exhibition try to make the presentation as a gesture of memorial service for an anonymous Athenian girl who deceased circa 2400 years ago. The pragmatic efforts of Greek scholars and museologists through exhibition show us a way to find a solution to the continuous threat of cultural resources by massive construction projects and land development, and to overcome public indifference to the history and cultural heritage.

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Comparison of Intraperitoneal and Retroperitoneal/Pelvic Contrast Extravasation: The Characteristics and Prognosis of the Each Patient Group with Arterial Embolization according to the Abdominal Computed Tomography Scanning after Blunt Trauma (둔상 후 복부 전산화단층촬영에서 조영제 유출로 동맥색전술을 시행받은 환자의 복강내와 후복막강/골반강내 출혈 비교)

  • Yoon, Ji Young;Kim, Sun Hyu;Ahn, Ryeok;Hwang, Jae Cheol;Hong, Eun Seog
    • Journal of Trauma and Injury
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    • v.22 no.2
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    • pp.199-205
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    • 2009
  • Purpose: This study compared the characteristics of and the prognosis for intraperitoneal and retroperitoneal/pelvic contrast extravasation, which had been confirmed by enhanced abdominal CT scan, after blunt trauma in patients who had undergone angiographic embolization. Methods: From January 2001 to March 2009, data were retrospectively collected regarding patients who had undergone contrast extravasation (CE) on CT scanning and arterial embolization after blunt trauma. The study patient group was divided into the intraperitoneal and the retroperitoneal/pelvic groups according to the area of contrast extravasation. We reviewed the initial demographic data, the location of injury, the solid organ injury, the embolized vessel, and the clinical outcome. Results: The mean age of the study subjects was $40.2{\pm}2.6$ years old, and there were 24 male patients. The intraperitoneal group included 10 patients, and retroperitoneal/pelvic group was comprised of 17 patients. The amount of transfusion from presentation to intervention and during the first 24 hours was greater in the retroperitoneal/pelvic group than in the intraperitoneal group. The intraperitoneal group showed a higher frequency and severity of liver injury than the retroperitoneal/pelvic group. Angiography revealed that the hepatic artery (n=4) was the most frequently embolized vessel in the intraperitoneal group, while the internal iliac artery (n=6), followed by the renal artery (n=4), internal pudendal artery (n=3), and the gluteal artery (n=2), were the most frequently injured vessels in the retroperitoneal/pelvic group. Conclusion: In patients with intra-abdominal contrast extravasation found on CT scanning and arterial embolization after blunt trauma, the need for transfusion was less in the intra-abdominal group than in the retroperitoneal/pelvic group. Liver injury was also more frequent and severe in the intraperitoneal group than in the retroperitoneal/pelvic group.

Radiation Therapy In Management Of Primary Non-Hodgkin's Lymphoma Of Central Nervous System (원발성 중추신경계 림프종에 대한 방사선치료)

  • Hong, Seong-Eon
    • Radiation Oncology Journal
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    • v.12 no.1
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    • pp.33-42
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    • 1994
  • From 1982 to 1991, sixteen Patients with primary non-Hodgkin's lymphoma of the central nervous system(CNS) were seen at Kyung Hee University Hospital. The most common subtypes were large, noncleaved cell lymphoma and immunoblastic lymphoma of B cells. Lesions most commonly involved were the parietal lobes and/or deep nuclei. Positive cerebrospinal fluid cytology was rare at initial presentation. Sixteen patients were treated with surgical biopsy or resection followed by whole brain radiotherapy at a median dose of 40 Gy(range=30-50 Gy) with variable boost doses. Of 16 patients who underwent surgery and postoperative radiotherapy, fourteen patients died between 2 and 49 months following treatment, and two are alive with no evidence of disease at 8 and 22 months. The 1-and 2-year survival rates were 55.6$ \% $ and 34.7$ \% $, respectively with 12 months of median survival. Patterns of failure were analyzed in eleven patients of total 16 patients. Failure at the original site of involvement was uncommon after radiotherapy treatment. In contrast, failure in the brain at sites other than those originally invovled was common in spite of the use of whole brain irradiation. Failure occurred in the brain 11/16(68.7$ \% $), in spinal axis 4/16(25.0$ \% $). The age, sex, location of involvement within CNS, numbers of lesion, or radiation dose did not influence on survival. The authors conclude that Primary CNS lymphoma is a locally aggressive disease that is poorly controlled with conventional radiation therapy. The limitation of current therapy for this disease are discussed, and certain promising modality should be made in regarding the management of future patients with this disease.

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Diagnosis and Endovascular Treatment of May-Thurner Syndrome (May-Thurner 증후군의 진단과 혈관내 치료)

  • 허균;이재욱;신화균;원용순
    • Journal of Chest Surgery
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    • v.37 no.11
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    • pp.911-917
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    • 2004
  • Background: There are limited number of reports on May-Thurner syndrome (Iliac vein compression syndrome) in Korea, We analysed the clinical features, diagnostic modalities and endovascular treatment of May-Thurner syndrome. Material and Method: We reviewed 12 cases of May-Thurner syndrome between March 2001 and June 2003. Mean age was $57.6\pm2$ years. We were used in venography, color doppler and computed tomographic angiography as diagnostic modalities and in thrombolysis, thrombectomy, angioplasty and stent insertion as endovascular treatment. Result: Clinical features showed edema of lower extremities in 4 patients, pain of lower extremities in 1 patient, edema with pain in 5 patients, and all in 1 patient. In one patient, he did not have any pain and any edema of lower extremities but was diagnosed as May-Thurner syndrome using venography due to varicose veins on lower extremities. Diagnostic modalities included venography, computed tomographic angiography in all patients with clinical presentation except in one patient and color doppler was only performed only in 4 patients. Four kinds of endovascular treatment were performed for May-Thurner syndrome, angioplasty in 11 patients, stent insertion in 10 patients, thrombectomy in 9 patients and thrombolysis for 7 patients. Nine patients were followed up and we can show good blood flow in Left iliac vein for 7 of 9 patients. Conclusion: it is necessary to recognize the possibility of May-Thurner syndrome in Deep vein thrombosis patients and we should use a variety of modalities to diagnose May-Thurner syndrome. Finally, endovascular treatment is a safe and effective therapy for May-Thurner syndrome.

Clinical Review about Corrective Surgery of Tetralogy of Fallot (팔로사징증의 근치 수술에 관한 임상적 고찰)

  • 조광현
    • Journal of Chest Surgery
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    • v.24 no.7
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    • pp.674-684
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    • 1991
  • The surgical treatment of tetralogy of Fallot [TOF] was initiated by Blalock and Taussig in 1945 with the establishment of the subclavian artery to pulmonary artery anastomosis. In an imaginative and daring effort, in 1954, Lillehei and collaborators [1955] using controlled cross-circulation, carried out the first intracardiac repair of TOF by closing the ventricular septal defect [VSD] and relieving the pulmonary stenosis under direct vision. Nowadays, total correction is the ideal operation for treatment of TOF and is accomplished with extracorporeal circulation. And the results of surgery for TOF have steadily improved over the years, thanks to important contributions of many surgeons. Nevertheless because of its protean physiologic and anatomic presentation, TOF continues to offer challenges to cardiologist and cardiac surgeons. Thirty two cases of TOF have undergone total corrective surgery using extracorporeal circulation in the Department of Thoracic & Cardiovascular Surgery, Pusan Paik Hospital, Inje University, from Oct. 1985 to Feb. 1990. Clinical considerations were applied to these cases and the results were obtained as follows. 1. The heart lung machine used for extracorporeal circulation was SarnsO 7000, 5-head roller pump, and the number and type of oxygenators were 10 of bubble type and 22 of membrane type. The mean bypass time was 148.9 minutes and the mean aortic cross clamp time was 123.8 minutes. The GIK [glucose-insulin-potassium] solution was used as cardioplegic solution for myocardial protection during operation. 2. 20 cases were male and 12 were female, the mean age was 8 years old and the mean body weight was 25Kg. 3. The preoperative symptoms were cyanosis [29 cases], squatting [27 cases] and etc. The mean values of preoperative Hb., Hct., and SaO2 were 16.5 gm /dl, 50.3%, and 78.5%. 4. Combined anomalies were noticed in 16 cases [50%]. Among them 10 cases were PFO and 6 cases were ASD. 5. The degree of aorta overriding were 25% in 5 cases, 25 ~ 50% in 22 cases and above 50% in 5 cases. The dPA/Ao [ratio of diameter of pulmonary artery trunk to ascending aorta] were below 25% in 5 cases, 25 ~ 50% in 10 cases, 50 ~ 70% in 6 cases and above 75% in 11 cases. 6. The types of RVOT [right ventricular outflow tract] stenosis were valvular and infundibular in 14 cases [43.6%], diffuse hypoplastic type in 12 cases [37.5%], infundibular in 5 cases, and valvular and supravalvular in 1 case. 7. One stage radical corrective surgery was applied to the all cases. In widening of the RVOT, 3 types of patches were used: MVOP [monocusp ventricular outflow patch, Polystan BioprosthesesO] in 3 cases, knitted Dacron vessel patches in 2 cases, and double layer with bovine pericardium and woven Dacron prosthesis in 26 cases. 8. Postoperative complications were occurred in 15 cases. Among them, low output syndrome were occurred in 10 cases [31.3%] and 2 of them were expired postoperatively.

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Surgical Treatment of the Primary Mediastinal Tumors and Cysts (원발성 종격동종양 및 낭종의 외과적 치료)

  • 김병구;오태윤;장운하
    • Journal of Chest Surgery
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    • v.29 no.6
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    • pp.632-638
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    • 1996
  • A retrospective analysis of primary mediastinal tumors and cysts was performed on 42 patients who underwent surgical resection at our institution from january, 1985 to December, 1995. The patients consisted of 27 males and 15 females. The mean age was 40 years with a range of 10 month to 76 years. The patients were composed of thymlc tumor 12 cases (28.6 %), germ cell tumor 8 cases (19.0 %), primary cyst 7 cases (16.7 %), neurogenic tumor 6 cases (14.3 %) and other miscellanious tumor 9 cases. Overall, 3) (78.6 %) of the tumors were histologically benign, and 9 (21.4 %) were malig- nant. The noted clinical manifestations were respiratory symptoms such as chest pain, dyspnea and coughing. All of the patients with malignancy and 55 oyo of the patients with benign tumor were symptomatic on presentation. All of the patients were operated for tis ue diagnosis and curative resection. All the benign tumors ex- cept two cases of sarcoidosis were performed adequate curative resection. A few patients with malignant unresectable tumors were treated with chemotherapy or radiotherapy There were 7 (18 %) postoperative complications without mortality. In conclusion, Mediastinal tumors have long fascinated the thoracic surgeon because of their variety and unpredictability of diagnosis prior to exploration. We consider that active surgery and various combined modality can be accomplished with satisfactory result.

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Clinical Features, Response to Treatment, Prognosis, and Molecular Characterization in Korean Patients with Inherited Urea Cycle Defects

  • Yoo, Han-Wook;Kim, Gu-Hwan;Seo, Eul-Ju
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.2 no.1
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    • pp.77-79
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    • 2002
  • The urea cycle, consisting of a series of six enzymatic reactions, plays key roles to prevent the accumulation of toxic nitrogenous compound and synthesize arginine de novo. Five well characterized diseases have been described, resulting from an enzymatic defect in the biosynthesis of one of the normally expressed enzyme. This presentation will focus on two representative diseases; ornithine transcarbamylase(OTC) deficiency and citrullinemia(argininosuccinate synthetase deficiency). OTC deficiency is one of the most common inborn error of urea cycle, which is inherited in X-linked manner. We identified 17 different mutations in 20 unrelated Korean patients with OTC deficiency; L9X, R26P, R26X, T44I, R92X, G100R, R141Q, G195R, M205T, H214Y, D249G, R277W, F281S, 853 del C, R320X, V323M and 10 bp del at nt. 796-805. These mutations occur at well conserved nucleotide sequences across species or CpG hot spot. The L9X and R26X lead to the disruption of leader sequences, required for directing mitochondrial localization of the OTC precursor. Their phenotypes are severe, and neonatal onset. The G100R, R277W and V323M mutations were uniquely identified in patients with late onset OTC deficiency. The other genotypes are associated with neonatal onset. Out of 20 patients with OTC deficiency, only 6 patients are alive; two were liver transplanted, and normal in growth and development at 2, 4 years after transplantation respectively. Citrullinemia is an autosomal recessive disease, caused by the mutations in the argininosuccinate synthetase(ASS) gene. We identified in 3 major mutations in 11 unrelated Korean patients with citrullinemia; G324S, $IVS6^{-2}$ A to G, and 67 bp ins at nt 1125-1126. Among these, the 67 base pair insertion mutation is novel. The allele frequency of each mutation is; G324S(45%), IVS6-2 A to G(32%), and 67 base pair insertion(14%). All patients are diagnosed at neonatal or infantile age. Interestingly, two patients presented with stroke like episode. Out of 11 patients, 5 patients died. Among 6 patients alive, one patient was successfully liver transplanted.

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A Case of Short-chain Acyl-CoA Dehydrogenase Deficiency Detected by Newborn Screening

  • Park, Kyungwon;Ko, Jung Min;Jung, Goun;Lee, Hee Chul;Yoon, So Young;Ko, Sun Young;Lee, Yeon Kyung;Shin, Son Moon;Park, Sung Won
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.15 no.1
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    • pp.40-43
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    • 2015
  • Short-chain acyl-CoA dehydrogenase (SCAD) deficiency is an autosomal recessive mitochondrial disorder of fatty acid oxidation associated with mutations in the ACADS gene. While patients diagnosed clinically have a variable clinical presentation, patients diagnosed by newborn screening are largely asymptomatic. We describe here the case of a 1-year-old male patient who was detected by newborn screening and diagnosed as SCAD deficiency. Spectrometric screening for inborn errors of metabolism at 72hrs after birth showed elevated butyrylcarnitine (C4) level of 1.69 mol/L (normal, <0.83 mol/L), C4/C2 ration of 0.26 (normal, <0.09), C5DC+C60H level of 39 mol/L (normal, <0.28 mol/L), and C5DC/C8 ration of 7.36 (normal, <4.45). The follow-up testing at 18 days of age were performed: liquid chromatography tandem mass spectrometry (LC-MS/MS), urine organic acids, and quantitative acylcarnitine profile. C4 carnitine was elevated as 0.91; urine organic acid analysis showed elevated ethylmalonic acid as 62.87 nmol/molCr (normal, <6.5), methylsuccinate 6.81 nmol/molCr (normal, not detected). Sequence analysis of ACADS revealed a homozygous missense mutation, c.164C>T (p.Pro55Leu). He is growing well and no episodes of seizures or growth retardation had occurred.