Premium
Neurocutaneous Melanosis in Association with the Dandy–Walker Complex, Complicated by Melanoma: Report of a Case and Literature Review
Author(s) -
MenaCedillos Carlos Alfredo,
ValenciaHerrera Adriana M.,
ArroyoPineda Alma Iris,
SalgadoJiménez M. Angeles,
EspinozaMontero Rubén,
MartínezAvalos Armando Bernardo,
PeralesArroyo Antonio
Publication year - 2002
Publication title -
pediatric dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.542
H-Index - 73
eISSN - 1525-1470
pISSN - 0736-8046
DOI - 10.1046/j.1525-1470.2002.00073.x
Subject(s) - medicine , leptomeninges , fourth ventricle , hydrocephalus , cerebellar vermis , melanoma , hypoplasia , tentorium , pathology , anatomy , radiology , cerebellum , central nervous system , cancer research , endocrinology
Neurocutaneous melanosis is a rare congenital neurocutaneous syndrome in which benign and malignant melanocytic tumors of the leptomeninges with large or numerous congenital melanocytic nevi develop. The Dandy–Walker malformation occurs as a broad posterior fossa with high insertion of the tentorium, hypoplasia or aplasia of the cerebellar vermis, and cystic dilation of the fourth ventricle communicating with the posterior fossa. Association of these entities is very unusual and only 10 previous reports were found in the literature. Our patient had multiple, medium‐size to small melanocytic nevi present since birth. At 5 years of age the patient has intracranial pressure secondary to hydrocephalus. A diagnosis of Dandy–Walker malformation and suspected neurocutaneous melanosis was established after a skull computed tomography (CT) scan. Three months later the patient developed a right frontal tumor shown on the CT scan. The histologic finding was nevomelanocytic infiltration with strong pleomorphism. The tumor grew rapidly, producing neurogenic shock and death. The postmortem report indicated malignant melanoma.