J Korean Neurosurg Soc.  2012 Oct;52(4):417-419. 10.3340/jkns.2012.52.4.417.

Intradural Extramedullary and Subcutaneous Tumors in Neonate : Atypical Myxoid Spindle Cell Neoplasm

• Yu DW
• Choi JH
• Lee ES
• Kim SH

• KMID: 2190664
• DOI: http://doi.org/10.3340/jkns.2012.52.4.417

Abstract

Tumors of the central nervous system are common in the pediatric population and constitute the second most prevalent tumor type in children. Within this group, spinal cord tumors are relatively rare and account for 1 to 10% of all pediatric central nervous system tumors. We describe a very rare case of an intradural extramedullary spinal cord tumor with a subcutaneous mass and discuss its clinical presentation, pathogenesis, and treatment. A male infant was delivered normally, with uneventful development. At 16 days post-delivery, his family took him to a pediatrician because of a mass on his upper back. Magnetic resonance imaging of the thoracic spine revealed a well-demarcated soft-tissue mass with central cystic change or necrosis at the subcutaneous layer of the posterior back (T2-7 level). Another mass was found with a fat component at the spinal canal of the T1-3 level, which was intradural extramedullary space. After six weeks, the spinal cord tumor and subcutaneous mass were grossly total resected; pathologic findings indicated an atypical myxoid spindle cell neoplasm, possibly nerve sheath in type. The final diagnosis of the mass was an atypical myxoid spindle cell neoplasm. The postoperative course was uneventful, and the patient was discharged after nine days without any neurological deficit. We report a rare case of an intradural extramedullary spinal tumor with subcutaneous mass in a neonate. It is necessary to monitor the patient's status by examining consecutive radiologic images, and the symptoms and neurological changes should be observed strictly during long-term follow-up.

Keyword

Intradural extramedullary spinal cord neoplasm; Neonate; Congenital

MeSH Terms

Central Nervous System
Central Nervous System Neoplasms
Child
Follow-Up Studies
Humans
Infant
Infant, Newborn
Magnetic Resonance Imaging
Male
Necrosis
Organothiophosphorus Compounds
Spinal Canal
Spinal Cord Neoplasms
Spine
Organothiophosphorus Compounds

Figure

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  Fig. 1 Preoperative sagittal T1-weighted gadolinium enhanced MRI of the thoracic spine showing the presence of an inhomogeneous high signal intradural mass between the T1 to T3 level, and another mass in the subcutaneous layer of the posterior back (T2-7 level). A : Initial MRI at 16 days post delivery. B : Follow up MRI at 58 days post delivery. Intradural extramedullary mass was increased, and the spinal cord was markedly compromised by the tumor mass. MRI : magnetic resonance imaging.

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  Fig. 2 A : Photographs of the whitish and soft subcutaneous mass (5.0×4.0×0.8 cm). B : The cut surface shows a homogenous, whitish appearance.

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  Fig. 3 Most of the lesional cells have ovoid, tapering, or wavy nuclei with rather neural-like morphology distributed in a myxoid or collagenous stroma (H & E stain, ×100).

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