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Giant Congenital Melanocytic Nevi: Selected Aspects of Diagnostics and Treatment

机译:巨型先天性黑素细胞痣:诊断和治疗的某些方面

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Background Treatment of giant melanocytic nevi (GMN) remains a multidisciplinary challenge. We present analysis of diagnostics, treatment, and follow- up in children with GMN to establish obligatory procedures in these patients. Material and Methods In 24 children with GMN, we analyzed: localization, main nevus diameter, satellite nevi, brain MRI, catecholamines concentrations in 24-h urine collection, surgery stages number, and histological examinations. The t test was used to compare catecholamines concentrations in patient subgroups. Results Nine children had “bathing trunk” nevus, 7 had main nevus on the back, 6 on headeck, and 2 on neck/shoulder and neck/thorax. Brain MRI revealed neurocutaneous melanosis (NCM) in 7/24 children (29.2%), symptomatic in 1. Among urine catecholamines levels from 20 patients (33 samples), dopamine concentration was elevated in 28/33, noradrenaline in 15, adrenaline in 11, and vanillylmandelic acid in 4. In 6 NCM children, all catecholamines concentrations were higher than in patients without NCM (statistically insignificant). In all patients, histological examination of excised nevi revealed compound nevus, with neurofibromatic component in 15 and melanoma in 2. They remain without recurrence/metastases at 8- and 3-year-follow-up. There were 4/7 NCM patients with more than 1 follow-up MRI; in 1 a new melanin deposit was found and in 3 there was no progression. Conclusions Early excision with histological examination speeds the diagnosis of melanoma. Brain MRI is necessary to confirm/rule-out NCM. High urine dopamine concentration in GMN children, especially with NCM, is an unpublished finding that can indicate patients with more serious neurological disease. Treatment of GMN children should be tailored individually for each case with respect to all medical/psychological aspects.
机译:背景巨黑素细胞痣(GMN)的治疗仍然是多学科挑战。我们对GMN儿童进行诊断,治疗和随访分析,以建立这些患者的强制性程序。材料和方法在24例GMN儿童中,我们分析了:定位,主要痣直径,卫星痣,脑MRI,24小时尿液中儿茶酚胺浓度,手术分期和组织学检查。 t检验用于比较患者亚组中的儿茶酚胺浓度。结果9例儿童有“沐浴干”痣,7个患儿的主要痣位于背部,6个患儿的头部/颈部,2个患儿的颈部/肩部和颈部/胸部。脑MRI显示7/24名儿童(29.2%)有症状的神经皮肤黑素病(NCM),其中1名有症状。在20名患者(33个样本)的尿儿茶酚胺水平中,多巴胺浓度升高了28/33,去甲肾上腺素升高了15,肾上腺素升高了11在4名NCM儿童中,所有儿茶酚胺浓度均高于无NCM的患者(统计上无统计学意义)。在所有患者中,切除的痣的组织学检查均显示复合痣,其中15例有神经纤维蛋白成分,2例有黑色素瘤。随访8年和3年,它们均无复发/转移。 NCM患者中有4/7例接受了1次以上的MRI检查;在1处发现了新的黑色素沉积,在3处没有进展。结论早期切除和组织学检查可加快黑色素瘤的诊断速度。必须进行脑部MRI检查以确认/排除NCM。 GMN儿童,特别是患有NCM的儿童,尿液中的多巴胺浓度很高,这是尚未发表的发现,可以表明患者患有更严重的神经系统疾病。 GMN儿童的治疗应针对每种情况在所有医学/心理方面进行量身定制。

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