Radiation Necrosis with Proton Therapy in a Patient with Aarskog-Scott Syndrome and Medulloblastoma.

Autor:	Puthenpura V; Section of Pediatric Hematology and Oncology, Department of Pediatrics, Yale University School of Medicine, New Haven, CT, USA., DeNunzio NJ; Department of Radiation Oncology, Massachusetts General Hospital/Harvard Medical School, Boston, MA, USA., Zeng X; Department of Genetics, Yale University School of Medicine, New Haven, CT, USA., Giantsoudi D; Department of Radiation Oncology, Massachusetts General Hospital/Harvard Medical School, Boston, MA, USA., Aboian M; Section of Neuroradiology and Nuclear Medicine, Department of Radiology, Yale University School of Medicine, New Haven, CT, USA., Ebb D; Department of Pediatric Hematology/Oncology, Massachusetts General Hospital/Harvard Medical School, Boston, MA, USA., Kahle KT; Department of Genetics, Yale University School of Medicine, New Haven, CT, USA.; Department of Neurosurgery, Yale University School of Medicine, New Haven, CT, USA., Yock TI; Department of Radiation Oncology, Massachusetts General Hospital/Harvard Medical School, Boston, MA, USA., Marks AM; Section of Pediatric Hematology and Oncology, Department of Pediatrics, Yale University School of Medicine, New Haven, CT, USA.
Jazyk:	angličtina
Zdroj:	International journal of particle therapy [Int J Part Ther] 2021 Jul 29; Vol. 8 (3), pp. 58-65. Date of Electronic Publication: 2021 Jul 29 (Print Publication: 2022).
DOI:	10.14338/IJPT-21-00013.1
Abstrakt:	Purpose: Medulloblastoma is known to be associated with multiple cancer-predisposition syndromes. In this article, we explore a possible association among a patient's Aarskog-Scott syndrome, development of medulloblastoma, and subsequent brainstem radiation necrosis. Case Presentation: A 5-year-old male with Aarskog-Scott syndrome initially presented to his pediatrician with morning emesis, gait instability, and truncal weakness. He was ultimately found to have a posterior fossa tumor with pathology consistent with group 3 medulloblastoma. After receiving a gross total resection and standard proton beam radiation therapy with concurrent vincristine, he was noted to develop brainstem radiation necrosis, for which he underwent therapy with high-dose dexamethasone, bevacizumab, and hyperbaric oxygen therapy with radiographic improvement and clinical stabilization. Conclusion: Based on several possible pathologic correlates in the FDG1 pathway, there exists a potential association between this patient's Aarskog-Scott syndrome and medulloblastoma, which needs to be investigated further. In patients with underlying, rare genetic syndromes, further caution should be taken when evaluating chemotherapy and radiation dosimetry planning. Competing Interests: Conflicts of Interest: The authors have no relevant conflicts of interest to disclose. (©Copyright 2021 The Author(s).)
Databáze:	MEDLINE
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