眼科 ›› 2012, Vol. 21 ›› Issue (3): 210-214.

• 论著 • 上一篇    下一篇

原发性眼眶恶性黑色素瘤的临床特征(附3例报告)

顼晓琳 葛心 史季桐 李彬   

  1. 100005 首都医科大学附属北京同仁医院 北京同仁眼科中心 北京市眼科研究所
  • 收稿日期:2012-02-10 出版日期:2012-05-25 发布日期:2012-05-31
  • 通讯作者: 史季桐, Email: shijt168@163.com E-mail:shijt168@163.com

Three cases with primary orbital malignant melanoma

XU Xiao-lin, GE Xin, SHI Ji-tong, LI Bin   

  1. Beijing Institute of Ophthalmology, Beijing Tongren Eye Centre, Beijing Tongren Hospital, Capital Medical University, Beijing 100005, China
  • Received:2012-02-10 Online:2012-05-25 Published:2012-05-31
  • Contact: SHI Ji-tong, Email: shijt168@163.com E-mail:shijt168@163.com

摘要: 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。

关键词: 黑色素瘤, 眼眶, 磁共振扫描, 病理学

Abstract: Objective To describe characteristic presentation of rare primary orbital malignant melanoma in Chinese, and analyze the differences between Chinese cases and Caucasian cases. Design Retrospective case series. Participants Three cases with primary orbital melanoma under the care of the Oncology Clinic at Beijing Tongren Hospital. Methods Retrospective review of clinical presentation, treatment, radiology and pathology characteristics for the three cases. Main Outcome Measures Clinical manifestation, MRI appearances, and pathology characteristics. Results Thorough physical examination of all the three patients demonstrated the absence of cutaneous or mucous melanoma, excluding the possibility of metastatic melanoma. No pigmentary disorders were visible on face, eyelid, conjunctiva, and sclera, excluding the pathologic basis of congenital melanosis. MRI of two cases was consistent with the typical characteristics of melanoma, but MRI of one case didn’t suggest melanoma. Pathologic examination revealed the pathologic type of the three cases, which was spindle cell, epithelial cell and mixed cell melanoma, respectively. One case originated from the inner wall of cyst, which is a definite cystic epithelial basis, so it could be presumed that the melanocytes migrated from the neural crest and adherent to the cystic wall epithelial cells might be the origination of orbital melanoma. All the three cases were treated by primary resection. After surgery, two cases accepted systemic chemotherapy and one patient did not. There has been no recurrence or metastasis in 40 to 48 months follow-up. Conclusion MRI may provide some clues for pre-operation diagnosis of primary orbital malignant melanoma. The melanocytes adherent to the inner wall of cysts may be the origination of the tumor. These encapsulated tumors may be treatable by primary resection.

Key words: melanoma, orbital, MRI, pathology