晚期视网膜母细胞瘤53例的临床挑战

目的：评价肿瘤的位置、大小、组织学以及复发率。 方法：前瞻性研究Larkana的Chandka医学院附属医院眼科的53例组织学诊断为视网膜母细胞瘤患者,肿瘤侵及眼球、眼眶、颅内以及其他组织。为研究肿瘤广泛性,检查包括：眼眶X线片,眼球B超,眼眶及头颅CT扫描／MRI,全骨扫描,胸部前后位X线片,腹部B超,腰椎穿刺,全血细胞计数,包括肝肾功能检查的血生化,根据肿瘤的位置、大小、复发及转移选择包括手术、局部放疗、系统化疗的治疗方案。有局部和系统转移的患者,由Larkana的核医学和放疗机构的肿瘤专家进行局部放疗和系统化疗。术后1,3,6,12mo复查随访,随后5a及5a以上,1a复查一次。评估肿瘤的位置、大小、组织类型和复发率。 结果：患者53例,年龄8mo～8岁。男25例（47％）,女28例（53％）。肿瘤的眼别,左眼25例（47％）,右眼19例（36％）,双眼9例（17％）。患者的首发症状,斜视3例（6％）,假性眼内炎4例（8％）,眼眶炎性假瘤6例（11％）,转移性包块7例（13％）,蕈样生长的包块8例（15％）,白瞳征12例（23％）,眼眶浸润13例（25％）。行单纯眼球摘除术19例（36％）,行眼球摘除及改良型眶内容物剜除术并局部放疗和系统化疗19例（36％）,行眼球摘除及完全型眶内容物剜除术并局部放疗和系统化疗15例（28％）。肿瘤的组织类型,含大量染色质的高分化视网膜母细胞瘤25例（47％）,高度未分化视网膜母细胞瘤28例（53％）。首次手术后,由于手术切除点以上视神经受累,眼眶浸润和系统转移,发生复发和转移37例（70％）。 结论：儿童红眼或白瞳征在确诊之前应怀疑视网膜母细胞瘤,应该及时经眼科专家检查,因为患者在肿瘤初期的早期表现提示会降低肿瘤的扩散、复发和死亡率。     

全身化疗联合局部化疗治疗视网膜母细胞瘤眼眶侵犯

目的 探讨全身化疗联合局部化疗对视网膜母细胞瘤(Rb)眼眶侵犯患者的临床效果.方法 从2009年2月到2011年1月,中山大学中山眼科中心诊断的17例17只眼影像学显示明显Rb眼眶侵犯患者,接受CEV方案(卡铂、依托泊甙和长春新碱)全身化疗联合卡铂眶周注射治疗.其中12例(原发眼眶侵犯9例,继发眼眶侵犯3例)诊断后立即联合化疗;其余5例(原发眼眶视神经侵犯)先行眼球摘除术再用联合化疗.结果 所有患者对术前化疗有明显反应.9例原发眼眶侵犯的患者中,8例在联合化疗后3个疗程行眼球摘除术,7例术后病理回报肿瘤坏死或限于球内,1例回报视神经断端肿瘤浸润;另1例仅联合化疗后6个月复发视神经侵犯,行眼球摘除术病理显示视神经断端肿瘤浸润.3例继发眼眶侵犯的患者对联合化疗有完全反应.随访时间平均12.5个月(7～23个月).7例因中枢神经系统转移死亡.13个月的生存率为48.5％.结论 采用全身化疗联合眼局部化疗对眼眶视网膜母细胞瘤是有效的,有可能替代眶内容剜除术和眼外放疗.     

鼻腔鼻窦鳞癌侵犯眼眶时眼球的处理

目的探讨鼻腔鼻窦鳞状细胞癌侵犯眼眶时保留或剜除眼球对生存率和复发率的影响.方法回顾性分析1989年1月～1998年2月我院收治的43例侵犯眶骨壁和眶骨膜的鼻腔、鼻窦鳞状细胞癌患者的临床和随访资料,其中25例剜除眼球,18例保留眼球.比较患者的3年生存率和复发率.结果随访38～126个月,43例患者中,眼球剜除组3年生存率为56%(14/25),局部复发率为36%(9/25),保留眼球组3年生存率为55.5%(10/18),局部复发率为38.8%(7/18),其中1例为眶内复发.结论经术前或术后放疗,对侵犯眶骨壁和眶骨膜的鼻腔、鼻窦鳞癌,保留眼球组与剜除眼球组相比:有相似的局部复发率和生存率,但保留眼球提高了术后生活质量.     

眶内容摘除术41例临床分析

本文对我院1973～1989年作眶内容摘除的41例患者进行回顾性临床分析,发现作眶内容摘除术的原因,成人最多是眼睑泪腺肿瘤,儿童主要是视网膜母细胞瘤。其中9例系肿瘤复发。一期植皮效果好,成功率高。视网膜母细胞瘤如有眼眶组织或视神经残端侵犯,即使作眶内容剜除,再加放疗也难以存活。成人眶内容剜除,辅以放疗,部份患者可以长期存活,不应轻易放弃手术。     

局部治疗加化疗治疗眼内视网膜母细胞瘤

目的探讨局部治疗加化疗治疗眼内视网膜母细胞瘤的效果。方法对11例眼内视网膜母细胞瘤病人使用联合化疗(长春新碱、卡铂、环磷酰胺、VP 16)加局部治疗。有4例在3个方案的化疗后行眼球摘除术。结果停药后平均随访9.4月,有2例双眼视网膜母细胞瘤复发,其余患眼随访中无新病灶或对侧眼无病灶。结论对于眼内期视网膜母细胞瘤病人使用化疗加局部治疗是有效的,化疗可能有预防肿瘤转移的作用。     

视网膜母细胞瘤化疗进展

视网膜母细胞瘤是婴幼儿的一种先天性眼内恶性肿瘤,其治疗手段有化疗、眼球摘除术、外放疗、光凝治疗、冷冻治疗、内放射敷贴术.近年来化疗作为一种很有前途的疗法而受到重视.在视网膜母细胞瘤的眼内阶段,人们通过化疗来缩小肿瘤体积,以便于进一步进行局部治疗,化疗亦能预防眼内肿瘤转移及预防三侧性视网膜母细胞瘤.但单用化疗不能治愈视网膜母细胞瘤.化疗具有副作用,主要为骨髓抑制.     

良性病变行眼眶内容剜除术37例临床分析

目的分析良性病变行眶内容剜除术的适应证。设计回顾性病例系列。研究对象1999年11月至2010年8月武警总医院眼眶病研究所行眶内容剜除术的良性病变患者37例。方法回顾性分析患者的病例资料。主要指标眶内容剜除术原因、术式,病理诊断。结果全眶内容剜除术35例（94．6％）,后部眶内容剜除术2例（5．4％）。全眶内容剜除术联合放射性^131I粒子植入术1例（2．7％）。病理诊断为炎性假瘤11例（29．7％）,多形性腺瘤8例（21．6％）,脑膜瘤6例（16．2％）,韦格纳肉芽肿性病变4例（10．8％）,血管外皮细胞瘤2例（5．4％）,副神经节瘤、血管瘤、神经纤维瘤、纤维脂肪血管瘤、婴儿色素性神经外胚层肿瘤、眼眶结核各1例（2．7％）。眶内容剜除术原因：肿瘤切除术后复发或多次复发占86．5％,术眼视力无光感占54．1％,不可控制的疼痛占43．2％,肿瘤大或范围广占40．5％,长期用糖皮质激素或放疗但病情仍进展占27．0％。结论对于眼眶良性病变因局部切除术后复发而困扰的患者,如伴有视力丧失,肿瘤大或范围广,不可控制的疼痛者,可行眶内容剜除术。该术式用于眼眶良性病变治疗时,可彻底清除病灶、最大限度降低复发率。     

22例眼眶转移癌临床病理分析

目的探讨眼眶转移癌的临床特征和原发部位。设计回顾性病例系列。研究对象22例眼眶转移癌患者。方法分析1999～2007年武警总医院眼眶病研究所22例眼眶转移癌患者的临床、影像学和病理诊断资料。主要指标临床表现、影像学表现、病理形态学表现、原发部位。结果22例眼眶转移性肿瘤中原发肿瘤来自乳腺6例(27.3%),肺癌5例(22.7%),来源未明低分化腺癌3例(13.6%),肝癌2例(9.1%),甲状腺癌、宫颈癌、直肠类癌、下肢恶性黑色素瘤、腹膜后恶性副神经节细胞瘤及神经母细胞瘤各1例。女性13例,男性9例,平均年龄50.2岁(中位数年龄51岁,范围6～67岁)。病变位于右眼眶11例,左眼眶5例,双侧眼眶1例,同侧眼内加眶内转移2例,眶颅沟通3例。最常见的症状为视力下降、其次是眼球运动不良、眼球突出、眼痛和眼睑肿胀。10例(45.5%)眼部表现是首发症状,3例(13.6%)未找到原发灶。结论眼眶转移性肿瘤来自乳腺占第一位,其次是肺癌和来源不明的低分化腺癌。部分病例眼部症状为首发症状。(眼科,2007,16:403-406)     

视网膜母细胞瘤-发展中国家治疗中的挑战

目的:回顾发展中国家在治疗视网膜母细胞瘤中的挑战。方法:回顾1998-01/2002-12,埃努古的尼日利亚大学教学医院和Ebrans临床中心的视网膜母细胞瘤患者的临床及组织病理学资料。结果:总共34例通过组织学被确诊为视网膜母细胞瘤患者。男25例(74%),女9例(26%),男女比例为1∶0.4。平均年龄2.9±1.5岁(5mo～7岁)。6例(18%)患者为双眼发病,28例(82%)为单眼。所予以的治疗为眼内容物剜除术或眼球摘除术联合化疗。结论:在发展中国家,由于患者未及时就诊、贫穷、医疗资源匮乏等因素,视网膜母细胞瘤的治疗现状很差。改善其在发展中国家的治疗,应包括健康教育、早期诊断及提供现代化治疗设施。     

正电子发射体层摄影-CT检查对眼眶眼球恶性肿瘤的诊断价值

目的 探讨正电子发射体层摄影-X线电子计算机体层摄影（positron emission tomography-computer tomography,PET-CT）检查对眼眶及眼球恶性肿瘤的诊断价值.方法 对眼眶及眼球恶性肿瘤33例（41眼）.病程1月～5年,行18氟-2-氟-2-脱氧-D-葡萄糖（18F-2-fluoro-2-deoxy-D-glucose,18F-FDG） PET-CT肿瘤代谢全身体层融合显像.结果 术后病理学证实眼眶眼球恶性肿瘤33例,其中淋巴瘤16例,脉络膜黑色素瘤7例,视网膜母细胞瘤4例,眼眶转移瘤6例.17例在眼眶或眼球局部或在其外的皮肤、淋巴结、骨骼、内脏等组织器官有18F-FDG高代谢灶,明确提示为眼眶或眼球恶性肿瘤或转移瘤,3例未能给予明确提示,结合临床进一步检查确诊.13例提示术后或化疗后改变,其中4例可见18F-FDG放射性浓集灶,提示恶性肿瘤术后残留、复发或转移,9例未见18F-FDG浓聚,提示治疗效果良好.结论 PET-CT肿瘤全身断层融合显像通过人体组织的分子功能及CT复合影像,对眼眶及眼球肿瘤的良恶性质鉴别有重要参考价值;对肿瘤术后有无残留、复发和疗效监测有重要临床意义.     

Vitrectomy in eyes with unsuspected retinoblastoma

OBJECTIVE: To analyze patient management and prognosis after vitrectomy in eyes with unsuspected retinoblastoma. DESIGN: Retrospective, noncomparative case series. PARTICIPANTS: Eleven consecutive patients who had undergone vitrectomy on an eye with unsuspected retinoblastoma. MAIN OUTCOME MEASURES: The two main outcome measures were ultimate patient management and the development of retinoblastoma metastasis. RESULTS: Of more than 900 consecutive patients with retinoblastoma managed on the Ocular Oncology Service at Wills Eye Hospital in Philadelphia, 11 (1%) had prior vitrectomy in an eye with viable tumor before referral to us for suspected retinoblastoma. The main preoperative diagnoses included vitreous hemorrhage in seven patients (64%), toxocariasis in two patients (18%), toxoplasmosis in one patient (9%), and endophthalmitis in one patient (9%). In no case was retinoblastoma suspected before vitrectomy. The mean patient age at vitrectomy was 6 years. Retinoblastoma was later suspected during vitrectomy in two patients (18%), on cytologic examination of the vitrectomy specimen in eight patients (73%), and after referral in one patient (9%). The mean interval between vitrectomy and referral to us was 23 days. On examination, the globe was classified as Reese-Ellsworth group Vb in all 11 patients (100%). Anterior chamber tumor cells were clinically visible in four eyes (36%), hyphema in two eyes (18%), and iris neovascularization in two eyes (18%). Retinoblastoma cells were visualized in the vitreous in seven eyes (64%) and not visualized in four eyes (36%) that had vitreous blood. Enucleation was necessary in all 11 patients (100%). Adjuvant treatment was delivered in 10 patients (91%), using orbital radiotherapy in nine patients (82%) and chemotherapy in nine patients (82%). Histopathologic evidence of retinoblastoma invasion was documented in the episclera (two eyes; 18%), anterior chamber (seven eyes; 64%), iris (five eyes; 45%), ciliary body (five eyes; 45%), choroid (three eyes; 27%), and optic nerve (four eyes; 36%; prelaminar, two eyes; postlaminar, two eyes). The vitrectomy ports, Tenon's fascia, cut end of the optic nerve, and orbit were free of tumor. Of the 10 patients who received prophylactic chemotherapy, radiotherapy, or both in addition to enucleation for prevention of retinoblastoma metastasis, none (0%) experienced metastasis or orbital recurrence during the mean follow-up of 7 years (range, 0.2-24 years) from the time of retinoblastoma diagnosis. However, one patient was referred to us after the development of metastatic retinoblastoma, and despite aggressive chemotherapy and radiotherapy after enucleation, died 24 months later. CONCLUSIONS: Retinoblastoma may present with atypical features such as vitreous hemorrhage or signs of vitreous inflammation, particularly in older children. Vitrectomy should be avoided in these cases until the possibility of underlying retinoblastoma is excluded. If vitrectomy is performed in an eye with unsuspected retinoblastoma, enucleation combined with adjuvant chemotherapy, radiotherapy, or both without delay is advised to prevent systemic tumor dissemination.     

Continuing challenges in the management of retinoblastoma with chemotherapy

The management of retinoblastoma has gradually changed over the past 10 years. Over 95% of children with retinoblastoma in the United States are cured with modern techniques. The challenge remains, however, in maintaining the eye and vision. There is a trend away from enucleation and external beam radiotherapy toward focal conservative treatments involving primary chemoreduction in conjunction with thermotherapy and cryotherapy. This is related to earlier detection of the disease, recognition of more effective chemotherapeutic agents, more focused local treatment modalities, and, most importantly, knowledge of the long-term risks of external beam radiotherapy. Enucleation is still preferable for retinoblastoma that fills most of the eye, especially when the disease is unilateral or when there is concern for tumor invasion into the optic nerve, choroid, or orbit. The orbital integrated implant is placed after enucleation and provides acceptable prosthesis motility and appearance. External beam radiotherapy is still vital for treating advanced retinoblastoma, especially when there is diffuse vitreous or subretinal seeding after failure of other methods and preservation of vision is a priority. The most important recent advance in the management of retinoblastoma is the use of intravenous chemotherapy for tumor reduction, a technique of neoadjuvant chemotherapy termed "chemoreduction." This is followed by tumor consolidation with focal measures such as thermotherapy, cryotherapy, and plaque radiotherapy. This strategy provides reduced tumor volume and often permits consolidation with methods other than radiotherapy. It appears that vision can be preserved in some cases with these methods, avoiding some of the local complications like radiation cataract or macular edema that have been found with radiotherapy techniques. External beam radiotherapy and enucleation can now be avoided in most cases of Reese-Ellsworth groups I (minimal disease) through IV (moderate disease) retinoblastoma. The most advanced stage of retinoblastoma, Reese-Ellsworth group V, continues to provide the greatest difficulty for management, and external beam radiotherapy and enucleation are often employed in addition to chemoreduction to save the child's life. A collaborative prospective study in North America is currently under way to further study the benefits and risks of chemoreduction for minimal, moderate, and advanced retinoblastoma.     

Current treatment of retinoblastoma

Chemotherapy has recently achieved a major role in the primary management of intraocular retinoblastoma. Tumor reduction by first-line chemotherapy (chemoreduction) followed by local treatments is now accepted as treatment strategy for intraocular retinoblastoma with the goal of avoiding external beam radiotherapy (EBRT) or enucleation. Although efficient in reducing tumor volume, chemotherapy cannot cure retinoblastoma. Different chemoreduction protocols are used to shrink the tumor, making it treatable with cryotherapy, laser photocoagulation, thermotherapy, and plaque radiotherapy. Systemic chemotherapy used with local ophthalmic therapies during or after the chemotherapy can eliminate the need for enucleation or external beam radiotherapy in Reese-Ellsworth group 1, 2, or 3 retinoblastoma. This combination is not sufficient to obtain tumor control in most eyes with large tumors and diffuse vitreous and subretinal seeds (Reese-Ellsworth group 4 and 5 tumors), and EBRT or enucleation is eventually required. The resultant visual acuity after globe-conserving therapies in those eyes with Reese-Ellsworth group 4 and 5 tumors is often poor. Preliminary results of a phase I/II study of subconjunctival carboplatin injection are encouraging. Enucleation is still recommended in situations such as eyes containing large tumors, long standing retinal detachment, neovascular glaucoma, pars plana tumor seeding, anterior chamber involvement or choroid, optic nerve or orbital tumor extension, and no expectation for useful vision. Chemoprophylaxis is necessary for patients with tumor extending to the surgical margin of the optic nerve and is likely beneficial in preventing metastases in patients with tumor extending beyond the lamina cribrosa. Intensified chemotherapy with autologous stem cell rescue appears effective for patients with metastatic retinoblastoma.     

Orbital malignant fibrous histiocytoma following irradiation for retinoblastoma

PURPOSE: An unusual case is reported of orbital malignant fibrous histiocytoma that developed after irradiation for retinoblastoma. METHODS: Case report. RESULTS: A 5-month-old girl underwent enucleation of the left eye, external beam irradiation of the right eye, and systemic chemotherapy for bilateral sporadic retinoblastoma. At age 17 years, a malignant fibrous histiocytoma developed in the medial aspect of the orbit and nasal cavity. After attempted excisional biopsy, she underwent craniofacial resection, orbital exenteration, maxillectomy, and tracheostomy. The patient was alive 12 months postoperatively without local or metastatic disease. CONCLUSIONS: Although orbital fibrous histiocytoma occurs usually as a primary tumor of adulthood, it can also develop as a secondary tumor after irradiation for retinoblastoma.     

视网膜母细胞瘤眼球摘除并放疗后Ⅱ期义眼座植入的临床观察

目的观察RB眼球摘除并放疗后Ⅱ期义眼座植入的临床效果。方法回顾分析27例RB眼球摘除并放疗后义眼座Ⅱ期植入的临床资料,并观察其疗效。结果27例患者术后眼窝塌陷和义眼运动都得以明显改善和恢复,24眼动度较好,3眼动度较差。追踪随访1月￣5年,无1例复发、感染;2例义眼座暴露,4例上睑板沟轻度凹陷,1例上睑下垂。结论RB眼球摘除并放疗后义眼座Ⅱ期植入可获得良好效果,应尽可能早期手术,并注意手术方法与技巧。     

Local extraocular extension of retinoblastoma following intraocular surgery.

Three cases of local extraocular extension of retinoblastoma following intraocular surgery via a transscleral approach have been seen in one centre during a 12-month period. This number represents a significant increase on the previous incidence of this serious complication, which may be related to more widespread use of vitrectomy techniques. Orbital and local lymph node recurrence of retinoblastoma have been associated with a very poor survival rate in the past, and steps must be taken to limit the chance of this happening should an intraocular surgical procedure be performed inadvertently on an eye containing a retinoblastoma. Early enucleation without implant is advised, and adjuvant orbital radiotherapy is strongly recommended, even when there is no histopathological evidence of local extraocular tumour spread.     

Malignant fibrous histiocytoma in a patient with history of treated retinoblastoma

Retinoblastoma is one of the most important childhood tumors with genetic implications and necessity for family counseling and long term follow up. We are presenting a 22 year-old Saudi male with history of bilateral retinoblastoma, who was initially treated by enucleation of his left eye. The histopathology showed a moderately differentiated tumor with vitreous seeding and he received chemotherapy in addition to radiotherapy to his right eye. He presented 20 years later with a right orbital tumor, frozen globe and proptosis. The excisional biopsy of his orbital mass revealed a spindle cell sarcoma with features of malignant fibrous histiocytoma. His clinical and radiological findings are presented together with the histopathologic features of his orbital tumor and brief review of secondary tumors in retinoblastoma patients.     

Recurrence of peripheral primitive neuroectodermal tumor of the orbit with systemic metastases

CASE REPORT: A six-year-old boy presented with proptosis of the right eye. Imaging studies detected a mass in the medial wall of the right orbit. This mass was biopsied revealing a histopathologic diagnosis of primitive neuroectodermal tumor, so chemotherapy treatment was given. After seven years in remission he presented with a recurrence of the orbital tumor and was found to also have systemic metastases. Treatment with chemotherapy, radiotherapy and orbital exenteration was unsuccessful. DISCUSSION: The orbital occurrence of these tumors is extremely rare. Differentiation from other small round cell tumors requires immunohistochemical and ultrastructural techniques.     

Chemoreduction plus focal therapy for retinoblastoma: factors predictive of need for treatment with external beam radiotherapy or enucleation

PURPOSE: To report the results of chemoreduction and focal therapy for retinoblastoma with determination of factors predictive of the need for treatment with external beam radiotherapy or enucleation. DESIGN: Interventional case series. METHODS: One-hundred three patients with retinoblastoma (158 eyes with 364 tumors) at the Ocular Oncology Service at Wills Eye Hospital of Thomas Jefferson University in conjunction with the Division of Oncology at Children's Hospital of Philadelphia from June 1994 to August 1999 were enrolled for this prospective clinical trial. The patients received treatment for retinoblastoma with six planned cycles (one cycle per month) of chemoreduction using vincristine, etoposide, and carboplatin combined with focal treatments (cryotherapy, thermotherapy, or plaque radiotherapy). The two main outcome measures after chemoreduction and focal therapy were the need for external beam radiotherapy and the need for enucleation. The clinical features at the time of patient presentation were analyzed for impact on the main outcome measures using a series of Cox proportional hazards regressions. RESULTS: Using Reese-Ellsworth (RE) staging for retinoblastoma, there were nine (6%) eyes with group I disease, 26 (16%) eyes with group II disease, 16 (10%) eyes with group III disease, 32 (20%) eyes with group IV disease, and 75 (48%) eyes with group V retinoblastoma. All eyes showed initial favorable response with tumor regression. The median follow-up was 28 months (range, 2-63 months). Failure of chemoreduction and need for treatment with external beam radiotherapy occurred in 25% of eyes at 1 year, 27% at 3 years, and no further increase at 5 years. More specifically, external beam radiotherapy was necessary at 5 years in 10% of RE groups I-IV eyes and 47% of RE group V eyes. Multivariate factors predictive of treatment with external beam radiotherapy included non-Caucasian race, male sex, and RE group V disease. Failure of chemoreduction and the need for treatment with enucleation occurred in 13% eyes at 1 year, 29% at 3 years, and 34% at 5 years. More specifically, enucleation was necessary in 15% of RE groups I-IV eyes at 5 years and in 53% of RE group V at 5 years. Multivariate factors predictive of treatment with enucleation included patient age older than 12 months, single tumor in eye, and tumor proximity to foveola within 2 mm. Overall, of the 158 eyes, 50% required external beam radiotherapy or enucleation and 50% were successfully managed without these treatments. No patient developed retinoblastoma metastasis, pinealoblastoma, or second malignant neoplasms over the 5-year follow up. CONCLUSIONS: Chemoreduction offers satisfactory retinoblastoma control for RE groups I-IV eyes, with treatment failure necessitating additional external beam radiotherapy in only 10% of eyes and enucleation in 15% of eyes at 5-year follow-up. Patients with RE group V eyes require external beam radiotherapy in 47% and enucleation in 53% at 5 years.