NS-04A CASE REPORT OF A GIANT CONGENITAL TERATOMA MANAGED THROUGH EX UTERO INTRAPARTUM TREATMENT PROCEDURE AND MULTIPLE STAGED SURGERIES

Abstract

NS-04. A CASE REPORT OF A GIANT CONGENITAL TERATOMA MANAGED THROUGH EX UTERO INTRAPARTUM TREATMENT PROCEDURE AND MULTIPLE STAGED SURGERIES Ulrika Sandvik1, Caroline Gahm2, Georgios Papatziamos2, Andreas Ekborn2, Erik Neovius3, Yinghua Li4, and Bengt Gustavsson1; Section for Neurosurgery, Department of Clinical Neuroscience. Karolinska University Hospital, Stockholm, Sweden; Department of Otorhinolaryngology, Head and Neck Surgery, Karolinska University Hospital, Stockholm, Sweden; Stockholm Craniofacial Center, Department of Reconstructive Plastic Surgery, Karolinska University Hospital, Stockholm, Sweden; Departmentof Neonatology, Astrid Lindgrens Childrens Hospital, Karolinska University Hospital, Stockholm, Sweden Our patient was prenatally diagnosed with a giant teratoma. The delivery was performed in gestational week 32 + 5 with birth weight 2.3kg through an EXIT (ex utero intrapartum treatment) procedure including caesarean section and an immediate tracheostomy followed by neonatal intensive care. MRI revealed a multicystic, contrast enhancing tumor extending from the left shoulder area up to the face including the left orbita. The tumor extended also intracranially to the left posteriorand middle fossa dislocating both brainstem and temporal lobe. After thorough the ethical discussions and planning, the child was operated by staged surgeries. At four days of age the child was operated for the cervical part of the teratoma by a team of ENT-surgeons. A second surgery performed by a team of ENT-surgeons, plastic surgeons and neurosurgeons was done five days later. A residual mass in the epipharynx was removed one month later. The histopathological evaluation was consistent with congenital immature teratoma WHO grade III. The tracheostoma was successfully removed two months later and the patient was discharged after three month neonatal intensive care without infection or intracranial hemorrhage. The patient is currently 21 months and displays normal mental, motor, and physical development and has so far been relapse-free with low levels of alpha fetoprotein. Congenital giant teratomas are associated with significant surgical risks mainly due to blood loss and difficulties in maintaining a patent airway. Here we present a strategy where a highly specialized delivery process and multiple staged surgeries have been essential for successful management. Neuro-Oncology 18:iii127–iii134, 2016. doi:10.1093/neuonc/now078.4 #The Author(s) 2016. Published by Oxford University Press on behalf of the Society for Neuro-Oncology. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.