Atrt-25. Cervical Atypical Teratoid Rhabdoid Tumor in Pediatric Patients: A Study of the Last Decade

Abstract

BACKGROUND: Atypical rhabdoid teratoid tumor (AT/RT) is an extremely rare primary tumor of the central nervous system (CNS) with a high incidence in children under 3 years of age. The existing literature involving this tumor is scarce, even though AT/RT is considered one of the most malignant brain tumors due to its aggressive course and short survival time, which generally does not exceed 1 year. METHODS: The “PubMed” database was used for this study using the MeSH Terms “Child” and “Atypical Teratoid Rhabdoid Tumor” and the filter “Case Reports” in order to carry out a study with the clinical cases of the last decade and the involvement of this tumor in the cervical region of pediatric patients. 96 articles were found, however, only 10 articles were selected for this review. RESULTS: It is possible to affirm that the cases of AT/RT are found in greater numbers in children under 3 years of age and are predominantly located in the cervical region when they affect the spinal cord. In addition, MRI usually shows a solid heterogeneous mass that results in the appearance of symptoms, since the mass compresses the conus medullaris and consequently generates pain in the spine. The prognosis is reported to be a difficult one and the median patient survival time is less than one year. CONCLUSION: Therefore, AT/RT is rare, fatal, primary and malignant in the CNS that have a high degree of recurrence and dissemination. The diagnosis is made through anatomopathological examination and the loss of INI-1 expression constitutes an important finding for its conclusion. Treatment is multimodal and may combine surgery, chemotherapy and radiotherapy.