Nasal Glial Heterotopia (Nasal Glioma)
Article Outline
A 5-day-old-female was referred for evaluation of a congenital tumor on the nasal root and glabella area. She was born at 40 weeks gestation via normal spontaneous vaginal delivery. No complications occurred during the pregnancy or delivery. On examination the tumor showed a 3- × 3-cm, red-colored, firm, nonmobile, and nonpulsatile subcutaneous mass located at the most superior portion of the left side of the nose (Figure 1). Echo-Doppler scanning revealed a solid mass with no proliferation of vessels. Facial magnetic resonance imaging (MRI) demonstrated a soft tissue and well-delimited mass without intracranial extension (Figure 2). The patient was sent for complete excision to the plastic surgery department via an extranasal approach. Histologic examination revealed a prominent dermal and subcutaneous neural proliferation composed of astrocytes, neurons, and neuroglial fibers intermixed with a fibrous connective tissue stroma (Figure 3; available at www.jpeds.com). Immunohistochemical staining for glial fibrillary acidic protein confirmed the presence of the extensive glial tissue. A final diagnosis of nasal glial heterotopia was made.
Figure 1
Red-colored, firm, not mobile and nonpulsatile subcutaneous mass located at nasal root.
Figure 2
Facial MRI scan shows a soft tissue and well-delimited mass without intracranial extension.
Glial heterotopia or the occurrence of isolated nonteratomatous extracranial glial tissue is rare.1 The nose and the nasopharynx are the most common sites of location; hence, the terms “nasal glial heterotopia” or “nasal glioma” are used to describe these lesions.2 Extranasal sites of glial heterotopia are still rarer.3 The differential diagnosis includes hemangiomas, dermoid/epidermoid cysts, encephalocoeles, as well as malignant tumors such as rhabdomyosarcoma. Imaging studies must be performed before surgery to exclude other diagnosis. Echo-Doppler scanning is a noninvasive method that can help to distinguish between vascular and solid tumors. It should be performed in every patient with a congenital subcutaneous tumor. MRI and computed tomography are useful to exclude encephalocele and other congenital tumors.4 Complete surgical excision is the treatment of choice in this disease. Recurrences after surgery rarely occur.2
Figure 3
Dermal and subcutaneous neural proliferation composed of astrocytes, neurons, and neuroglial fibers intermixed with a fibrous connective tissue stroma.
References
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- . Nasal glial heterotopia: a clinicopathologic and immunophenotypic analysis of 10 cases with a review of the literature. Ann Diagn Pathol. 2003;7:354–359
- . Glial heterotopia of the orbit and extranasal region: an unusual entity. Clin Experiment Ophthalmol. 2005;33:513–515
- . Diagnostic imaging in the evaluation of vascular birthmarks. Dermatol Clin. 1998;16:455–488
PII: S0022-3476(09)00898-1
doi:10.1016/j.jpeds.2009.09.015
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