神经母细胞瘤术前化疗的病理观察

报告神经母细胞瘤术前化疗病例的临床病理观察，与术前未化疗病例比较，提示化疗使肿瘤细胞固缩、坏死、变性，肿瘤间质纤维组织增生，淋巴细胞浸润，包膜增厚，从而使肿瘤完全切除率从１４．３％提高到７０．０％，术后生存率和生存时间也显著提高和延长，病理改变与治疗效果呈正相关。从病理组织学的角度证实了术前化疗延期手术治疗神经母细胞瘤的可行性和临床价值。     

术前化疗对肾母细胞瘤瘤体的影响

目的　探讨术前化疗对肾母细胞瘤瘤体大小及瘤体耐药的影响。方法　本组 37例 ,术前影像学分期 ,Ⅰ～Ⅱ期 ,手术切除 ;Ⅲ～Ⅴ期 ,术前化疗 2～ 6周。免疫组化检测术后瘤体组织P 糖蛋白 (P glycoprotein ,P gp)、谷胱甘肽 硫转移酶 (glutathione s transferase π ,GST π)、拓扑异构酶Ⅱ(topoisomeraseⅡα ,TOPOⅡ )表达状况。结果　影像学分期 ,Ⅰ～Ⅱ期 2 0例 ,Ⅲ～Ⅴ期 17例。术前化疗组 ,瘤体缩减率 :1～ 2周为 (18.84± 3.5 4 ) %、3～ 4周为 (10 .78± 4 .2 6 ) %、5～ 6周为 (3.18±1.0 8) % ;瘤体GST π、TOPOⅡ表达阴性。瘤体在术前化疗 2周以内无P gp表达 ,4周以内有或弱阳性表达 ,4周以后P gp表达阳性。 结论　术前化疗应针对Ⅲ～Ⅴ期患儿 ,瘤体缩小主要集中于化疗 4周以内 ,超过 4周瘤体缩小不明显 ,且有引起肿瘤耐药的可能     

术前化疗在肾母细胞瘤中的应用

肾母细胞瘤是儿童时期最常见的肾脏实体恶性肿瘤,随着以手术为主的综合治疗的不断发展,肾母细胞瘤术后无瘤生存率有了显著提高,而术前化疗在其中起了举足轻重的作用。目前长春新碱、放线菌素D及表柔比星是术前化疗的基础药物。术前化疗有不同的给药途径及方法,各种方法又有不同的化疗方案,其使肿瘤缩小增加肿瘤全瘤切除率和预测肾母细胞瘤患者预后方面的作用得到了越来越广泛的认可。未来术前化疗的目标将是提高化疗有效性,降低化疗药物近期、远期毒性。     

术前化疗对小儿进展期神经母细胞瘤血管形成状态的影响及其临床意义

目的 探讨术前化疗对进展期（Ⅲ、Ⅳ期）小儿神经母细胞瘤血管形成状态的影响及其可能的作用机制和临床意义。方法 利用第Ⅷ因子相关抗原（Ｆ８－ＲＡ）抗体,应用免疫组化法检测小儿神经母细胞瘤中的微血管计数。结果 １５例进展期（Ⅲ、Ⅳ期）小儿神经母细胞瘤患儿中,术前化疗组９例,平均微血管数为２５．６３。非术前化疗组６例,平均微血管数为３９．００,明显高于术前化疗组（Ｐ〈０．０５）;术前化疗组患儿的平均生存时     

单眼眼内期视网膜母细胞瘤168例疗效及生存质量分析

背景 视网膜母细胞瘤(Rb)是儿童期最常见的恶性肿瘤,在90％Rb患儿所在的发展中国家,减少死亡仍是一个挑战,且患儿的生存质量较少受到关注.目的 总结单眼眼内期Rb患儿疗效、安全性及生存质量,为优化治疗方案和提高治疗安全性及有效性提供证据.设计回顾性非随机对照研究.方法 回顾性分析2009年11月6日至2019年9月5...     

术前化疗对进展期神经母细胞瘤儿童手术切除率及预后的影响

目的探讨术前化疗对进展期神经母细胞瘤（NB）患儿手术切除率及预后的影响。方法收集2000年1月－2006年12月本院收治的采取手术治疗的进展期（Ⅲ、Ⅳ期）NB患儿55例，均经病理确诊。男31例（56．4％），女24例（43．6％）；年龄54．1（4～147）个月。根据国际NB分期标准（INSS），本组病例Ⅲ期27例（49．1％），Ⅳ期28例（50．9％）。55例患儿均行手术治疗，其中45例患儿采取术前化疗，平均化疗5．3（1～14）个疗程，10例未采取术前化疗。结果随访44例，随访率为80％。生存18例，总体生存率为40．9％。化疗前肿瘤体积[B超：（524．9±480．7）cm^3，CT：（339．3±289．4）cm^3]；手术前肿瘤体积[B超：（195．9±309．8）cm^3，CT：（75．4±73．0）cm^3]，二者比较均有统计学差异（Pa〈0．05）；瘤体缩减率为（63．68±24．99）％。术前化疗组肿瘤切除率（包括完全切除和大部分切除）为88．9％，术前未化疗组肿瘤切除率为50％，2组比较有统计学差异（P〈0．05）。肿瘤切除者（包括完全和大部分切除）1a生存率为78．9％，2a生存率为63．9％；肿瘤未切除者（包括部分切除和探查活检）1a生存率为37．5％，2a生存率为18．8％，2组比较有统计学差异（Pa〈0．05）。结论术前化疗可以提高进展期NB患儿的手术切除率，进而改善预后。     

CTV化疗方案治疗视网膜母细胞瘤的不良反应及其防治

目的 观察CTV化疗方案治疗视网膜母细胞瘤的相关不良反应及其防治措施.方法 选取2008年1月至2008年12月临床确诊为视网膜母细胞瘤并化疗的患儿,共154例.采用CTV化疗方案(卡铂28 mg/kg、替尼泊苷8 mg/kg、长春新碱0.05 mg/kg),观察患儿化疗时及化疗后的不良反应.结果 未摘除眼球的眼内期患儿化疗后肿瘤明显缩小.化疗时及化疗后无任何不良反应者3例,占1.9%;有不良反应按WHO化疗不良反应分级为Ⅰ度者48例,占31.2%;Ⅱ度64例,占41.6%;Ⅲ度28例,占18.2%,Ⅳ度11例,占7.1%.不良反应主要为血液系统和消化系统症状、脱发,少数病例出现发热、过敏和周围神经症状;除脱发外,其余不良反应均恢复.结论 CTV化疗方案治疗视网膜母细胞瘤安全、有效.     

婴儿期视网膜母细胞瘤217例临床特点及预后分析

目的:探讨婴儿期视网膜母细胞瘤(RB)的临床特点及预后。方法:收集2009年7月1日至2019年6月30日首都医科大学附属北京同仁医院儿科收治的217例(患眼335只)婴儿期RB患儿的临床资料,分析其综合治疗的临床疗效及预后。结果:1．临床特点：217例婴儿期RB患者中,男129例,女88例,男女比例为1.47∶1.0...     

VEC方案治疗眼内期视网膜母细胞瘤的效果及安全性分析

目的探讨VEC方案治疗儿童眼内期视网膜母细胞瘤的效果并评价其安全性。方法对2006年1月至2008年2月间在复旦大学附属儿科医院血液肿瘤科门诊接受化疗的眼内期视网膜母细胞瘤患儿进行临床观察分析。结果共36例(48只眼),其中单侧24例,双侧12例,首次诊断平均年龄21.3月(2~93月),中位随访时间25.8月(15~36月)。12只眼IIRC分期为AC期,应用化疗结合局部治疗,1只眼(8.3%)经化疗仍发生进展,其余11只眼(91.7%)随访均持续缓解。36只眼IIRC分期为D~E期,应用眼球摘除术结合化疗,1例对侧眼出现新发病灶,1眼进展再次手术摘除眼球,其余34只眼(94.4%)随访均持续缓解。所有病例都能耐受化疗,仅1例(2.8%)发生轻度肝功能损害。结论化疗与局部治疗、手术相结合能对眼内期视网膜母细胞瘤进行有效的综合治疗。化疗的应用能避免外放疗的使用,降低眼球摘除术率,提高患儿生存质量,且安全可行,并发症少。     

眼外期及远处播散期视网膜母细胞瘤133例

目的 总结晚期视网膜母细胞瘤(Rb)患儿的临床特点和疗效.方法收集2005年9月-2010年5月本科收治133例(162只眼;男78例,女55例)眼外期(101例)及远处播散期(32例)Rb患儿综合诊疗资料,并进行回顾性分析.133例患儿中位年龄2.04岁(0.33～12.66岁).首发眼别:右眼48例(眼外期36例,远处播散期12例),左眼56例(眼外期46例,远处播散期10例),双眼29例.结果眼外期及远处播散期患儿初诊症状以黄白色反光为主要临床表现,占69.92%(93/133例),其次为流泪、红肿、畏光及眼球突出(10.52%),斜视(9.77%),视力下降、失明、白内障(6.77%).误诊1.51%,术后复发1.51%.133例中男性患儿就诊率为58%(78/133例),略高于女性患儿;存在Rb家族史者占5.26%(7/133例);98.49%的患儿(131/133例)神经元特异性烯醇化酶(NSE)增高, 最大值370.00 μg·L-1;眼外期患儿NSE水平为(34.16±19.63) μg·L-1,远处播散期患儿NSE水平为(91.83±96.55) μg·L-1.放弃治疗或失访14例(远处播散期9例,眼外期5例),获随访119例,随访中位时间27个月,生存率82.35%(98/119例).远处播散期存活率为26.08%(6/23例),眼外期为95.83%(92/96例).结论晚期Rb患儿一旦出现远处器官及组织侵犯,病死率较高.但眼外期患儿经规律治疗后生存率较高,应重视Rb的早期、综合诊疗.     

Experience with chemoreduction and focal therapy for intraocular retinoblastoma in a developing country

BACKGROUND: Chemoreduction is used for the treatment of retinoblastoma in industrialized nations; however, there are fewer data from developing countries. Before the implementation of this program, radiotherapy was used in almost all preserved eyes. METHODS: Retrospective evaluation from 1995 to 2001 at the Hospital Garrahan (Argentina). Carboplatin 18.7 mg/kg/day 1 and vincristine (0.05 mg/kg/day 1) were offered to patients with Reese-Ellsworth (RE) groups I-III and all unilateral cases. Etoposide (3.3 mg/kg/day 1 and 2) was added for groups IV and V. The number of cycles was tailored according to response. RESULTS: Fifty-eight patients (78 eyes) were evaluated (39 bilateral, 19 unilateral). With a median follow-up of 47 months, 40 patients had unilateral enucleation, 14 were not enucleated, and 4 had bilateral enucleation. Nineteen patients had unilateral initial enucleation. Eye preservation at 5 years was: RE groups I-III (n = 24 eyes), 0.9 (SE: 0.095) IV-V (n = 54), 0.45 (SE 0.07). Patients received a median of four cycles of chemotherapy. Acute toxicity was mild. External beam radiotherapy was avoided in 41% of eyes with groups I-III. Etoposide was avoided in 24 patients. Two patients died of metastasis. No secondary malignancy occurred. CONCLUSIONS: Compared to our previous experience, eye preservation was better and even though less radiotherapy was used, it was prescribed more often than currently recommended in eyes with less advanced disease because of limited availability of sophisticated local therapy.     

Giardiasis in a patient undergoing chemotherapy for retinoblastoma and acute myelogenous leukemia

Giardiasis is a common cause of diarrhea in undeveloped countries, but is very rare in developed countries. A patient with acute myelogenous leukemia and retinoblastoma presented with a high fever and severe watery diarrhea during induction chemotherapy. On microscopy, cysts were seen in her stool, suggesting Giardia intestinalis, which was confirmed on polymerase chain reaction (PCR). G. intestinalis was also detected in the patient's asymptomatic parents, who may have transmitted it to the patient. Giardiasis should be tested for in patients with severe and persistent diarrhea during chemotherapy, when other etiologies have been excluded. PCR used to amplify the DNA of G. intestinalis is rapid and sensitive.     

A phase II trial of carboplatin for intraocular retinoblastoma

BACKGROUND: Retinoblastoma patients with RB1 germline mutations are at risk of developing second malignancies and external beam radiation therapy increases the risk. Carboplatin-containing chemotherapy regimens in conjunction with local therapies have been investigated for intraocular retinoblastoma, but the lack of data regarding the efficacy of single agent intravenous carboplatin prompted this phase II study. PROCEDURE: Twenty-five patients (43 eyes) were treated with intravenous carboplatin (18.7 mg/kg for patients < 12 kg, 560 mg/m(2) for patients >/= 12 kg). Patients received a median of two cycles of carboplatin (range one to five cycles) beginning at a median age of 5 months (range 14 days to 22 months). RESULTS: All patients were extraocular disease free during the follow-up period (median 76.3 months). Responses were noted in 33 of 36 evaluable eyes (92%). The 5-year overall ocular and ocular event-free survivals were 93.3% (95% CI, 84.4-100%) and 43.5% (95% CI, 25.8-61.3%) for eyes treated for Reese-Ellsworth (RE) group 1-3 disease and 25.0% (95% CI, 1.0-50.0%) and 8.3% (95% CI, 0-24.0%) for RE group 4-5 disease, respectively. No non-hematopoietic serious or permanent toxicities related to the chemotherapy were observed. CONCLUSION: When used as a neoadjuvant agent, carboplatin usually leads to objective responses of intraocular retinoblastoma. The 5-year ocular event-free survival appears inferior to other protocols using more extensive chemotherapy, but with greater radiation therapy usage, overall ocular survival rate for RE group 1-3 eyes was excellent.