Neurocutaneous melanosis associated with intraspinal lipoma

Abstract

A case of neurocutaneous melanosis developing a leptomeningeal melanoma is described in a ten year old girl in whom an intraspinal lipoma had been resected seven years earlier. Standard and CT myelographic findings are described and the complementary role of Gadolinium-enhanced MRI is discussed. The literature is reviewed and the association of a lipoma and neurocutaneous melanosis has not been encountered.

Neurocutaneous Melanosis Associated with Dandy-Walker Malformation

“…Other CNS or systemic abnormalities such as midline vertebral column defects, intraspinal lipomas, arachnoid cysts, intracranial mass and syringomyelia may be associated with neurocutaneous melanosis [1, 2, 3, 4, 5, 15, 34, 35]. The coexistence of these developmental abnormalities supports the pathogenesis discussed above rather than foraminal obstruction as an underlying mechanism.…”

“…CNS involvement commonly manifests as increased intracranial pressure, seizures and infrequently cranial nerve palsies. Bowel and bladder dysfunction may also be present in the later stages of the disease [1, 4, 6, 7, 35]. The neurological manifestations are usually progressive and eventually fatal.…”

Neurocutaneous melanosis: radiological–pathological correlation

“…This dysplasia occasionally may be associated with other neurocutaneous syndromes as NF1 or Sturge-Weber syndrome [1,8]. Neurocutaneous melanosis has also been described in association with intracerebral melanoma [1,9,10], intraspinal lesions (lipoma of the spinal cord, subarachnoid CSF collections due to leptomeningeal infiltration) [6,11] and Dandy-Walker complex (DWC) [12]. It is proposed that in cases of concurrent NCM and DWC, the leptomeningeal melanosis interferes with the normal inductive effects of primitive meningeal cells on deposition of extracellular matrix, neuronal cell migration and formation of 1351 Fig.…”

“…Both melanocytes and leptomeninges derive from the neural crest. That is why the melanotic cells are normally present in the pia matter and could be at the origin of the genesis of leptomeningeal melanosis [1,6,7,8]. This dysplasia occasionally may be associated with other neurocutaneous syndromes as NF1 or Sturge-Weber syndrome [1,8].…”

Melanose neurocutânea. Relato de caso com melanoma maligno do sistema nervoso central

“…Encontra-se, também, associado à MNC: divertículo de Meckel 5 , anormalidades do trato urinário, como cistos renais e neoplasias; lipoma intra-espinhal 6 , meningioma e cisto aracnóide melanótico intra-espinhal 7 e hipoplasia do vermis inferior 8 . A infecção das meninges, assim como do trato respiratório, são comuns e fatais.…”