J Korean Soc Radiol.  2018 Jun;78(6):417-421. 10.3348/jksr.2018.78.6.417.

Atypical Teratoid/Rhabdoid Tumor of Lumbar Spine in an Infant: A Case Report

• Song H
• Kim Y
• Lee J
• Chang S
• Sohn M

• KMID: 2412361
• DOI: http://doi.org/10.3348/jksr.2018.78.6.417

Abstract

Atypical teratoid/rhabdoid tumor (AT/RT) of spine usually reported to develop in the brain, while it rarely manifest in the spine. It consists of rhabdoid cells and is highly malignant. AT/RT appears at various sites throughout the body, such as in the central nervous system, liver, kidneys, abdomen, and soft tissues. Among them, spinal AT/RT is rare, and AT/RT of lumbar spine is extremely rare; only a few cases have been reported. We present the case of an AT/RT of lumbar spine in a 16-month-old boy.

MeSH Terms

Abdomen
Brain
Central Nervous System
Chromosomes, Human, Pair 22
Humans
Infant*
Kidney
Liver
Male
Rhabdoid Tumor
Spine*

Figure

• 

  Fig. 1 Atypical teratoid/rhabdoid tumor of lumbar spine in an infant. A. T2-weighted sagittal image shows a heterogeneously isointense and hyperintense mass in the spinal canal at the level of L1-L3. B. The mass exhibits isointensity on the T1-weighted sagittal image. C. The gadolinium-enhanced, T1-weighted, fat-suppressed sagittal image shows heterogeneous enhancement of the mass. D. The gadolinium-enhanced, T1-weighted, fat-suppressed axial image shows a dumbbell-shaped mass in the spinal canal on the right, extending to the right lumbar plexus through the right intervertebral foramen. E. The tumor shows a variety of histologic patterns. The tumor cells have eosinophilic cytoplasm with eccentric nuclei; these are typical rhabdoid features. Some areas show large tumor cells with abundant eosinophilic cytoplasm and prominent nucleoli. Cords of tumor cells appear in a myxoid matrix resembling chondroid differentiation (hematoxylin and eosin stain, a: × 200, b: × 200, c: × 100). F. Immunohistochemical staining for integrase interactor 1, the product of the SMARCB1 gene, shows loss of expression in the nuclei of tumor cells, compared to retained staining of the nuclei of inflammatory cells. The tumor cells are positive for epithelial membrane antigen and cytokeratin (a: integrase interactor 1 stain, × 200, b: epithelial membrane antigen stain, × 200, c: cytokeratin stain, × 200).

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