Atypical teratoid/rhabdoid tumor of the velum interpositum presenting as a spontaneous intraventricular hemorrhage in an infant: case report with long-term survival

Pediatric Neurosurgery
Daniel J DonovanGregory W Petermann

Abstract

Atypical teratoid/rhabdoid tumors (ATRT) of infancy are highly malignant neoplasms that are most common in the first 2 years of life. We present the case of a 3-month-old girl who presented with the acute onset of generalized seizures and was found to have a large spontaneous intraventricular hemorrhage. The blood masked an underlying ATRT of the velum interpositum in the midline of the lateral ventricles and roof of the third ventricle, the first reported case in this location. Serial imaging studies and two ventriculoscopic biopsies were required to establish the diagnosis of the tumor in this unique location and in the midst of an evolving hematoma. After surgical resection, the patient received adjuvant chemotherapy. At 4-year follow-up, the child is neurologically intact, meeting normal developmental milestones, and imaging studies show no evidence of tumor. ATRT were previously associated with an extremely poor prognosis, but more recent evidence with complete surgical resection and adjuvant chemotherapy shows extended survival in some cases, supporting an aggressive and comprehensive approach to give these patients the best chance for a good outcome. Spontaneous brain hemorrhage in a full-term infant requires a diligent ...Continue Reading

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Citations

Nov 13, 2012·Pediatric Radiology·Kong Jung Au YongMichael R Ditchfield
Dec 21, 2007·Surgical and Radiologic Anatomy : SRA·R Shane TubbsW Jerry Oakes
Feb 13, 2009·Child's Nervous System : ChNS : Official Journal of the International Society for Pediatric Neurosurgery·Yeşim ErtanTaner Akalin
May 2, 2009·Brain Tumor Pathology·Ichiyo ShibaharaTeiji Tominaga
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Mar 19, 2013·Pediatric and Developmental Pathology : the Official Journal of the Society for Pediatric Pathology and the Paediatric Pathology Society·Michael A ArnoldChristopher R Pierson
Apr 3, 2012·Journal of Neurosurgery. Pediatrics·Junko Takahashi-FujigasakiMasaharu Yasue
May 29, 2012·Asian Journal of Neurosurgery·Ahmed Zohdi, Sherif Elkheshin
Jul 24, 2014·Cancer Genetics·Simone T SredniTadanori Tomita
Mar 11, 2016·Journal of Pediatric Neurosciences·Mehdi DarmoulMohamed Nejib Hattab
Aug 20, 2008·Journal of Clinical Neuroscience : Official Journal of the Neurosurgical Society of Australasia·Biji BahuleyanAri G Chacko
Jul 28, 2012·Seizure : the Journal of the British Epilepsy Association·Jeffrey G OjemannMark D Holmes
May 3, 2012·Journal of Child Neurology·Fang LiYueshan Piao
Apr 21, 2020·British Journal of Neurosurgery·Zhongzhong JiangMengqiang Yu

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