目的总结分析原发性眼眶恶性黑色素瘤的临床特征,并与国外已报道病例进行比较,使眼科医生加深对该罕见肿瘤的理解和认识。设计回顾性病例系列。研究对象2008年在北京同仁医院就诊的3例原发性眼眶恶性黑色素瘤患者。方法回顾性分析上述3例患者的临床表现、影像学特点、组织病理学特征以及治疗和随诊情况,通过复习国外相关文献,比较我国患者与国外患者在临床特征方面的异同。主要指标临床表现、磁共振成像(MRI)、病理学特征。结果 3例眼眶黑色素瘤患者中,主要临床症状均为不同程度且伴随不同症状的眼球突出。经过详细的全身检查,未发现原发于其他部位的黑色素瘤,排除转移性黑色素瘤的可能;眼睑、颜面部皮肤、结膜、巩膜等均无黑色素性病变,排除了先天性黒变病的病理基础。MRI检查显示:2例肿瘤均表现为短T1短T2信号,强化不明显,符合黑色素瘤的典型特征;1例肿瘤表现为主体呈短TI长T2不强化区,依据通常经验未能提示黑色素瘤。组织病理学检查显示:3例肿瘤均为恶性黑色素瘤,分别为上皮样细胞型、梭形细胞型、混合细胞型。其中1例肿瘤发生于眶内囊肿内壁的上皮组织,具有明确的囊膜上皮基础,据此推测,胚胎时期从神经嵴移行到囊壁上皮细胞间的黑色素细胞可能是原发性眶内黑色素瘤的组织学来源。治疗均采用单纯肿瘤切除术,术后1例患者未行任何辅助性放化疗,另2例患者分别行4个周期和7个周期化疗。随访40～48个月,3例患者均健在,无肿瘤复发和转移迹象。结论 MRI检查对于原发性眼眶恶性黑色素瘤术前诊断具有一定提示作用。附着于眼眶内囊肿上皮细胞间的黑色素细胞可能是该肿瘤的起源。由于肿瘤具有包膜,单纯肿瘤切除术治疗有效。 Objective To summarize and analyze the clinical features of primary orbital malignant melanoma and compare it with those reported in foreign countries so that ophthalmologists can deepen the understanding and understanding of this rare tumor. Design retrospective case series. Participants Three primary orbital malignant melanoma patients were treated in Beijing Tongren Hospital in 2008. Methods The clinical manifestations, imaging features, histopathological features, treatment and follow-up of the above three cases were retrospectively analyzed. The similarities and differences in clinical features between Chinese patients and foreign patients were compared by reviewing relevant literature from abroad. The main indicators of clinical manifestations, magnetic resonance imaging (MRI), pathological features. Results Among the 3 cases of orbital melanoma, the main clinical symptoms were different degree and the eyeballs with different symptoms were prominent. After a detailed examination of the whole body, no melanoma was found in other parts of the body to rule out the possibility of metastatic melanoma. Eyelid, facial skin, conjunctiva, sclera and so on had no melanin lesions, which excluded the pathology of congenital black degeneration basis. MRI examination showed that both of the 2 tumors showed short T1 short T2 signal, which was not obviously enhanced, which was in line with the typical features of melanoma. One of the tumors showed a short TI and T2 non-enhancement area, and no evidence of melanoma . Histopathological examination showed that all three tumors were malignant melanoma, which were epithelioid cell type, spindle cell type and mixed cell type, respectively. One of the tumors occurred in the epithelial tissue of the inner wall of the orbital cyst, with a clear basis of the epithelium of the capsular epithelium. Therefore, it is speculated that melanocytes migrating from the neural crest to the cyst wall epithelial cells during the embryonic period may be primary orbital melanoma The source of histology. All patients underwent simple tumor resection. One patient did not receive adjuvant radiotherapy and chemotherapy after operation, and the other two patients received 4 cycles and 7 cycles of chemotherapy respectively. All patients were followed up for 40 ~ 48 months. All the 3 patients were alive without tumor recurrence and metastasis. Conclusion MRI examination can be helpful for the preoperative diagnosis of primary orbital malignant melanoma. Melanocytes attached to the intercalated orbital epithelial cells may be the origin of this tumor. As the tumor has a capsule, simple tumor resection is effective.