Peshtani Ani, Kaliki Swathi, Eagle Ralph C, Shields Carol L
Department of Ocular Oncology, Wills Eye Institute, Thomas Jefferson University, 840 Walnut Street, 14 Floor, Philadelphia PA, 19107.
Department of Pathology, Wills Eye Institute, Thomas Jefferson University, 840 Walnut Street, 14 Floor, Philadelphia PA, 19107.
Oman J Ophthalmol. 2014 May;7(2):93-5. doi: 10.4103/0974-620X.137171.
Intraocular medulloepithelioma arises from the primitive medullary epithelium and is diagnosed at a median age of five years. This tumor most commonly appears as a white, gray, or yellow-colored ciliary body tumor. The growth of medulloepithelioma is slow and it is locally invasive. Poor vision and pain are the most common presenting symptoms. The most common clinical signs include cyst or mass in iris, anterior chamber or ciliary body, glaucoma, and cataract.
A 22-month-old Caucasian female twin presented with leukocoria and poor vision in OS. Examination revealed normal findings OD and a mass in OS. Based on the clinical features of leukocoria, lens changes and a white cystic ciliary body mass in a young child, ultrasonographic, and transillumination features, the lesion was diagnosed as a non-pigmented ciliary epithelial medulloepithelioma. After enucleation, the diagnosis of malignant teratoid medulloepithelioma of the non-pigmented ciliary epithelium was confirmed. There was no evidence of tumor recurrence or systemic metastasis at three years follow-up.
Medulloepithelioma in a child can present as a clinical triad of leukocoria, lens changes, and a white cystic ciliary body mass.
眼内髓上皮瘤起源于原始髓上皮，中位诊断年龄为5岁。该肿瘤最常表现为白色、灰色或黄色的睫状体肿瘤。髓上皮瘤生长缓慢且具有局部侵袭性。视力下降和疼痛是最常见的就诊症状。最常见的临床体征包括虹膜、前房或睫状体的囊肿或肿块、青光眼和白内障。
一名22个月大的白种女性双胞胎，左眼出现白瞳症和视力下降。检查发现右眼正常，左眼有肿块。根据幼儿白瞳症、晶状体改变以及白色囊性睫状体肿块的临床特征、超声检查和透照特征，该病变被诊断为无色素睫状上皮髓上皮瘤。眼球摘除术后，确诊为无色素睫状上皮恶性畸胎样髓上皮瘤。随访三年，无肿瘤复发或全身转移的证据。
儿童髓上皮瘤可表现为白瞳症、晶状体改变和白色囊性睫状体肿块的临床三联征。
