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Choroidal melanoma in a 7-year-old child treated by trans-scleral local resection.

机译:经巩膜局部切除术治疗的7岁儿童脉络膜黑色素瘤。

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PURPOSE: To report a choroidal melanoma in a 7-year-old child treated by trans-scleral local resection and adjuvant brachytherapy with a family history of neurofibromatosis type I (NF1) and cutaneous melanoma. PATIENT AND METHODS: A 7-year-old child was referred for treatment of a choroidal tumor in her left eye with a differential diagnosis of melanoma, neurilemmoma, leiomyoma, and neurofibroma. Trans-scleral local resection and, subsequently, adjuvant brachytherapy were performed. RESULTS: Histopathology and immunohistochemistry of the specimen diagnosed an amelanotic melanoma of spindle cell type, with a moderately high number of mitoses (7/40 HPF). Multiplex ligation-dependent probe amplification (MLPA) analysis showed two copies of chromosome 3, three copies of the short arm of chromosome 6, and two copies of chromosome 8, strongly suggesting a good prognosis. Postoperative ophthalmic evaluation at 6 months showed no visible tumor and flat retina with visual acuity (VA) of 6/60. CONCLUSIONS: Trans-scleral local resection with adjuvant brachytherapy in children is possible using the same techniques as for adults. Although the follow-up is short, our patient retained the eye with good vision and our cytogenetic studies allowed us to reassure the mother.
机译:目的:报道一名7岁儿童经脉巩膜局部切除术和辅助近距离放射疗法治疗的脉络膜黑色素瘤,该家族史有I型神经纤维瘤病(NF1)和皮肤黑色素瘤。患者和方法:一名7岁的儿童被转介治疗左眼的脉络膜肿瘤,并有黑色素瘤,神经鞘瘤,平滑肌瘤和神经纤维瘤的鉴别诊断。经巩膜局部切除术,然后进行辅助近距离放射治疗。结果:标本的组织病理学和免疫组织化学诊断为梭形细胞型黑色素瘤黑色素瘤,有中等数量的有丝分裂(7/40 HPF)。多重结扎依赖性探针扩增(MLPA)分析显示两个拷贝的3号染色体,三个拷贝的6号短臂和两个拷贝的8号染色体,强烈提示预后良好。术后6个月的眼科评估显示无可见肿瘤和视网膜平坦,视力(VA)为6/60。结论:采用与成人相同的技术,可以在儿童中进行巩膜近端切除联合辅助近距离放射治疗。尽管随访时间很短,但我们的患者眼睛保持了良好的视力,并且我们的细胞遗传学研究使我们可以放心地照顾母亲。

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