宋某某，女，7岁
Miss. Song, 7 years old, Female
一、病例特点
General Information
主诉
右眼黑色素细胞瘤切除术后一年半，眼球渐进性突出2个月。
Chief Complaint
The parents reported their child’s right eye gradually protopsis for 2 month and post-operative of right eye melanocytoma for one and a half years.
病史
现病史：患者1年半前因“右眼结膜肿物”于外院行右眼结膜肿物切除术，术后病理诊断为：右结膜黑色素细胞瘤，术后病情稳定。半年前右眼睑肿瘤复发，遂再次于该院行肿瘤切除，术后病理诊断仍为“黑色素细胞瘤”。2个月前无明显诱因出现右眼球突出，随时间推移渐进性加重，伴右眼流泪、视力下降、眼红、眼痛，为求进一步诊治来我院，门诊以右眼眶占位病变收入院。
眼病史：患儿自出生右眼周皮肤发黑。
个人史、家族史无异常。
Medical History
Present history: One and a half years ago, the patient underwent conjunctival neoplasm resection of right eye in other hospital. The pathology diagnosis was melanocytoma. The tumor recurred six months ago and was removed. The pathology diagnosis still was melanocytoma. The exophthalmos of right eye appeared 2 months ago without inducement, and the symptom aggravated gradually. At the meantime, the right eye presented with tearing, blurred vision, redness and ophthalmalgia. So the child was admitted to the hospital for further treatment options.
Past medical historyThe child was born with black skin around her right eye.
Personal and family history is unremarkable.
眼部检查
右眼：远视力0.05，矫正无提高，眼压T+1，眼球突出，眼突度24- 94-14 mm，黑色素沉着，范围全部上下眼睑。眶压（+++），眼球运动各方向均受限，提上睑肌肌力4mm，眼睑闭合可，眶下方可触及中等硬度肿物，边界不清，不可推动，颞侧球结膜高度水肿，部分突出于睑裂，突出睑裂球结膜出现糜烂，结膜可见色素沉着，鼻侧结膜瘢痕，角膜上皮粗糙，前房深，虹膜纹理清，瞳孔圆，对光反射存，晶状体透明，眼底：视乳头边清色可，后极部球壁隆起，视网膜皱褶，黄斑区中心凹反光未见，视网膜在位。
头颈部淋巴结及胸腹部检查均未见异常。
左眼：远视力1.0，眼压13.5mmHg，眼内未见异常。
Ocular examination
Right eye: VA was 0.05, which can not be corrected. IOP was T+1 by finger touch. The eye presented with exophthalmus (24- 94-14 mm), eyelid skin melanin pigmentation.High intraorbital pressure (+++),limitation of ocular movements. Myodynamia of levator palpabrae muscle was 4mm. Normal catamysis. There was a palpable medium hardness tumor at the infraborbital area. The tumor was stable and it’s boundary was not clear. Severe temporal conjunctiva chemosis drove part of the conjunctiva prolapse, erosion and pigmentation. There was scar on nasal conjunctiva. Rough corneal epithelium , deep anterior chamber, normal pupil, transparent crystalline lens can be found. Retinal protuberance and retina fold were seen in the fundus.
Cervical lymph nodes, thoracic and abdominal examination showed no abnormalities.
Left eye: BCVA was 1.0. IOP was 13.5mmHg
There is no abnormality in left eye.
二、辅助检查
Accessory Examinations
MRI示：右眼眶占位性病变，T1WI中低信号，T2WI中高信号。
彩色多普勒示：右眼眶占位性病变，右眼球受压明显，可见丰富的血流信号。
MRI suggested right orbit was space occupying lesion, showed low signal intensities on T1WI and high singal intensitiesT2WI.
Color Doppler showed space occupying lesion in right orbit. The eyeball was displaced significantly. Tumor showed rich blood flow signal.
三、初步诊断
Impression
右眼眶、结膜占位病变
右眼睑分裂痣
Space occupying lesion in right conjunctiva and orbit.
Divided nevus in right eyelid.
四、住院治疗经过
Treatment and outcome
于2012年8月17日在全麻下行右眼眶深部肿物切除+眼窝再造术，术后病理结果回示：右眶黑色素瘤、侵及骨骼肌及结膜，右眼睑皮内痣。
All lesions were treated by simple excision and combined with eye socket reconstructionunder general anesthesia. The pathology diagnosis was intradermal nevus of right eye lid and malignant melanoma of right orbit, which had invaded skeletal muscle and conjunctiva.
五、查房记录
Discussion
刘凤副主任医师：右眶黑色素瘤为恶性肿瘤，眼眶内容物剜除手术指征明确，为减少肿瘤转移机会，应尽快手术。
Dr Liu Feng: It is well known that melanoma is malignant tumor. So tumor resection is necessary. Since the tumor recurred twice, I recommenden to perform orbital exenteration as soon as possible in order to decrease the opportunity of tumor metastasis.
刘淑英副主任医师：患儿右眼肿瘤病理已证实为黑色素瘤，眶内容物剜除术手术指征明确。但考虑到恶变的可能性，眼睑皮内痣是否需同时行手术切除，需大家进一步讨论。
Dr. Liu Shu-Ying:I agreed with Dr.Liu. But whether the intradermal nevus of right eyelid would be removed or not, we should made further discussion.
何彦津主任医师：患儿右眼肿瘤为黑色素瘤，眶内容物剜除术大家均无异议，对于是否保留眼睑，我认为目前眼睑病理检查为皮内痣，恶变的可能性极小，若切除眼睑，需行植皮，由于患儿年龄小，取皮范围有限，可能无法满足植皮要求，因此术中应尽量保留眼睑。术前向患儿家长详细说明病情，以取得患儿家长理解。术中注意止血，避免大出血及医源性脑脊液漏。
Doctor He Yan-Jin: The management of conjunctival melanoma varies with the extent of the lesion. When lesions extend into the orbit, orbital exenteration was required. About the intradermal nevus, in my opinion, it is not necessary to remove it, because malignant transformation barely happened in this type of nevus.Furthermore, the patient has to accept skin graft if we remove the eyelid tumor. But the patient was too young to get enough skin graft. Therefore, I suggestedpreserving the eyelid skin. Talk to the parents with a detailed description of the condition so that obtain the parents understand. We should avoid hemorrhage and iatrogenic cerebrospinal fluid leakage during the operation.
六、治疗经过
Treatment and outcome
遂于2012年8月28日在全麻下行右眼眶内容物剜除术。术后病理结果回报：结膜复合痣；结膜、眶内黑色素瘤；眼睑皮内痣。术后病情稳定，治愈出院。3周后计划行化疗治疗。
In August 28, 2012, the patient underwent right orbital exenteration under general anesthesia. The postoperative pathology diagnosis was conjunctival compound nevus, conjunctiva and orbit melanoma and intradermal nevus of right eyelid. Postoperative physical conditions of the patient were stable. Chemotherapy will be administrated three weeks later.
七、病例相关知识
Relative knowledge
结膜恶性黑色素瘤是一种潜在致死性肿瘤，其总死亡率大约为25%。结膜黑色素瘤主要是起源于结膜原发性获得性黑变病或色素痣恶变，大部分为上皮样细胞型黑色素瘤，伴有邻近结膜上皮内侵犯。典型结膜黑色素瘤首先转移到下颌下淋巴结和腮腺淋巴结。预后与肿瘤的位置，是否侵犯眼眶或巩膜，上皮内全层弥漫以及是否波及睑缘皮肤相关。手术治疗首选，但术后复发比较常见，尤其肿瘤范围较大者。
一项研究发现，手术操作治疗可能更易致肿瘤扩散转移并最终死亡。无色素的黑色素瘤经常被误诊。这在临床上很难做出正确诊断，确诊需组织病理学检查。因此，提高黑色素瘤的无色素类型的认识对早期诊断和治疗至关重要。
Conjunctival malignant melanoma is a potentially deadly tumor. The overall mortality rate from conjunctival melanoma is about 25%. Conjunctival melanoma is mainly originated from the conjunctival primary acquired melanosis or nevus, mostly belong to epithelioid cell type, present with adjacent conjunctival epithelial invasion. Typically, metastases first develop in submandibular and parotid lymph nodes. Unfavorable prognostic factors are conjunctival site, orbital or scleral invasion, full-thickness intraepithelial spread, and involvement of the eyelid skin margin. Once diagnosed, operation is the first choice, but the postoperative recurrence is common, especially in larger scope of tumor.
One research found surgical technique of tumor management maybe possibly related to tumor metastases and death. Amelanotic malignant melanoma is often associated with a poorer prognosis. The lack of pigmentation makes clinical diagnosis virtually impossible, and diagnosis can only be established histopathologically. Awareness of this nonpigmented variety of melanoma is crucial for early recognition and appropriate management.