CLINICOPATHOLOGIC STUDY OF 56 CASES OF INTRA-OCULAR MEDULLO-EPITHELIOMAS

CLINICOPATHOLOGIC STUDY OF 56 CASES OF INTRA-OCULAR MEDULLO-EPITHELIOMAS
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DOI:
10.1016/s0002-9394(14)77739-6
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发表时间:
1978-01-01
影响因子:
4.2
通讯作者:
ZIMMERMAN, LE
ZIMMERMAN, LE
中科院分区:
医学1区
文献类型:
--
作者:
BROUGHTON, WL;ZIMMERMAN, LE

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在眼科病理学登记处对56例眼内髓上皮瘤的临床病理和随访研究显示,确诊时的平均年龄为5岁,大多数患者在出现体征或症状后手术治疗延迟了1年以上。在37个肿瘤中观察到恶性的组织学证据,随访研究发现4例患者死于转移性疾病。在4例良性肿瘤和17例恶性肿瘤中观察到异形成分(脑组织、软骨或横纹肌母细胞)。这21例被指定为畸胎样髓上皮瘤。最重要的预后特征是眼外伸展,观察到10例。所有4例已知的肿瘤死亡均发生在临床上明显的眼眶扩散之前。另有4例眼眶受累患者失访。预后比本研究结果所显示的更差,尤其是那些观察到有眼外侵犯的病例。
A clinicopathologic and follow-up study of 56 intraocular medulloepitheliomas in the Registry of Ophthalmic Pathology revealed a mean age of 5 yr at the time of definitive diagnosis, most patients having had a delay in surgical therapy of more than 1 yr after onset of signs or symptoms. Histologic evidence of malignancy was observed in 37 tumors, and follow-up studies revealed that 4 patients died of metastatic disease. Heteroplastic elements (brain tissue, cartilage or rhabdomyoblasts) were observed in 4 benign and 17 malignant tumors. These 21 were designated teratoid medulloepitheliomas. The most important prognostic feature was extraocular extension, observed in 10 cases. All 4 of the known tumor deaths were preceded by clinically obvious orbital spread. In 4 additional cases with orbital involvement, the patients were lost to follow-up. The prognosis is worse than the results of this study indicate, especially among those cases observed to have extraocular extension.