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A Posterior Neck Mass in a 7-Month-Old Infant

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A full-term, 7-month-old male infant presented to an outpatient pediatric otolaryngology clinic with a firm, irregularly shaped posterior neck mass. He had been diagnosed prenatally with cerebellar hypoplasia, so a magnetic resonance imaging study of the brain was performed at 1 month of age. This study demonstrated an incidental solid, homogenous mass in the subcutaneous fat of the right occiput with irregular borders and infiltration into the paraspinal musculature (Figure, A). On examination, the upper posterior neck mass was deep to palpation without overlying skin changes. The patient had no discomfort, and neck movement was not restricted.

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D. Glial heterotopia

Glial heterotopia occurs when normal glial tissue abnormally appears at a location distant from the central nervous system (CNS). These lesions arise when fragments of embryonic neuroglial tissue become separated from the developing CNS.1 Although glial heterotopia lesions may be connected to the cranium via a fibrous stalk, complete separation (rather than herniation or invagination) from the CNS is what differentiates lesions such as glial heterotopia from meningoceles, encephaloceles, or meningoencephaloceles. While glial heterotopia in the head and neck area often presents in the nasal region (frequently referred to as nasal glioma), these masses may occur in other locations along the developing nervous system, including the paranasal sinuses, orbits, nasopharynx, scalp, face, neck, lungs, and gluteal regions.1,2 In many cases, glial heterotopia is asymptomatic and discovered incidentally.3 The incidence of nasal glioma is 1 in 20 000 to 40 000 births, with nonnasal glial heterotopia presenting even less commonly.4 Glial heterotopia may be associated with other congenital abnormalities, including cleft palate, congenital heart defects, Pierre Robin sequence, or posterior fossa anomalies, hemangioma, arterial anomalies, cardiac anomalies, and eye anomalies syndrome.5,6

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Article Information

Corresponding Author: Andrew R. Scott, MD, Division of Otolaryngology and Facial Plastic Surgery, Floating Hospital for Children at Tufts Medical Center, 800 Washington St, Boston, MA 02111 (ascott@tuftsmedicalcenter.org).

Published Online: October 17, 2019. doi:10.1001/jamaoto.2019.2861

Conflict of Interest Disclosures: None reported.

Additional Contributions: We thank the patient’s parents for granting permission to publish this information. We would also like to thank Julie Tse, MD, a pathologist at Tufts Medical Center, for providing the histopathologic images that were used in this article. She was not compensated for her contributions.

References
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McDermott  MB, Glasner  SD, Nielsen  PL, Dehner  LP.  Soft tissue gliomatosis. Morphologic unity and histogenetic diversity.  Am J Surg Pathol. 1996;20(2):148-155. doi:10.1097/00000478-199602000-00002PubMedGoogle ScholarCrossref
2.
Lim  RY, Capinpin  AG.  Extensive heterotopic brain tissue of the head and neck.  Otolaryngol Head Neck Surg. 1991;105(3):469-472. doi:10.1177/019459989110500320PubMedGoogle ScholarCrossref
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Kallman  JE, Loevner  LA, Yousem  DM,  et al.  Heterotopic brain in the pterygopalatine fossa.  AJNR Am J Neuroradiol. 1997;18(1):176-179.PubMedGoogle Scholar
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Haloob  N, Pepper  C, Hartley  B.  A series of parapharyngeal glial heterotopia mimicking lymphatic malformation.  Int J Pediatr Otorhinolaryngol. 2015;79(12):1975-1979. doi:10.1016/j.ijporl.2015.09.001PubMedGoogle ScholarCrossref
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Longo  D, Menchini  L, Delfino  LN,  et al.  Parapharyngeal neuroglial heterotopia in Pierre Robin sequence: MR imaging findings.  Int J Pediatr Otorhinolaryngol. 2009;73(9):1308-1310. doi:10.1016/j.ijporl.2009.05.014PubMedGoogle ScholarCrossref
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Boyer  AC, Krishnan  A, Goncalves  LF, Williams  L, Chaiyasate  K.  Prenatal diagnosis of nasal glioma associated with metopic craniosynostosis: case report and review of the literature.  J Radiol Case Rep. 2015;9(4):1-8.PubMedGoogle Scholar
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Navarro  OM, Laffan  EE, Ngan  BY.  Pediatric soft-tissue tumors and pseudo-tumors: MR imaging features with pathologic correlation: part 1. Imaging approach, pseudotumors, vascular lesions, and adipocytic tumors.  Radiographics. 2009;29(3):887-906. doi:10.1148/rg.293085168PubMedGoogle ScholarCrossref
8.
Darrow  DH, Greene  AK, Mancini  AJ, Nopper  AJ; Section on Dermatology, Section on Otolaryngology–Head and Neck Surgery, and Section on Plastic Surgery.  Diagnosis and management of infantile hemangioma.  Pediatrics. 2015;136(4):e1060-e1104. doi:10.1542/peds.2015-2485PubMedGoogle ScholarCrossref
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Skapek  SX, Ferrari  A, Gupta  AA,  et al.  Rhabdomyosarcoma.  Nat Rev Dis Primers. 2019;5(1):1. doi:10.1038/s41572-018-0051-2PubMedGoogle ScholarCrossref
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