Sawicka Ewa, Szczygielski Orest, Żak Klaudia, Pęczkowski Paweł, Michalak Elżbieta, Bekiesińska-Figatowska Monika
Clinic of Surgery of Children and Adolescents, Institute of Mother and Child, Warsaw, Poland.
Department of Diagnostic Imaging, Institute of Mother and Child, Warsaw, Poland.
Med Sci Monit. 2015 Jan 11;21:123-32. doi: 10.12659/MSM.891279.
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/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.
Nine children had "bathing trunk" nevus, 7 had main nevus on the back, 6 on head/neck, 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.
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 High urine dopamine concentration in GMN children, especially with NCM, is an unpublished finding that can indicate patients with more serious neurological disease.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.
巨大黑素细胞痣(GMN)的治疗仍然是一个多学科挑战。我们对GMN患儿的诊断、治疗及随访情况进行分析,以确定这些患者的必要诊疗程序。
材料/方法:对24例GMN患儿,我们分析了:痣的部位、主要痣直径、卫星痣、脑部磁共振成像(MRI)、24小时尿儿茶酚胺浓度、手术分期数量及组织学检查。采用t检验比较各亚组患儿的儿茶酚胺浓度。
9例患儿有“躯干”痣,7例主要痣位于背部,6例位于头颈部,2例位于颈部/肩部及颈部/胸部。脑部MRI显示7/24例(29.2%)患儿有神经皮肤黑素沉着症(NCM),其中1例有症状。在20例患者(33份样本)的尿儿茶酚胺水平中,28/33例多巴胺浓度升高,15例去甲肾上腺素升高,11例肾上腺素升高,4例香草扁桃酸升高。6例NCM患儿的所有儿茶酚胺浓度均高于无NCM的患者(无统计学意义)。所有患者切除痣的组织学检查均显示为复合痣,15例有神经纤维瘤成分,2例有黑色素瘤成分。在8年和3年的随访中,他们均无复发/转移。7例NCM患者中有4例进行了1次以上的随访MRI检查;其中1例发现新的黑色素沉着,3例无进展。
早期切除并进行组织学检查可加速黑色素瘤的诊断。脑部MRI对于确诊/排除NCM是必要的。GMN患儿,尤其是合并NCM的患儿,尿多巴胺浓度升高是一项尚未发表的发现,可提示患有更严重神经系统疾病的患者。GMN患儿的治疗应根据每个病例的所有医学/心理方面进行个体化定制。