1323. Pediatric Primary Spinal Atypical Teratoid/Rhabdoid Tumor: Case Series and Review of the Literature.

Authors: Daphne Li, MD; Daniel Heiferman, MD; Hasan Syed, MD; João Santos, MD; Robin Bowman, MD; Arthur DiPatri, MD; Tadanori Tomita, MD; Tord Alden, MD (Chicago, IL)

Introduction:

Atypical teratoid/rhabdoid tumors (ATRT) are rare malignant central nervous system tumors, commonly occurring before three years of age. Median overall survival of these tumors is about one year, despite aggressive multi-modal therapy. Pediatric primary spinal ATRT is even more rare.

Methods:

A systematic MEDLINE search was conducted using key words “atypical teratoid rhabdoid tumor” and “pediatric spinal rhabdoid tumor”. Reports were included for patients less than 21 years old, without evidence of intracranial or systemic disease at the time of diagnosis. Lurie Children’s Hospital of Chicago’s tumor database was reviewed for relevant cases and pertinent electronic medical records were accessed for clinical data.

 

Results:

Four pediatric patients with primary spinal ATRT were treated at Lurie Children’s Hospital of Chicago from 1996 to 2017. Patients (2 to 11 years old) presented with pain and weakness. They were each found to have single intradural extramedullary lesions at different locations in the spine. One patient also had extradural extension of his mass at the cervicothoracic junction. All patients underwent surgical resection and chemotherapy, two had radiotherapy and one had an autologous stem cell transplant. Three patients are known to be deceased (overall survival: 8.5 to 45 months). The fourth patient was last seen in remission 19 years after initial diagnosis.
46 additional cases were reported in the English literature. Patients (2 months to 19 years old) presented with similar symptoms as in our series. The majority were intradural tumors, ten were limited to extradural structures, while ten involved the intra- and extra-dural spine.

Conclusion:

To our knowledge, this is the largest series of pediatric primary spinal ATRT documented at a single institution, and includes the longest overall survival documented. Although rare, spinal ATRT should be considered in the differential diagnosis of pediatric patients presenting with a new spinal mass.