视网膜母细胞瘤—发展中国家治疗中的挑战
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首席医学网
2009年04月08日 16:32:29 Wednesday
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作者：E N Onwasigwe I R Ezegwui2 C N Onwasigwe3 G Ilechukwu4 A E Aghaji1 作者单位：尼日利亚埃努古1尼日利亚大学教学医院眼科;2埃努古州科技大学教学医院眼科;3尼日利亚大学教学医院社区医学系;4尼日利亚大学教学医院儿科
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【摘要】 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。
【关键词】 发展中国家 治疗 视网膜母细胞瘤
INTRODUCTION
Oculoorbital tumors are rare and there are few composite studies of these tumors.Most reported series of the frequency of orbital tumors are based on factors such as a specialy interest of the reviewer, whether only histopathologically confirmed lesions were included, the range of patients age, the geographic area of the patients, and other factors[1]. Reviews that analyze orbital tumor incidence from an ophthalmology practice show a different array of tumors than seen in a neurosurgery or otolaryngology practice[24]. It is important to obtain information about different aspects of orbital tumors to determine the appropriate treatment strategy in order to prevent postoperative visual impairment. Information about the type of tumor is very important when the treatment strategy is detecting[5]. The aim of this study is awareness of demographic, type of oculoorbital tumors based on patients age, location of the tumor, pathological report and follow up studies.
SUBJECTS AND METHODS
Conducted based on retrospective, observational case series study from 1997 to 2007. Two hundred cases are verified oculoorbital tumors in our institution from 1997 to 2007. The oculoorbital excisional biopsy specimen from operation room of Shahid Sadughi and Rahahan Hospital, sent to pathology department. The pathology reports were selected from profiles and data collected in special questionnaire .
All patient who had oculoorbital tumors included in this study and categorized to two age groups of under and over 18 years old.
RESULTS
We studied the relationships between the pathological reports of 200 cases of oculoorbital tumors, their locations and the age of patients at diagnosis time. The sex distribution revealed 111(55.5 %) males versus 89(44.5%) females.The orbital tumors, which were mainly divided into three types, including benign, Malignant and Metastatic tumors of the eye and adnexa are gathered in this study. 110 cases (55.0%) were benign, 86 (43.0%) malignant, and 4 (2.0%) metastatic tumors. Nevus (15.0%), dermoid cyst (5.5%) and hemangioma (5.0%) were the most common benign tumors. There were 90 cases (45.0%) of malignant and metastatic tumors, including BCC 51(25.5%), SCC 12(6.0%) malignant melanoma 11 (5.5%), and sebaceous cell carcinomas 5(2.5%) etc. All of 4(2.0%) metastatic tumors that were reported were metastatic melanoma. The age distribution of patients at diagnosis were recorded.We categorized the tumors by age of under 18 years old and the over 18 years old . In the under 18 years old group (37 cases), the most common tumors were benign (90.1%), and malignant and metastatic tumors were 8.4% and 1.5% respectively,while in patients over 18years (163 cases), the most common tumors were malignant (51.1%), benign tumors (47.1%) and metastatic tumors (1.8%).The relationships between pathological findings and location of the tumors:119 tumors (59.5%) were eyelid tumors, 21 (10.5%) were orbital and 60 cases (30.0%) were in globe.The most common tumors in all three oculoorbital areas are different in childhood and adulthood patients. Eyelid tumors(63.5%) are the most frequent tumors of older patients based on location while the globe tumor(43.0%) were the most frequent tumors in childhood.
DISCUSSION
The pathologic profiles of oculoadnexal tumors in our study were characterized according to patients age and location of the tumor.
The age of cases is an important factor affecting relative incidence of oculoadnexa tumors. In this study,the frequency of orbital tumors was high in adulthood group ,which is similar to Shield[1] study wheras in Japan, in Ohtsuka[5] study two peaks were reported in the age distribution. The incidence of orbital tumors was high in children aged 0 to 9 years and in older patients aged 60 to 69 years. Also Uddin[6] reported the same age distribution of patients with orbital tumors . Based on sex distribution,our results show 111(55.5%) males and 89(44.5%) females that were similar to those of other studies in CongoKinshasa 99(60%) male and 65(40%) female[7].
In this series of 200 tumor cases, 110(55%) were benign, 90(45%) were malignant and metastatic, these results are similar to Roh[8] report, that were 159 cases (63.6%) of benign tumors and 91 cases (36.4%) of malignant tumors and comparable with result of Haloń[9] study, in which 2031 cases, 62.5%(1262) were benign and against the Uddin[6] study and Ni C[10] report that was seen. The 5 leading benign orbital tumors, cavernous hemangioma (304 cases), benign mixed tumor (109 cases), inflammatory pseudotumor of the orbit (101 cases), dermoid cyst (100 cases), and optic meningioma (65 cases).
In this study the most common tumors in childhood were benign (90.1%) but, Lipowski[11] belived with increasing age, malignant orbital tumors become more common. VdDin & Roh[6,8] study also found the same age distribution of patients with orbital tumors.
Between the benign tumors nevus(15.0%),dermoid cyst (5.5%) and hemangioma (5.0%) were the most frequent, this is similar to UdDin[6] study, (33%). In Roh[8] report, in Korea, the most frequent benign tumor of the eye and its adnexa was nevus .In relation with frequency of eyelid benign tumors,Welch & Duke[12] report the most frequent tumor was papilloma.
The frequency of malignant tumor in this study are comparable to other studies. The most common tumors in this series were BCC 51 (25.5%), SCC 12(6.0%) and melanoma 11(5.5%), sebaceous carcinoma 5(2.5%). These finding are in agreement with Ohtsuka[5] report and were different from studies, such as Haioń[9] study that were described, epidermoid carcinoma and retinoblastoma, the most common histologic forms, representing respectively 33.5% and 31.7% of all malignant tumors of the eye and adnexa and Roh[8] clarified that retinoblastoma is more common in Singaporeian patients and in Scat Y[13] study reported melanoma.
CONCLUSION
The pathologic profiles of oculoadnexal tumors were characterized according to patients age and location of the tumor and shows us the relative frequency of benign, malignant and metastatic tumor distribution in eyelid ,orbit and globe.
【参考文献】
1 Shields JA, Shields CL, Scartozzi R. Survey of 1264 patients with orbital tumors and simulating lesions: the 2002 Montgomery Lecture, part 1. Ophthalmology 2004;111(5):9971008
2 Iliff CE. Tumors of the orbit. Trans Am Ophthalmol Soc 1958;55:505541
3 Kennedy RE. An evaluation of 820 orbital cases. Trans Am Ophthalmol
Soc 1984;82:134157
4 Henderson JW, Campbell RJ, Farrow GM, Garrity JA. The tumor survey. In: Henderson JW, Campbell RJ, Farrow GM, Garrity JA, eds.Orbital Tumors. 3rd ed. New York: Raven Press; 1994:4352
5 Ohtsuka K, Hashimoto M, Suzuki Y. A review of 244 orbital tumors in Japanese patients during a 21year period: Origins and Locations. Jpn J
Ophthalmol 2005;49(1):4955
6 UdDin N, Mushtaq S, Mamoon N, Khan AH, Malik IA. Morphological spectrum of ophthalmic tumors in northern Pakistan. J Pak
Med Assoc 2001;51(1):1922
7 Poso MY, Mwanza JC, Kayembe DL. Malignant tumors of the eye and adnexa in CongoKinshasa. J Fr Ophthalmol 2000;23(4):327332
8 Roh KK, Lee JH, Youn DH. Clinical Analysis of Tumors of the Eye and its Adnexa Korean. J Ophthalmol 1988;2(1):2731
9 Haioń A, Biazejewska M, Sabri H, Rabczyński J. Tumors and tumorlike lesions of eyelids collected at Department of Pathological Anatomy, Wroclaw Medical University, between 1946 and 1999. Kin Oczna 2005;107(79):475 478
10 Ni C, Ma X. Histopathologic classification of 1921 orbital tumors. Yan Ke Xue Bao 1995;11(2):101104
11 Lipowski P, Raczyńska K, Murawska J, IwaszkiewiczBilikiewicz B. Orbital tumors in the material of Department of Ophthalmology of Medical University of Gdańsk in years 19912002. Klin Oczna 2004;106(3 Suppl):460 462
12 Welch RB,Duke JR.Lesions of the lids;statistical note. Am J Ophthalmol 1958;45(3):415 426
13 Scat Y, Liotet S, Carre F. Epidemiological study of 1705 malignant tumors of the eye and adnexa. J Fr Ophthalmol 1996;19(2):8388
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