Primary intracranial melanoma in a child with a giant congenital melanocytic naevus and normal MRI

Abstract

We present a case of a 10-year-old girl with a giant congenital melanocytic naevus, and malignant cerebral melanoma.

Congenital Melanocytic Nevus: A Case Study and Review of the Literature

“…Kadorega and Friedento defined coexistence of a large melanocytic nevus or of multiple ones (>3) with meningeal melanocytosis or melanoma, without signs of cutaneous melanoma [1]. MRO scan of the brain and the spinal marrow is advised in giant CMN and >20 satellite nevi, as these individuals have greater probability to have NCM [1,4,5,7,8]. Individuals with giant CMN and PAS >40 with large and giant CMN on the back, on the neck or on the head are in increased danger for NCM even in absence of melanoma, inspite of the fact that many symptomatic patients survive with various neurological defects [6].…”

“…Mild to severe developmental retardation and non-normal muscular tone have been described. In bibliography there is a report of a primary intracranial melanoma in child with a giant CMN and a normal MRI [4]. The cutaneous melanoma tends to penetrate deep in the skin or in the fat tissue of the LCMN, rendering the early diagnosis difficult.…”

“…The cutaneous melanoma tends to penetrate deep in the skin or in the fat tissue of the LCMN, rendering the early diagnosis difficult. MRI brain is advisable in multiple CMN or one CMN with more than 20 satellite nevi [1,4,7]. Also, every patient with newly appearing neurological symptoms, like urination, tip-toe walking, epilepsy and sunset sign must undergo neurological evaluation and suitable imaging.…”

“…These lesions can be present at birth and/or may proliferate significantly during the first years of life. Their number must be recorded, because a number > 20 is connected to neurological abnormalities [1][2][3][4].…”

“…Inspite of their great rarity, their diagnosis is important, as they have predisposition for development of dermal or extra-dermal melanoma [1,2,3]. Every patient with a giant CNM should be considered a suspect for development of neurodermal melanosis regardless of the fact that he may or may not present neurological symptoms and MRI brain and the spinal marrow is advisable before the myelination of the brain [1,4,5]. The management of each case must be individualized.…”