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Imaging Characteristics of Atypical Teratoid–Rhabdoid Tumor in Children Compared with Medulloblastoma

¹ Department of Radiology, University of Texas Southwestern Medical Center at Dallas and Children's Medical Center of Dallas, 1935 Motor St., Dallas, TX 75235.
² Department of Neuro-oncology, Children's Medical Center of Dallas, Dallas, TX.
³ Department of Pediatrics, University of Texas Southwestern Medical Center at Dallas and Children's Medical Center of Dallas, Dallas, TX.
⁴ Department of Pathology, University of Texas Southwestern Medical Center at Dallas and Children's Medical Center of Dallas, Dallas, TX.
⁵ Department of Neurosurgery, Children's Medical Center of Dallas, Dallas, TX.

OBJECTIVE. The purpose of our study was to compare the imaging characteristics of atypical teratoid–rhabdoid tumor with medulloblastoma and seek distinguishing features that can aid in preoperative diagnosis.

MATERIALS AND METHODS. Preoperative MRI examinations of 55 patients (36 medulloblastomas and 19 atypical teratoid–rhabdoid tumors) were analyzed retrospectively. Imaging characteristics of atypical teratoid–rhabdoid tumor and medulloblastoma were assessed with conventional MRI and CT. Diffusion-weighted imaging (DWI) was available in 27 patients (19 medulloblastomas and eight atypical teratoid–rhabdoid tumors). Apparent diffusion coefficient (ADC) values were calculated for 14 medulloblastomas and six atypical teratoid–rhabdoid tumors.

RESULTS. Both atypical teratoid–rhabdoid tumors in general and infratentorial atypical teratoid–rhabdoid tumors presented at a younger age than medulloblastomas. Eleven of 19 atypical teratoid–rhabdoid tumors were infratentorial. Cerebellopontine angle (CPA) involvement was more frequent (8/11, 72.7%) in atypical teratoid–rhabdoid tumor than in medulloblastoma (4/36, 11.1%) (p < 0.001). Intratumoral hemorrhage was more common in atypical teratoid–rhabdoid tumor (9/19, 47.4%) than in medulloblastoma (2/36, 5.6%) (p < 0.0001). All atypical teratoid–rhabdoid tumors and all medulloblastomas for which DWI was available displayed increased signal intensity on DWI compared with normal brain parenchyma. The mean ADC values for tumor types were not significantly different.

CONCLUSION. Atypical teratoid–rhabdoid tumor presents at a younger age than medulloblastoma. Although atypical teratoid–rhabdoid tumor and medulloblastoma display similar imaging characteristics on conventional MRI, CPA involvement and intratumoral hemorrhage are more common in atypical teratoid–rhabdoid tumor. If a pediatric posterior fossa mass that displays restricted diffusion is involving the CPA, atypical teratoid–rhabdoid tumor is a more likely consideration than medulloblastoma.

Keywords: atypical teratoid–rhabdoid tumor • brain tumor • children • medulloblastoma

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