J Korean Neurosurg Soc.  2014 Jul;56(1):61-65. 10.3340/jkns.2014.56.1.61.

Neurocutaneous Melanosis in Association with Dandy-Walker Complex with Extensive Intracerebral and Spinal Cord Involvement

• Sung KS
• Song YJ

• KMID: 2067080
• DOI: http://doi.org/10.3340/jkns.2014.56.1.61

Abstract

Neurocutaneous melanosis (NCM) is a rare congenital syndrome consisting of benign or malignant melanotic tumors of the central nervous system with large or numerous cutaneous melanocytic nevi. The Dandy-Walker complex (DWC) is characterized by an enlarged posterior fossa with high insertion of the tentorium, hypoplasia or aplasia of the cerebellar vermis, and cystic dilatation of the fourth ventricle. These each two conditions are rare, but NCM associated with DWC is even more rare. Most patients of NCM with DWC present neurological symptoms early in life such as intracranial hemorrhage, hydrocephalus, and malignant transformation of the melanocytes. We report a 14-year-old male patient who was finally diagnosed as NCM in association with DWC with extensive intracerebral and spinal cord involvement.

Keyword

Melanoma; Neurocutaneous syndrome; Dandy-Walker syndrome

MeSH Terms

Adolescent
Central Nervous System
Dandy-Walker Syndrome*
Dilatation
Fourth Ventricle
Humans
Hydrocephalus
Intracranial Hemorrhages
Male
Melanocytes
Melanoma
Melanosis*
Neurocutaneous Syndromes
Nevus, Pigmented
Spinal Cord*

Figure

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  Fig. 1 Large and numerous congenital melanocytic nevi.

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  Fig. 2 The skin showed nevus cell infiltration with pigmentation in the reticular dermis (hematoxylin and eosin, ×40).

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  Fig. 3 T1-weighted MR scan images of brain and spine with gadolinium injection performed show significant enhancement of cortex in posterior frontal area (brain axial), significant enhancement of pons and hypoplasia of superior or inferior cerebellar vermis and both cerebellar hemisphere with dilatation of the inferior fourth ventricle, cystic enlargement of the posterior fossa (brain sagittal), and extensive enhancement of leptomeninges in spinal cord (spine sagittal).

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  Fig. 4 T1-weighted MR scan (axial and sagittal) images with gadolinium injection performed show significant enhancement of large mass lesion in left frontoparietal area. Intraoperative view shows black diffuse infiltration of leptomeninges and large mass.

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  Fig. 5 Pathological findings show diffuse or solid pattern of small to medium sized malignant cells with dense and hyperchromatic round nuclei and scanty cytoplasm [hematoxylin and eosin (H&E), ×400] (A). Extensive geographic shaped necrosis surrounded by pallisading malignant cells also noted (H&E, ×600) (B). Melanin-pigment laden neoplastic cells are frequently observed (Melanin A, ×400) (C). The positron emission tomography with 18-FDG shows no pathologic deposits of the skin area (D).

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  Fig. 6 T1-weighted MR scan images with gadolinium injection performed. Significant enhancement of recurred mass lesion in left frontoparietal area (A) and new mass on left medial temporal area are observed (B).

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  Fig. 7 T1-weighted MR scan images of spine performed after gadolinium injection show extensive enhancement of leptomeninges involving whole spinal cord and new mass lesion in lower cervix and upper thorax. Intraoperative view in spine shows black diffuse infiltration of leptomeninges with extremely poorly defined border and mass.

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