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Renal epithelioid aggressive angiomyolipoma in a patient with tuberous sclerosis and past contralateral benign renal angiomyolipoma. 结节性硬化症合并对侧良性肾血管平滑肌脂肪瘤患者的肾上皮样侵袭性血管平滑肌脂肪瘤。
IF 0.9 4区 医学 Q4 ONCOLOGY Pub Date : 2025-01-01 Epub Date: 2025-05-16 DOI: 10.4103/ijc.ijc_501_21
Irene Pecorella, Andrea Tornese, Emma Rullo

Abstract: Twenty-five percent of all aggressive angiomyolipomas (AMLs) are related to tuberous sclerosis. Our patient was a 42-year-old man with tuberous sclerosis and a medical history of a benign AML in the left kidney. Presently, an epithelioid AML was detected in his right kidney and, despite our conducting extensive surgery, a large pulmonary metastasis with mediastinal lymph node involvement followed 20 months later. Close follow-up of the patient is advised when epithelioid cells are observed in angiomyolipoma. Immunohistochemistry for mismatch repair proteins showed that PMS2, MSH2, and MSH6 were absent in both the tumor and normal kidney. Microsatellite instability may contribute along with a mutation of TSC gene to the oncogenic events leading to AML.

25%的侵袭性血管平滑肌脂肪瘤(AMLs)与结节性硬化症有关。我们的病人是一名42岁的男性结节性硬化症和良性AML在左肾病史。目前,在他的右肾检测到一种上皮样AML,尽管我们进行了广泛的手术,但20个月后,他出现了大的肺转移并累及纵隔淋巴结。当在血管平滑肌脂肪瘤中观察到上皮样细胞时,建议密切随访患者。错配修复蛋白免疫组化检测显示,PMS2、MSH2和MSH6在肿瘤和正常肾脏中均不存在。微卫星不稳定性可能与TSC基因突变一起导致AML的致癌事件。
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引用次数: 0
Rare scenarios in germ cell tumors: A case series. 生殖细胞肿瘤的罕见情况:一个病例系列。
IF 0.9 4区 医学 Q4 ONCOLOGY Pub Date : 2025-01-01 Epub Date: 2025-05-16 DOI: 10.4103/ijc.ijc_1230_21
Lakshmi H Kamala, Geetha Narayanan, Arun Vasudevan, Neelima Radhakrishnan

Abstract: Germ cell tumors are native to the testis, retroperitoneum, and mediastinum. Here, we report our experience on rare scenarios in this entity. The first patient presented with a primary mediastinal germ cell tumor with 2° hemophagocytosis. This dysregulated immune milieu portends a dismal outcome irrespective of treatment. Gastrointestinal involvement in germ cell tumor accounts for only 5% of cases. The Second case with duodenal involvement and hematemesis is believed to be due to contiguous infiltration of the retroperitoneal node. Another case with bleeding ileal intussusception was seen after high-dose chemotherapy in a relapsed non-gestational choriocarcinoma. Bone metastases are exceptional in seminomas and we report such a case as well. Our next patient had thrombotic cardiac involvement which is scarce in the literature. Last patient in this series is an 81-year-old man with sertoli leydig cell tumor. This rare disease in elderly should be differentiated from other entities as the management for each differs.

摘要:生殖细胞肿瘤原发于睾丸、腹膜后和纵隔。在这里,我们报告了我们在该实体中罕见情况下的经验。第一例患者表现为原发性纵隔生殖细胞肿瘤伴2°噬血。无论治疗如何,这种失调的免疫环境预示着一个令人沮丧的结果。生殖细胞瘤累及胃肠道的病例仅占5%。第二例十二指肠受累和呕血被认为是由于腹膜后淋巴结的连续浸润。另一例复发的非妊娠期绒毛膜癌在大剂量化疗后出现回肠肠套叠出血。骨转移是罕见的精原细胞瘤,我们报告这样的情况下,以及。我们的下一个病人有血栓性心脏受累,这在文献中是罕见的。本系列的最后一位患者为81岁男性,患有支持leydig细胞瘤。这种罕见的老年疾病应与其他疾病区分开来,因为每种疾病的治疗方法不同。
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引用次数: 0
Distribution of genetic polymorphisms of nucleotide excision repair genes and risk of gastrointestinal cancer: Findings from a case-control study. 核苷酸切除修复基因的遗传多态性分布与胃肠道癌症的风险:一项病例对照研究的结果。
IF 0.9 4区 医学 Q4 ONCOLOGY Pub Date : 2025-01-01 Epub Date: 2025-05-16 DOI: 10.4103/ijc.ijc_1_22
Madhavi N Patil, Kailas Dhondibhau Datkhile

Background: Gastrointestinal cancer (GI) is one of the most common and deadly cancers worldwide. In the present study, we assessed the association between single nucleotide polymorphisms (SNPs) within nucleotide excision repair (NER) pathway genes (xeroderma pigmentosum complementation group C [XPC], xeroderma pigmentosum complementation group G [XPG], and xeroderma pigmentosum complementation group D [XPD]) and the GI cancer risk in the rural population of Maharashtra.

Methods: The genotyping of XPC, XPD, and XPG genes was studied by polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP) method using 200 clinically confirmed GI cancer cases and equal number of healthy controls. The association of polymorphisms was confirmed by odds ratio (OR) with 95% confidence interval (CI).

Results: The frequency distribution of XPD gene (C22541A) polymorphism showed that variant A/A genotype increased four times (OR = 4.08; 95% CI = 2.14-7.77; P: < 0.0001) and G23591A polymorphism with heterozygous G/A genotype showed significant correlation (OR = 6.90; 95% CI = 4.28-11.11; P < 0.0001) with risk of GI cancer compared to variant A/A genotype (OR = 1.92; 95% CI = 1.00-3.69; P = 0.04). The results of genetic association analysis of XPC at codon 939 of exon 15 and XPG at codon 1104 of exon 15 showed no association with GI cancer risk in the studied population.

Conclusion: Our results indicated that XPD Arg156Arg and Asp312Asn polymorphisms were significantly associated with GI cancer risk whereas XPC Lys939Gln and XPG His1104Asp polymorphisms were not statistically significant.

背景:胃肠道癌(GI)是世界范围内最常见和最致命的癌症之一。在本研究中,我们评估了核苷酸切除修复(NER)途径基因(着色性干皮病补体组C [XPC]、着色性干皮病补体组G [XPG]和着色性干皮病补体组D [XPD])内的单核苷酸多态性(snp)与马哈拉施特拉邦农村人群GI癌风险的关系。方法:采用聚合酶链反应-限制性片段长度多态性(PCR-RFLP)方法对200例临床确诊的胃肠道肿瘤患者和同等数量的健康对照进行XPC、XPD和XPG基因分型研究。通过比值比(OR)和95%置信区间(CI)证实多态性的相关性。结果:XPD基因(C22541A)多态性频次分布显示变异A/A基因型增加4倍(OR = 4.08;95% ci = 2.14-7.77;P: < 0.0001), G23591A多态性与杂合子G/A基因型呈显著相关(OR = 6.90;95% ci = 4.28-11.11;P < 0.0001)与变异A/A基因型相比(OR = 1.92;95% ci = 1.00-3.69;P = 0.04)。15外显子939密码子XPC和15外显子1104密码子XPG的遗传关联分析结果显示,与研究人群的GI癌风险无相关性。结论:XPD的Arg156Arg和Asp312Asn多态性与GI癌风险显著相关,而XPC的Lys939Gln和XPG的His1104Asp多态性与GI癌风险无统计学意义。
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引用次数: 0
Carbimazole-induced acute myeloid leukemia: A very rare case report and review of the literature. 卡马唑致急性髓系白血病:一例罕见病例报告及文献复习。
IF 0.9 4区 医学 Q4 ONCOLOGY Pub Date : 2025-01-01 Epub Date: 2025-05-16 DOI: 10.4103/ijc.ijc_366_21
Lalchand Mittal, Sandeep Jasuja

Abstract: Neutropenia is a common side effect in hyperthyroid patients with long-term use of antithyroid drugs. Antithyroid drugs have been used for the management of hyperthyroidism and rarely may cause life-threatening side effects such as pancytopenia and very rarely may cause acute leukemia. The current case report is of a 34-year-old man diagnosed with hyperthyroidism who presented with pancytopenia after treatment with carbimazole for past 7 months; he initially improved with supportive treatment. The patient restarted treatment with carbimazole but with a reduced dose. He was readmitted for fever and high total leukocyte counts after 3 months of restarting carbimazole. The patient was thoroughly investigated, and bone marrow aspiration examination showed acute myeloid leukemia. This presentation of acute myeloid leukemia was likely attributed to the reexposure to carbimazole, although the association of acute myeloid leukemia with autoimmune thyroid disease cannot be denied.

摘要:中性粒细胞减少是长期使用抗甲状腺药物的甲亢患者常见的副作用。抗甲状腺药物已被用于治疗甲状腺功能亢进,很少会引起危及生命的副作用,如全血细胞减少症,很少会引起急性白血病。目前的病例报告是一名34岁的男性,诊断为甲状腺功能亢进,在使用卡马唑治疗过去7个月后出现全血细胞减少症;他最初通过支持性治疗有所改善。患者重新开始卡马唑治疗,但剂量减少。重新使用卡咪唑3个月后,患者因发热和白细胞总数高再次入院。对患者进行了彻底的检查,骨髓穿刺检查显示急性髓性白血病。尽管不能否认急性髓性白血病与自身免疫性甲状腺疾病的关联,但这种急性髓性白血病的表现可能归因于再次暴露于卡马唑。
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引用次数: 0
News in oncology. 肿瘤学新闻。
IF 0.9 4区 医学 Q4 ONCOLOGY Pub Date : 2025-01-01 Epub Date: 2025-05-16 DOI: 10.4103/ijc.ijc_3_25
Prahlad H Yathiraj, Anuja Raniwala, Ritika Harjani Hinduja
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引用次数: 0
Nonoperative management in low-lying rectal cancers undergoing chemoradiation. 低位直肠癌放化疗的非手术治疗。
IF 0.9 4区 医学 Q4 ONCOLOGY Pub Date : 2025-01-01 Epub Date: 2025-05-16 DOI: 10.4103/ijc.ijc_1248_21
Debanjali Datta, Reena Engineer, Avanish Saklani, Ashwin D'souza, Akshay Baheti, Suman Kumar, Rahul Krishnatry, Vikas Ostwal, Anant Ramaswamy, Prachi Patil

Background: To evaluate outcomes of postneoadjuvant chemoradiotherapy (NACTRT) wait and watch strategy (WWS) in distal rectal cancers.

Methods: All consecutive patients from December 2012 to 2019 diagnosed with distal rectal tumors (T2-T4 N0-N+) having a complete or near-complete response (cCR or nCR respectively) post-NACTRT and wished for nonsurgical treatment option of WWS were included in this study. Patients were observed with 3 monthly MRI, sigmoidoscopy, and digital rectal examination (DRE) for 2 years and 6 monthly thereafter. Organ preservation rate (OPR), local regrowth rate (LRR), nonregrowth recurrence-free survival, and overall survival (OS) were estimated using the Kaplan-Meier method, and factors associated with LRR were identified on univariate and multivariate analysis using the log-rank test (P < 0.05 significant).

Results: Sixty-one consecutive patients post-NACTRT, achieving cCR [44 (72%)] and nCR [17 (28%)], respectively, were identified. All patients received pelvic radiotherapy to a dose of 45-50Gy conventional fractionation with concurrent Capecitabine. Additional boost dose with either external beam or brachytherapy was given to 39 patients. At a median follow-up of 39 months, 11 (18%) patients had local regrowth, of which seven were salvaged with surgery and the rest are alive with disease, as they refused surgery. The overall OPR, nonregrowth recurrence-free survival, OS was 83%, 95%, and 98%, respectively. Seven (11%) patients developed distant metastasis, of which six underwent metastasectomy and are alive and well. LRR was higher in patients with nCR versus cCR (P = 0.05).

Conclusion: The wait-and-watch strategy is a safe nonoperative alternative management for selected patients attaining cCR/nCR after NACTRT with excellent outcomes.

背景:评价远端直肠癌新辅助后放化疗(NACTRT)等待观察策略(WWS)的疗效。方法:所有2012年12月至2019年12月诊断为直肠远端肿瘤(T2-T4 N0-N+), nactrt后完全或接近完全缓解(分别为cCR或nCR)并希望非手术治疗WWS的患者均纳入本研究。随访2年,随访6个月,每月进行MRI、乙状结肠镜检查和直肠指检(DRE)。采用Kaplan-Meier法估计器官保存率(OPR)、局部再生率(LRR)、非再生无复发生存率和总生存率(OS),并通过单因素和多因素log-rank检验确定与LRR相关的因素(P < 0.05显著)。结果:连续61例nactrt患者,分别达到cCR[44(72%)]和nCR[17(28%)]。所有患者均接受盆腔放疗,剂量为45-50Gy,常规分割,同时使用卡培他滨。对39名患者进行了外束或近距离治疗的额外增强剂量。在39个月的中位随访中,11例(18%)患者局部再生,其中7例通过手术挽救,其余患者因拒绝手术而存活。总OPR、无复发生存率、OS分别为83%、95%和98%。7例(11%)患者发生远处转移,其中6例进行了转移切除术,存活良好。nCR患者的LRR高于cCR患者(P = 0.05)。结论:对于NACTRT后达到cCR/nCR的患者,等待观察策略是一种安全的非手术治疗方法,具有良好的预后。
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引用次数: 0
Demystifying the conundrum of medullary thyroid cancers and providing a brief review of literature. 揭开甲状腺髓样癌的神秘面纱,并提供简短的文献回顾。
IF 0.9 4区 医学 Q4 ONCOLOGY Pub Date : 2025-01-01 Epub Date: 2025-05-16 DOI: 10.4103/ijc.ijc_992_21
Arvind Krishnamurthy, Srikamakshi Kothandaraman, Mss Keerthi, Krishna Kumar Ramachandran

Background: The aim of this study was to evaluate the clinicopathological features, patterns of care, and the survival outcomes of patients with medullary thyroid cancers (MTCs) treated at our tertiary care center.

Methods: We conducted a retrospective analysis of all patients with a histological diagnosis of MTC and who had been treated at our tertiary care center from August 1, 1986 through November 31, 2019.

Results: MTC in 83 patients were managed in the said period and comprised 44 men and 39 women. The median age of the patient cohort was 45 years. Seventy-seven of 83 patients underwent upfront surgery that entailed a total thyroidectomy and a central compartment neck dissection and a lateral compartment neck dissection, as was deemed appropriate. The disease-free survival rates at 5, 10, and 20 years for the present study were 62%, 51%, and 46%, respectively, and the overall survival rates for the same periods were 85%, 80%, and 76%, respectively. A formal analysis of the various prognostic factors was made. In multivariate analysis, the factors that significantly influenced the disease-free survival included gender and lymph node ratio. Furthermore, on multivariate analysis, the factors that significantly influenced the overall survival included age and gender, with male patients and patients aged >55 years faring poorly.

Conclusion: Our study has clearly demonstrated the favorable long-term survival outcomes of patients with MTCs. Furthermore, the analysis of various prognostic factors adds to our understanding of the biology of this rare form of thyroid cancer.

背景:本研究的目的是评估在我们三级医疗中心治疗的甲状腺髓样癌(MTCs)患者的临床病理特征、护理模式和生存结果。方法:我们对1986年8月1日至2019年11月31日期间在我们三级保健中心接受治疗的所有组织学诊断为MTC的患者进行了回顾性分析。结果:83例MTC患者,男44例,女39例。患者队列的中位年龄为45岁。83例患者中有77例接受了术前手术,包括甲状腺全切除术、中央室颈清扫术和外侧室颈清扫术,这被认为是适当的。本研究5年、10年和20年的无病生存率分别为62%、51%和46%,同期的总生存率分别为85%、80%和76%。对各种预后因素进行了正式分析。在多因素分析中,显著影响无病生存的因素包括性别和淋巴结比例。此外,在多变量分析中,显著影响总生存率的因素包括年龄和性别,男性患者和年龄在bb0 ~ 55岁之间的患者预后较差。结论:我们的研究清楚地证明了MTCs患者良好的长期生存结果。此外,对各种预后因素的分析增加了我们对这种罕见形式甲状腺癌生物学的理解。
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引用次数: 0
A role of silicon spacer for mandibular reconstruction in oral cavity squamous cell carcinoma- A prospective evaluation. 硅垫片在口腔鳞状细胞癌下颌骨重建中的作用——一项前瞻性评价。
IF 0.9 4区 医学 Q4 ONCOLOGY Pub Date : 2025-01-01 Epub Date: 2025-05-16 DOI: 10.4103/ijc.ijc_824_21
Dhruv G Patel, Sanjay M Desai, Vinod Dhakad, Elroy Saldanha, Bonny Joseph, Sandeep Ghosh

Background: Although using a free flap with a microvascular technique is the gold standard of care, its use is limited in underdeveloped nations due to a lack of experience and resources. As a result, alloplastic materials and pedicled musculocutaneous flaps are commonly used in mandibular repair. Although numerous alloplastic materials have been employed with this flap, the majority of research has shown that they have a negative effect. The aim of the study was to see how effective silicon spacers are as a replacement for alloplastic materials in mandibular reconstruction.

Methods: A prospective study was conducted on patients who had mandibular reconstruction using soft-tissue repair with or without the use of a silicon spacer. The study comprised a total of 82 patients, 40 of whom had a silicon spacer and 42 of whom did not. The Chi-square test was utilized to assess the functional and cosmetic outcomes in both groups by using the EORTC head-and-neck questionnaire-35 analysis.

Results: The majority of the patients were in the T3 and T4a stages. The most common sub-site was buccal mucosa. The average silicon spacer retention time was 62 days, with a retention rate of 22.5%. Infection at the local portion, with or without orocutaneous fistula, was a common reason for silicon spacer removal. Only the speech parameter improved in the silicon spacer group, according to the EORTC head-and neck-questionnaire (P = 0.0290). In 16 individuals, radiation therapy was postponed, which was also significant (P = 0.0123).

Conclusion: In patients with oral cavity squamous cell carcinoma who underwent mandibular resection, using a silicon spacer in combination with soft-tissue restoration did not improve functional or cosmetic outcomes except for speech. For comprehensive mandibular reconstruction, only a myocutaneous flap is sufficient.

背景:尽管使用游离皮瓣与微血管技术是护理的金标准,但由于缺乏经验和资源,其使用在不发达国家受到限制。因此,同种异体材料和带蒂肌皮瓣常用于下颌骨修复。尽管许多同种异体材料已被用于该皮瓣,但大多数研究表明它们具有负面影响。这项研究的目的是为了了解在下颌骨重建中硅垫片作为同种异体材料的替代品是如何有效的。方法:对使用或不使用硅垫片进行下颌骨软组织修复的患者进行前瞻性研究。这项研究共包括82名患者,其中40名患者使用了硅衬垫,42名患者没有。通过EORTC头颈部问卷-35分析,采用卡方检验评估两组患者的功能和美容结果。结果:患者以T3、T4a期为主。最常见的亚部位为颊黏膜。硅隔离剂的平均滞留时间为62天,滞留率为22.5%。局部感染,有无口皮瘘,是硅垫片移除的常见原因。根据EORTC头颈调查问卷(P = 0.0290),只有硅垫片组的语音参数有所改善。16例患者延迟放疗,差异有统计学意义(P = 0.0123)。结论:在接受下颌骨切除术的口腔鳞状细胞癌患者中,使用硅垫片联合软组织修复并没有改善功能或美容结果,除了语言。对于全面的下颌骨重建,只有一个肌皮瓣是足够的。
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引用次数: 0
Role of precise management model in the maintenance of central venous indwelling catheters for cancer patients. 精准管理模式在肿瘤患者中心静脉留置管维持中的作用。
IF 0.9 4区 医学 Q4 ONCOLOGY Pub Date : 2025-01-01 Epub Date: 2025-05-16 DOI: 10.4103/ijc.ijc_1462_21
Hui Xu, Yibo Cai, Lingnv Xie

Background: Cancer is the leading cause of death worldwide. However, there is not a single study that has investigated the effect of a precise management model in cancer patients. This study aimed to explore the role of precise management in maintaining central venous indwelling catheters for cancer patients.

Methods: In total, 106 patients with central venous indwelling catheters were randomly divided into observation and control groups. The control group was given routine nursing intervention. A precise management model was adopted for the observation group. The incidence of central venous indwelling catheter-associated complications, patient treatment compliance, anxiety and depression scores, and patient satisfaction with the nursing services were compared between the two groups.

Results: We noticed that the psychological state of the patients in the two groups significantly improved after the nursing intervention compared to before the nursing intervention (P < 0.05), and this effect was more prominent in the observation group than in the control group (P < 0.05). In addition, the incidence of complications in the observation group was lower than that in the control group (P < 0.05). Moreover, we observed that treatment compliance and patient satisfaction with the nursing services were much higher in the observation group than in the control group (P < 0.05).

Conclusions: In the maintenance of central venous indwelling catheters for cancer patients, the implementation of a precise management model can significantly reduce the incidence of central venous indwelling catheter-associated complications, improve treatment compliance of patients, positively modulate patients' psychological state, and harmonize the nurse-patient relationship.

背景:癌症是世界范围内死亡的主要原因。然而,没有一项研究调查了精确管理模式对癌症患者的影响。本研究旨在探讨精准管理在肿瘤患者中心静脉留置导管维持中的作用。方法:106例中心静脉留置导尿管患者随机分为观察组和对照组。对照组给予常规护理干预。观察组采用精细化管理模式。比较两组患者中心静脉留置管相关并发症发生率、患者治疗依从性、焦虑抑郁评分及患者对护理服务的满意度。结果:我们注意到,护理干预后两组患者的心理状态较护理干预前均有显著改善(P < 0.05),且观察组的效果较对照组更为显著(P < 0.05)。观察组并发症发生率低于对照组(P < 0.05)。观察组患者的治疗依从性及对护理服务的满意度明显高于对照组(P < 0.05)。结论:在肿瘤患者中心静脉留置管的维护中,实施精准化管理模式,可显著降低中心静脉留置管相关并发症的发生率,提高患者的治疗依从性,积极调节患者心理状态,协调护患关系。
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引用次数: 0
Survival and prognostic factors of childhood Ewing sarcoma - Experience from a cancer center in South India. 儿童尤因肉瘤的生存和预后因素——来自印度南部一个癌症中心的经验。
IF 0.9 4区 医学 Q4 ONCOLOGY Pub Date : 2025-01-01 Epub Date: 2025-05-16 DOI: 10.4103/ijc.ijc_1149_21
Binitha Rajeswari, V R Prasanth, Priyakumari Thankamony, Manjusha Nair, Guruprasad Chellappan Sojamani, Arun Sankar, Bhaskar Subin Sugath, K M Jagathnath Krishna, P Kusumakumary, K Jayasree

Background: Childhood Ewing sarcoma (ES) - both osseous and extraosseous - has good outcomes with aggressive treatment that includes chemotherapy, surgery, and radiation therapy (RT). In this paper, we report our experience on the survival and prognostic factors of ES.

Materials and methods: Seventy-four newly diagnosed patients with ES, less than 14 years of age, registered in Pediatric Oncology Department of our center, over a period of 5 years, from July 1, 2010 to June 30, 2015, were included.

Results: Mean age at presentation was 8.93 years. Primary osseous sites were the extremity (n = 30), pelvis (n = 10), chest wall (n = 9), spine (n = 7), and skull (n = 5), and the extraosseous primary site was involved in 17.6% (n = 13). Metastatic disease was present in 17 patients (23%). Also, 52 patients received three-weekly vincristine/doxorubicin/cyclophosphamide (VDC)/ifosfamide/etoposide (IE) and 21 patients received only VDC. There was no treatment-related mortality. Local control modalities were surgery alone (n = 12), definitive RT (n = 33), surgery and RT (n = 20), palliative RT (n = 4), or none (n = 4). At a median duration of follow-up of 64 months, the 5-year event-free survival (EFS) for the entire cohort was 50.4% (60.6% for localized and 17.6% for metastatic) and the 5-year overall survival (OS) was 54.6% (64.2% for localized and 23.5% for metastatic). Among the prognostic factors analyzed (age, gender, lactate dehydrogenase [LDH] levels, site, and metastases), presence of metastases was the only factor significantly associated with poorer EFS and OS.

Conclusion: Localized ES has a favorable outcome even in resource-limited settings, and treatment intensification by interval-compressed chemotherapy may improve the outcomes further. Presence of metastasis at presentation confers a poor outcome.

背景:儿童尤文氏肉瘤(ES) -骨性和骨外性-通过积极的治疗包括化疗,手术和放疗(RT)具有良好的预后。在本文中,我们报告了我们对ES的生存和预后因素的经验。材料与方法:纳入2010年7月1日至2015年6月30日在我中心儿科肿瘤科登记的5年间74例14岁以下新诊断ES患者。结果:平均发病年龄为8.93岁。原发骨性部位为四肢(n = 30)、骨盆(n = 10)、胸壁(n = 9)、脊柱(n = 7)和颅骨(n = 5),骨外原发骨性部位累及17.6% (n = 13)。17例患者(23%)存在转移性疾病。此外,52例患者每3周接受长春新碱/阿霉素/环磷酰胺(VDC)/异环磷酰胺/依托泊苷(IE)治疗,21例患者仅接受VDC治疗。没有与治疗相关的死亡率。局部控制方式为单纯手术(n = 12)、明确RT (n = 33)、手术加RT (n = 20)、姑息性RT (n = 4)或无RT (n = 4)。在64个月的中位随访期间,整个队列的5年无事件生存率(EFS)为50.4%(局部60.6%,转移性17.6%),5年总生存率(OS)为54.6%(局部64.2%,转移性23.5%)。在分析的预后因素(年龄、性别、乳酸脱氢酶(LDH)水平、部位和转移)中,转移的存在是唯一与较差的EFS和OS显著相关的因素。结论:即使在资源有限的情况下,局部ES也有良好的预后,通过间隔压缩化疗加强治疗可能进一步改善预后。出现转移的患者预后不佳。
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引用次数: 0
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Indian journal of cancer
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