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结膜局限性外周原始神经外胚层肿瘤(PNET)

Localised peripheral primitive neuroectodermal tumour (PNET) of the conjunctiva.

作者信息

Moore Andrew S, Wilson Peter G, McKelvie Penny, La Nauze Jamie, Hirst Lawrence W

机构信息

Section of Paediatric Oncology, The Institute of Cancer Research & Royal Marsden Hospital, Sutton, Surrey, UK.

出版信息

Pediatr Blood Cancer. 2009 Oct;53(4):669-71. doi: 10.1002/pbc.22062.

Abstract

A 16-year-old male presented with a 3-month history of an asymptomatic, enlarging conjunctival lesion. An excisional biopsy was performed and histologic and immunohistochemical examination showed characteristic features of a peripheral primitive neuroectodermal tumour (PNET) adjacent to a benign compound naevus. FISH analysis, demonstrating a split-signal at 22q12, confirmed the diagnosis. Staging investigations were negative confirming the primary nature of the lesion. The patient was treated with local wide re-excision and chemotherapy. He remains alive and well 29 months after initial resection.

摘要

一名16岁男性患者,结膜病变无症状且不断增大,病程3个月。进行了切除活检,组织学和免疫组化检查显示为与良性复合痣相邻的外周原始神经外胚层肿瘤(PNET)的特征性表现。荧光原位杂交(FISH)分析显示22q12处有分裂信号,确诊为此病。分期检查结果为阴性,证实病变为原发性。患者接受了局部广泛再次切除和化疗。初次切除后29个月,他仍然健在且情况良好。

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