晚期视网膜母细胞瘤53例的临床挑战(英文)

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。

Too late presentation of 53 patients with retinoblastoma:a big challenge

AIM:To evaluate the site, size,histology,and recurrence of the tumor.METHODS: We prospectively evaluated 53 patients with histological diagnosis of retinoblastoma involving the eye, orbit, intra cranial cavity and other tissues, at Department of Ophthalmology, Chandka Medical College Hospital, Larkana. To assess the extent of the tumor investigations, including X-ray-orbits, ocular B-scan, CT-scan/MRI-Oorbits-brain, whole body bone scanning, X-ray chest PA view, ultrasound abdomen, lumbar punc-ture, complete blood cell count, blood chemistry as-sessment, including renal and hepatic function para-meters, were performed. The selection of mode of treatment like surgical procedures, local radiotherapy and systemic chemotherapy used depend on the site, size, recurrence and metastasis of the tumor. The patients with local and systemic metastases were referred to the oncologist at the Larkana Institute of the nuclear medicine and radiotherapy for local radiation and systemic chemotherapy. Follow up examinations were carried out at 1, 3 , 6 and 12 months after the operation, and then annually for a further 5 years or longer. The tumor's site, size, histological type, and recurrence were evaluated.RESULTS: Of these 53 patients the age range was from 8 months to 8 years. 25 (47%) were male and 28 (53%) were female. The laterality of the tumor was in left eye 25 (47%) cases, right eye 19 (36%) cases, and both eyes 9 (17%) cases. The presentation of the patient at 1st visit was strabismus 3 (6%) cases, pseudohypopyon 4 (8%) cases, orbital pseudo orbital inflammation 6 cases (11%), mass with metastases 7 (13%) cases, fungating mass 8 (15%) cases, leu-kocoria 12(23%) cases and orbital invasion 13(25%) cases. The surgical procedures performed were enucleation alone in 19 (36%) cases, enucleation with modified exentration followed by local radiotherapy and systemic chemotherapy in 19 (36%) cases and enucleation with total exentration followed by local radiotherapy and systemic chemotherapy in 15 (28%) cases. The histological types of the tumor were well- differentiated retinoblastoma with abundant rosettes in 25 (47%) cases and highly undifferentiated retino- blastoma 28 (53%) cases . After primary surgery, the recu-rrences and metastases of the tumors were noticed in 37 (70%) cases due to optic nerve involvement beyond the point of surgical transaction, orbital invasion and sy-stemic metastasis.CONCLUSION: Any child with red eye or white pupillary reflex should be suspected of having retinoblastoma, until proved otherwise, and should be promptly referred to an ophthalmologist, because early presentation of patient in the initial stage of the tumor will result in decreased risk of tumor extension, recurrence and mortality.