康莱特注射液联合紫杉醇治疗晚期恶性胸腺瘤的疗效观察

目的:比较康莱特注射液联合紫杉醇及紫杉醇单纯化疗治疗晚期恶性胸腺瘤的不良反应和疗效。方法:2013年8月至2017年12月，经病理学和免疫组化确诊为恶性胸腺瘤并在我院肿瘤中心进行姑息性化疗的患者68例。康莱特注射液联合化疗为观察组（37例）；单纯化疗为对照组（31例）。28天为一周期，共化疗4周期。结果:两组患者均无人发生过敏反应。观察组可有效减轻患者化疗不良反应:恶性、呕吐、四肢麻木、关节肌肉酸痛和白细胞下降(P＜0.05)，可以有效减轻患者胸闷症状和全身疼痛(P＜0.05)，在肿瘤控制方面，康莱特联合化疗的疗效较单纯化疗稍好，但均未见有统计学差异(P＞0.05)。结论:康莱特注射液联合紫杉醇治疗晚期胸腺瘤，具有减轻患者疼痛、减轻化疗不良反应，从而提高患者化疗依从性的作用，值得临床进一步推广和研究。     

Ectopic Cervical Thymoma Excised through a Transcervical Approach Combined with Video-assisted Thoracoscopic Surgery: A Case Report

Ectopic cervical thymoma (ECT) is a rare tumor. We present a case of 56-year-old woman with an ECT in the anterior neck that was correctly diagnosed preoperatively. The patient had no symptoms of myasthenia gravis or other immune disorders, and the tumor was not invading any adjacent structures. We performed tumor resection and thymectomy through a transcervical approach using video-assisted thoracoscopic surgery with a multi-access single port. To our knowledge, this is a novel combined technique for the resection of an ECT.     

Good综合征诊治一例

Good综合征（伴胸腺瘤的免疫缺陷症）是一种成人免疫缺陷病,特点是合并胸腺瘤及低丙种球蛋白血症。该病有细胞和体液免疫双重缺陷,患者常表现为反复感染。该文报道1例62岁AB型胸腺瘤女性患者,反复细菌、真菌和病毒感染,血清Ig低下,B淋巴细胞缺如、CD4⁺ T淋巴细胞下降、CD4⁺/CD8⁺ T淋巴细胞比值下降,诊断为Good综合征。该患者同时有粒细胞缺乏、慢性病性贫血,进一步骨髓宏基因组测序发现KIT与ARID1A基因突变,予补充Ig及抗感染治疗,患者症状好转出院。随访患者仍有Ig下降,予定时复查血常规、Ig,并适时补充Ig。Good综合征相对罕见,临床表现复杂,容易延误诊断,临床医师需引起重视。     

TP53 and EGFR Mutational Status in Thymoma: A Genetic Sequencing Study

Background and objective: Thymoma is a rare malignant tumor that usually with an indolent presentation, which was falsely assumed to be benign previously. The tumor suppressor P53 (TP53) and EGFR gene mutate most frequently in human cancers. We tried to investigate the presence of TP53 and EGFR mutations among thymoma patients referred to an Indonesian referral respiratory hospital and to discuss its potential role in thymoma management and prognosis. Material and methods: Surgically resected tumor tissues were collected from thymoma patients and then underwent genomic analysis. PCR was performed on the extracted Paraffinized  DNA to amplify exon 6 of TP53 and exons 18, 19, and 21 of EGFR. The evaluation of mutational status was done using direct sequencing and sequence analysis of purified PCR products. Results: Among 27 collected samples, TP53 exon 6 mutation, namely  missense mutation and nonsense mutation, was observed in two samples (7.4%). EGFR exon 18 mutation, namely E709K and nonsense mutation, was found in 2 samples (7.4%). An intronic mutation in EGFR exon 19 (3.7%) and exon 21 (3.7%) was observed in one sample. Conclusion: TP53 and EGFR mutations were not most frequent, so it seems that these genes are not involved in the pathogenesis of thymoma in Indonesian patients. Nevertheless, we found two samples with a significant mutation in p53 and EGFR genes, suggesting further research on thymoma prognostification and targeted therapy.     

恶性胸腺瘤侵犯大血管的手术治疗及术后生存调查

目的分析恶性胸腺瘤患者的手术方式及其预后,以期寻找到侵犯大血管的恶性胸腺瘤的最佳治疗方案。方法回顾性总结2003年1月至2008年12月期间有明确肿瘤侵犯大血管的患者19例,其中行Y型血管置换术2例,行双根人工血管置换6例,行单根上腔静脉和(/或)其属支置换术6例,行上腔静脉和(/或)其属支成形术5例。5例患者行主动脉和(/或)其属支外膜剥离。13例患者同时切除受侵犯肺组织,行解剖学肺段切除2例,行肺叶切除11例。12例患者因肿块完全侵犯膈神经,行单侧膈神经切除11例,行双侧膈神经切除1例。结果 19例患者平均生存期44.1(3～114)个月。1年生存率88.2%(15/17),3年生存率64.7%(11/17),5年生存率50%(4/8)(截至2012年10月)。结论恶性胸腺瘤侵犯上腔静脉和无名静脉并致梗阻不应成为纵隔肿瘤局部根治性切除的禁忌。掌握手术指征和禁忌,争取根治性切除是提高生存率的关键。术后的后续治疗,尤其是放疗,也是延长患者生存时间的因素之一。     

胸腺切除术治疗重症肌无力的疗效及其影响因素分析

目的总结行胸腺切除术的重症肌无力(MG)患者的疗效,并探讨影响近远期疗效的相关因素。方法回顾性分析作者医院收治的103例行胸腺切除术的MG患者的临床资料,采用卡方检验及Logistic回归分析可能影响疗效的因素。MG患者的近期及远期疗效分别用Monden、美国重症肌无力联盟(MGFA)分型进行评估。结果 103例患者中完成随访94例(91.26%),随访时间3个月~8年。近期疗效总有效率为60.2%,远期疗效总有效率为73.4%。发病年龄、性别、Osserman分型与术后近远期疗效均无相关(P0.05);病程(OR=0.240、95%CI:0.081~0.712,P0.05)、手术方式(OR=0.289、95%CI:0.110~0.757,P0.05)与近期疗效密切相关,是否合并胸腺瘤与远期疗效密切相关(OR=0.073、95%CI:0.009~0.575,P0.05)。结论病程越短,手术近期疗效越好;行胸腔镜手术较开胸手术能明显提高患者近期预后;MG不合并胸腺瘤者行胸腺切除术较合并胸腺瘤的患者更能从手术中获益。     

Ectopic hamartomatous thymoma: report of a case and review of literature

Ectopic hamartomatous thymoma (EHT) is an exceedingly rare lesion that usually arises in the lower neck and mainly affects adult men. We present the clinicopathological features of a case of EHT in a 28-year-old Chinese male, together with a literature review. Ultrasound imaging and a computed tomography (CT) scan of the neck demonstrated a 3.0-cm well-defined nodule of heterogeneous density located within the left sternocleidomastoid muscle. The patient underwent a gross total resection of the tumor. Grossly, the well-demarcated, encapsulated mass had a predominantly solid and gray-white appearance admixed with microcystic foci filled with serous content and yellowish regions. The lesion consisted of an irregular admixture of spindle cells, epithelium, and mature adipose tissue. Immunohistochemistry showed that both the spindle cell and epithelial components were diffuse and had intense nuclear positivity for p63 and cytoplasmic reactivity for pan-cytokeratin, CK7, and CK19. The patient was followed for 18 months without any evidence of metastasis or recurrence.