Unusual Case of Combined Gliomeningeal Heterotopia on the Nose of an Infant

• Published in: The American journal of dermatopathology Vol. 40; no. 7; pp. 515 - 518
• Main Authors: Schauer, Anna, Harvey, Nathan T, Vijayasekaran, Shyan, Wood, Benjamin A
• Format: Journal Article
• Language: English
• Published: United States 01-07-2018
• Subjects: Choristoma - congenital; Choristoma - pathology; Humans; Infant; Meninges; Neuroglia; Nose Diseases - congenital; Nose Diseases - pathology
• ISSN: 0193-1091; 1533-0311
• DOI: 10.1097/DAD.0000000000001040

Nasal glial heterotopia ("nasal glioma") and cutaneous heterotopic meningeal nodules ("primary cutaneous meningioma") are rare congenital lesions characterized by the presence of heterotopic mature cerebral tissues. Nasal glial heterotopia occurs predominantly in the nasal area and typically does not contain meningothelial elements, whereas heterotopic meningeal nodules occur predominantly on the scalp and do not contain glial elements. In this article, we report an unusual case of cutaneous heterotopia on the nose of an infant composed of both glial and meningothelial elements. The glial component was characterized by irregular islands of predominantly astrocytic cells, on a fibrillary background. The meningothelial component was characterized by bland ovoid cells with focal intranuclear inclusions forming whorled arrangements, with associated psammomatous calcification. To our knowledge, this is the first time such a lesion has been documented. It has also provided us with an opportunity to review the literature regarding heterotopic deposits of both glial and meningothelial tissues.