小儿生殖细胞肿瘤的诊断和治疗(附20例报告)

目的:探讨小儿生殖细胞肿瘤(GCT)的临床特征、病理类型、诊断、治疗方法。方法:对20例GCT患儿的临床资料进行回顾性分析。患儿年龄5月至9岁,其中卵巢肿瘤14例,睾丸肿瘤6例。结果:20例均行手术治疗,术后恢复良好,随访1~5年,全部存活。其中1例术后脾脏及肝脏先后出现转移灶,已再次手术。10例恶性卵巢肿瘤术后行PEB方案化疗。所有病例均经术后病理学检查确诊。结论:小儿GCT其临床表现无特异性,多以腹痛、腹胀、下腹部、盆腔及睾丸包块为首发症状。其预后与肿瘤的分期,组织级别有关。分期越早、级别越低,患者预后越好。早期手术切除是主要治疗方法,恶性肿瘤需要化疗或放疗,定期随访十分重要。     

Engrosamiento del tallo hipofisario en niños y adolescentes con diabetes insípida central: causas y consecuencias

BackgroundCentral diabetes insipidus (CDI) is a rare disorder in children. The aetiology of CDI in childhood is heterogeneous. The aim of this study is to illustrate the importance of a careful clinical and neuro-radiological follow-up of the pituitary and hypothalamus region in order to identify the aetiology and the development of associated hormonal deficiencies.MethodsClinical and auxological variables of 15 children diagnosed with CDI were retrospectively analysed in a paediatric hospital. Evaluations of adenohypophyseal function and cranial MRI were performed periodically.ResultsThe mean age at diagnosis of CDI was 9.6 years (range: 1.32-15.9). The aetiological diagnosis could be established initially in 9 of the 15 patients, as 7 with a germinoma and 2 with a histiocytosis. After a mean follow-up of 5.5 years (range: 1.6-11.8), the number of idiopathic cases was reduced by half. At the end of the follow-up, the aetiological diagnoses were: 9 germinoma (60%), 3 histiocytosis (20%), and 3 idiopathic CDI (20%). There is a statistically significant association between stalk thickening and tumour aetiology. At least one adenohypophyseal hormonal deficiency was found in 67% of cases, with the majority developing in the first two years of follow-up. Growth hormone deficiency (60%) was the most prevalent.ConclusionThe follow-up of CDI should include hormone evaluation with special attention, due to its frequency, to GH deficiency. In addition, a biannual MRI in an idiopathic CDI should be performed, at least during the first 2-3 years after diagnosis, as 50% of them were diagnosed with a germinoma or histiocytosis during this period.     

晚期与复发性卵巢恶性生殖细胞肿瘤129例治疗结果的分析

目的探讨改进晚期及复发性卵巢恶性生殖细胞肿瘤治疗的方法。方法回顾性分析１９５８～１９９３年我院收治的１２９例晚期与复发性卵巢恶性生殖细胞肿瘤的治疗结果。对１９８４～１９９３年间采用ＶＡＣ方案（长春新碱＋更生霉素＋环磷酰胺）、ＰＶＢ方案（顺铂＋长春花碱＋博莱霉素）及ＢＥＰ方案（博莱霉素＋足叶乙甙＋顺铂）治疗的患者生存情况,与１９５８～１９８３年间采用随意治疗方案的患者生存情况进行比较。结果１２９例晚期与复发性卵巢恶性生殖细胞肿瘤总的５年生存率为３０％（３９／１２９）,其中无性细胞瘤的５年生存率为６８％（１９／２８）,而非无性生殖细胞瘤为２０％（２０／１０１）。近１０年,卵巢非无性生殖细胞瘤患者的５年生存率已由３％提高到４２％。联合化疗对生存率的提高起着关键作用。３个联合方案,以ＢＥＰ方案疗效较好。结论卵巢内胚窦瘤的发生率在中国各类型卵巢恶性生殖细胞瘤中位居首位。为改善晚期与复发性卵巢恶性生殖细胞瘤患者的预后,应对所有患者尽早采用目前较有效的化疗方案——ＢＥＰ方案治疗。     

腹盆生殖细胞瘤的MRI、CT表现

目的：研究腹盆生殖细胞瘤ＭＲＩ、ＣＴ表现。方法：本组８例经病理证实腹盆生殖细胞瘤,均为男性,年龄２１～４８岁,平均年龄３０．０岁。ＭＲＩ检查５例,ＣＴ检查３例。结果：肿瘤位于腹膜后４例,盆间隙４例。肿瘤直径８～２０ｃｍ,４例直径＜１０ｃｍ,４例直径＞１０ｃｍ。圆形、椭圆形３例,不规则状５例。位于腹膜后呈不规则状,内部见囊变及钙化,侵犯周围组织;位于盆内呈境界清之肿块,以推移周围器官改变为主。ＣＴ或ＭＲＩ不易鉴别原发或继发病变。结论：腹盆内生殖细胞瘤在腹膜后和盆间隙表现不同,原发或继发病变表现相似。     

Germ cell tumors of the CNS in children: recent advances in therapy

Primary germ cell tumors of the central nervous system are rare neoplasms, accounting for no more than 2% of all malignancies in children and young people under 20 in the Western hemisphere. They have unique features related to age at diagnosis and sites of origin, as well as race and gender predilection. Prognosis has been clearly shown to be strongly related to pathological classification as either pure germinoma or nongerminomatous germ cell tumor, although many of these lesions are comprised of mixed elements. The presence of serum or cerebrospinal fluid tumor marker elevation has been an essential determinant of response to treatment. Because of the deleterious effects of irradiation on the immature nervous system, investigators have used chemotherapeutic strategies that either reduce or eliminate radiation therapy. In this article, we review the most recent advances in therapy for CNS germ cell tumors in the pediatric population and highlight the importance of cooperative trials in this setting. Received: 12 April 1999     

脑胚胎癌影像学诊断及鉴别诊断

目的:探讨脑胚胎癌的MRI、CT表现特征及其诊断价值.方法:通过回顾性分析经手术病理证实4例脑胚胎癌MRI及CT表现并复习文献,总结MRI及CT影像特征.结果:CT表现为不均匀等密度或稍高密度影,MRI表现为T1WI不均匀低信号、T2WI混杂高信号影,增强扫描呈不均匀显著强化.早期无明显占位效应,晚期肿瘤内可有不同程度坏死、囊变及出血.结论:脑胚胎癌MRI及CT表现具有特征性,其影像学诊断需结合临床,必要时需行诊断性放、化疗.     

纵隔原发性恶性生殖细胞瘤的CT和MRI表现

目的：探讨纵隔原发性恶性生殖细胞瘤的CT和MRI影像表现特点,以提高对本病的认识和诊断水平.方法：经病理证实的纵隔原发性恶性生殖细胞瘤13例,总结和分析其临床、CT及MRI表现.观察项目包括病灶数目、部位、大小,内部结构、边界及与周围结构的关系等.结果：13例均为青壮年患者,单发病灶,位于前中上纵隔,病灶最大截面直径约63 mm.CT表现为病灶内密度不均匀,可见片状坏死,增强后呈明显不均匀轻度强化.4例行MRI扫描,T1WI示病灶呈等信号或稍低信号、T2WI呈等信号或稍高信号,肿瘤内信号不均匀,12例伴有出血、坏死灶,增强扫描呈不均匀轻度～中度强化.病灶边界欠清,其中11例病变与纵隔血管间脂肪间隙消失,10例可见病变周围结构受侵犯,9例血管受侵,3例合并上腔静脉闭塞,6例累及心包,3例侵犯前胸壁及胸骨.结论：纵隔原发性恶性生殖细胞瘤CT和MRI表现具有恶性肿瘤侵袭性的特征,但影像表现缺乏特异性;结合临床及实验室检查有助于恶性生殖细胞瘤的诊断.     

纵隔生殖细胞肿瘤的MSCT诊断

目的:分析纵隔生殖细胞肿瘤MSCT的表现,提高对本病的诊断水平。方法:共64例,采用GE Lightspeed 16层CT进行胸部平扫及增强扫描。结果:53例畸胎瘤,11例恶性生殖细胞瘤。53例畸胎瘤CT显示都含有脂肪,位于前纵膈。11例恶性生殖细胞瘤,肿瘤直径范围为7.0~28.0cm,均偏于纵隔一侧生长,位于中上前纵隔,平扫密度较低,CT值约20~30HU,增强扫描呈斑片状、边缘轻度强化;与周围大血管分界不清,肿瘤具有钻隙生长的趋势,肿瘤内部多发囊变。结论:CT对纵隔生殖细胞肿瘤,具有一定的诊断及鉴别诊断价值。