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。     

重症肌无力患者血清Ryanodine受体抗体检测及意义

目的探讨Ryanodine受体（RyR）抗体在重症肌无力（myasthenia gravis，MG）诊断中的临床意义。方法采用ELISA法检测89例MG患者、66例其他神经系统疾病患者和66名正常对照者血清RyR抗体水平。结果MG组血清RyR抗体阳性率显著高于其他神经系统疾病组和正常对照组（P〈0．05），其敏感性和特异性分别为55．0％和91．7％。晚发型MG患者血清RyR抗体阳性率（74．4％）明显高于早发型MG（37．0％，P〈0．05）。MG合并胸腺瘤（MGT）和未合并胸腺瘤（nMGT）患者血清RyR抗体阳性率差异无统计学意义（P〉0．05）。将MG患者根据Osserman评分进行分型，各型血清RyR抗体阳性率及其吸光度值大小与病情严重程度无相关性（P〉0．05）。结论RyR抗体多见于晚发型MG，对诊断MG具有较高的敏感性和特异性。     

Childhood Malignant Thymoma: Clinical, Therapeutic, and Immunohistochemical Considerations

Malignant thymomas are among the least common mediastinal tumors in the pediatric age group. Thymomas are considered malignant on the basis of macroscopic and microscopic invasiveness. As only 20 well-documented cases involving children have been reported in the literature, the pattern of responsiveness to therapy and the value of prognostic signs is obscure. Two cases of malignant pediatric thymomas are reported with pathognomonic histoimmunological features of aggressive thymoma. One was cured, with a follow-up of 70 months, and one died while on therapy. Analysis of the histological features and the immunoperoxidase staining displays the complexity of pediatric thymomas and the inability to prognosticate the outcome, respectively.     

Exacerbation of myasthenia gravis after removal of thymomas

It appeared from this prospective and non-randomized study, that the removal of thymomas in myasthenia gravis (MG) patients resulted in rapid exacerbation of the clinical severity of the disease and of anti-acetylcholine receptor antibodies titres, which peaked after about 300 days and continued for up to 2 years. Long-term follow-up after thymomectomy (mean duration ± SEM after surgery 5.5 ± 0.8 years) showed that the immunological and clinical state observed prior to surgery was eventually restored, but long-term benefit attributable to surgery could not be demonstrated. Non-thymoma MG cases, however, exhibited post-operative amelioration in clinical course and decreasing antibodies titres, both of which were already significant one year after surgery, and additional improvement was observed at the time of long-term follow-up (mean 4.3 ± 0.5 years). Furthermore, the prognosis for MG patients not operated on was also favourable. It is suggested that the occurrence of thymomas is linked to genetic factors and that neoplasia of the thymus may be part of immunoregulatory mechanisms with predominance of inhibition.     

''Organoid'' thymoma: a well-differentiated variant with distinctive clinicopathological features

The present paper describes the clinical, histological and immunohistochemical features of five cases of 'organoid' thymoma. The histological hallmark of this lesion is the prominent and diffuse 'organoid' pattern, defined by the presence of several areas of medullary differentiation. These areas, which are strictly reminiscent of the medullary area of the normal thymus, are scattered within a neoplastic tissue resembling the thymus cortex, the overall appearance mimicking that of normal thymus. All cases shared common clinical features; they were non-invasive or minimally invasive tumours arising in young or middle-aged female patients. Although the incidence of 'organoid' thymoma is low (5.2% in our series), our morphological, immunohistochemical and clinical data suggest that this peculiar tumour may represent a well-differentiated variant of thymoma, with low-grade aggressiveness and a distinct clinicopathological profile.     

Ectopic cervico-mediastinal thymoma confirmed by flow cytometric analysis of tumor-derived lymphocytes

Ectopic cervical or cervico-mediastinal thymomas are very rare and most of them are asymptomatic, except for the presence of a cervical mass. We present the case of a 71-year-old man with an ectopic cervico-mediastinal thymoma threatening superior vena cava syndrome. He had a slight headache and presented with venous dilatation on the chest wall. A computed tomographic scan and magnetic resonance, imaging of the chest demonstrated a mass extending from the right neck to the hilum, that indented the trachea and compressed and displaced the brachiocephalic veins anteriorly. Under a right hemicollar incision and median sternotomy, the mass was resected en bloc together with the thymus. The resected specimen was an encapsulated mass measuring 11×7×4 cm. The pathological diagnosis was type AB, non-invasive thymoma, confirmed by 3-color flow, cytometry of tumor-derived lymphocytes. Flow cytometry using biopsy material may contribute to the preoperative diagnosis of ectopic thymoma.     

Genetic mapping of genes regulating the thymus size in back-cross rats between the laboratory BUF/Mna strain and the MITE strain derived from the wild rat, Rattus norvegicus

The thymoma prone BUF/Mna (B) rat is a useful model for Studying the genes responsible for thymus enlargement during the stage of young growth. Among the strains of rats, B rats have the largest thymuses at al stages of life. A locus, Ten-1 , which contributes to thymus enlargement in back-cross (BC) rats between the B and WKY/NCrj (W) strains, was mapped on chromosome 1. To determine the precise location of the bus, (B×(B×MITE)F1) BC rats were generated by crossing the B strain with the Inbred MITE (M) strain, which was established from captured, Japanese wild rats, and were examined by linkage study using polymerase chain reaction with 67 microsatellite markers. Linkages with thymus enlargements were found In genotypes of seven markers, BSIS, LSN, MYL2, IGF2, PBPC2, D1Mgh11 , and D1Mit6 , by X^2-test and Student's t -test, which confirmed the presence of the genetic locus associated with thymus enlargement, Ten-1 , in this region. Paradoxically, a suppressive locus, Tsu-1 , to thymus enlargement was also found on chromosome 3, showing linkages of phenotype of the small thymus with genotypes of SCN2A, CAT D3Mit16 , and D3Mit13 . By analyses of mapmaker/exp and mapmaker/qtl, Ten-1 was mapped at 4.6 cM proximal from IGF2 locus on chromosome 1 and Tsu-1 at 4.0 cM proximal from CAT locus on chromosome 3, respectively.     

Flow cytometric analysis of the DNA content of thymomas

In this study, the prognostic value of determining the nuclear DNA content of thymomas by flow cytometry was evaluated. Of a total 31 resected thymomas, 10 (32%) showed DNA aneuploidy, the presence of which was significantly correlated with an advanced clinical stage of disease. The patients with an aneuploid tumor had a poorer prognosis than those with a diploid tumor, demonstrating a survival rate of 50% at 7 postoperative years, which was considerably less favorable than that of the patients with a diploid tumor, being 100% in the same period (p<0.05). Moreover, patients with a high DNA index (DI), i.e., a DI1.5, tended to have a poorer prognosis than those with a low DI. These findings indicated that the DNA content can be an important prognostic index in patients with thymomas.     

胸腺瘤伴重症肌无力的围手术期治疗

目的　研究胸腺瘤伴重症肌无力患者的围手术期治疗。方法　自１９９１年７月至１９９８年７月,手术治疗胸腺瘤伴重症肌无力患者６例,占同期收治胸腺瘤患者的２７．３％ 。结果　Ｍａｓａｏｋａ Ⅰ期、Ⅱ期、Ⅳ期各１ 例,Ⅲ期３ 例。无手术死亡。术后１ 例１个月后死于呼吸衰竭,１ 例２ ａ后死于肿瘤复发。生存４ 例,３ 例治愈,１ 例好转。结论　认为胸部Ｘ线检查是诊断本病的重要方法。其良恶性的判断主要依靠术中所见肿瘤的生长方式及组织细胞学类型。加强围手术期处理,是降低手术并发症及死亡率的关键。强调合理使用抗胆碱酯酶药物;采用低位胸部正中切口,尽可能切除肿瘤,清扫前纵隔;尽早拔除气管内插管;选用有效抗生素防治肺部感染,以降低肌无力危象的发生率。