ATRT-10. ATYPICAL TERATOID/RHABDOID TUMOR OF THE PINEAL REGION IN A PEDIATRIC PATIENT. (4th December 2020)

• Record Type: Journal Article
• Authors: Almeida, Carlos; Mançano, Bruna Minniti; Matsushita, Marcus; Previdelli, Stephanie; Lamim, Marina Lopes; Faustino, Fabio Costa; Souza, Fernanda Magalhaes; Lourenço, Lucas Dias
• Is Part Of: Neuro-oncology. Volume 22(2020)Supplement 3
• Page Start: iii277
• Page End: iii277
• Publication Date: 2020-12-04
• Subjects: Brain Neoplasms -- Periodicals; Brain -- Tumors -- Periodicals; Brain -- Cancer -- Periodicals; Nervous system -- Cancer -- Periodicals; 616.99481
• DOI: 10.1093/neuonc/noaa222.010
• Languages: English
• ISSNs: 1522-8517

Abstract

Abstract: BACKGROUND: Atypical teratoid/rhabdoid tumor (ATRT) is a malignant neoplasm of the central nervous system and corresponds to 1.5% of all intracranial tumors. Mainly affects children under three years of age and shows aggressive behavior (most pediatric patients succumb to their disease within a year after the initial diagnosis, despite the treatment performed). Its place of occurrence in children is preferably in the posterior fossa, and it is rare to appear in other regions. There are only seven patients with ATRT reported on literature; all of them are adults. We present the case of a pediatric patient with a tumor in the pineal region diagnosed as ATRT. CASE REPORT: Three-year-old female patient admitted with occipital headache, vomiting, and seizure. Magnetic resonance imaging (MRI) showed obstructive hydrocephalus secondary to a solid-cystic lesion located at the pineal region that was 3.0 x 3.0 x 3.5 cm in size. Spine MRI did not reveal leptomeningeal spreading. We performed an occipital transtentorial approach to achieve the best safe resection possible, and a ventriculoperitoneal shunt. Histological examination revealed ATRT. The patient received adjuvant treatment with radiotherapy and chemotherapy according to the "Head Start" protocol. One year after the surgery, MRI did not identify any remaining lesion. CONCLUSION: ATRT is an aggressive and rare neoplasm whose clinical picture depends on the location of the tumor; however, it must be considered in the differential diagnosis of tumors of the pineal region in the pediatric population.