Giant congenital melanocytic nevi: selected aspects of diagnostics and treatment.

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/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 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. 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 High urine dopamine concentration in GMN children, especially with NCM, is an unpublished finding that can indicate patients with more serious neurological disease.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.

Case reports of three patients showing optic nerve head melanocytoma and systemic hypertension.

PURPOSE: To report the association of optic disk melanocytoma with systemic hypertension. CASES: Ocular examination of 3 asymptomatic hypertensive patients revealed a pigmented lesion over the optic disk. After clinical examination and radiological evaluation a diagnosis of optic disk melanocytoma was made. One patient had a mild decrease in visual acuity associated with an increase in size of the lesion during the follow-up. Systemic evaluation revealed increased urinary vanillylmandelic acid in all the patients. MRI showed diffuse adrenal hyperplasia while a (131)I-MIBG scan showed increased uptake in 1 case. CONCLUSIONS: Optic disk melanocytoma is a benign tumor though the lesion may increase in size with the resultant decrease in visual acuity over few years. It may be associated with systemic hypertension secondary to increased levels of catecholamines in the body, which is related to the common neural crest origin of melanocytes, adrenal medullary cells and chromaffin cells.

[Malignant blue nevus]. / Naevus bleu malin.

Malignant blue naevi with an aggressive behaviour are rare tumours. To our knowledge, this is the first reported case who has developed generalized skin melanosis and melanuria in its terminal stage.

Melanoma specific protein: occurrence in the urine of patients with halo naevus and vitiligo.

Melanoma specific protein is immunologically related to altered naevus cell cytoplasm. It is excreted by patients with malignant melanoma but in no other malignancy. The protein has been detected in patients with actively developing halo naevi but not when repigmentation is taking place. It also occurs in patients with very active vitiligo but in no other pigment condition we have studied. It is suggested that the protein is a marker of active destruction of naevus cells by immune mechanisms and that the release of toxic materials during this immune reaction may be responsible for the production of the halo phenomenon and for the areas of vitiligo that may be seen elsewhere on the skin.