Neurofibroma of the Ascending Aorta Combined with Aortic Dissection

HomeRadiologyVol. 311, No. 1 PreviousNext Reviews and CommentaryFree AccessImages in RadiologyNeurofibroma of the Ascending Aorta Combined with Aortic DissectionLan Shang, Weifang Kong Lan Shang, Weifang Kong Author AffiliationsFrom the Department of Radiology, Sichuan Provincial People's Hospital, School of Medicine, University of Electronic Science and Technology of China, No 32 W Section 2, First Ring Rd, Chengdu 610072, China.Address correspondence to W.K. (email: [email protected]).Lan ShangWeifang Kong Published Online:Apr 2 2024https://doi.org/10.1148/radiol.232355MoreSectionsPDF ToolsAdd to favoritesCiteTrack CitationsPermissionsReprints ShareShare onFacebookXLinked In A 33-year-old man with a 10-year history of hypertension presented with several days of chest pain and was referred to our hospital in stable condition. Thoracic aorta CT angiography showed aortic dissection at the aortic root and thickening of the left side of the aortic wall, with attenuation of 64 HU (Figure, A, B). Cardiac MRI was performed to exclude myocarditis because serum cardiac enzymes were elevated. MRI revealed a crescentic soft tissue signal abnormality of the aortic wall with marked delayed enhancement (Figure, C, D). These findings were overlooked prior to surgery. The thickened aortic wall with ill-defined margins was partly resected (Figure, E) and diagnosed as a neurofibroma at histopathologic examination; this is an extremely rare benign aortic tumor. No recurrence was observed at follow-up CT angiography 38 months later.Images in a 33-year-old man with neurofibroma in the ascending aorta. (A) Coronal CT angiogram demonstrates a small dissection (solid black arrows) of the aortic root (dashed black arrows) and a focus (white arrows) with intermediate attenuation on the left extending from the aortic root superiorly to the aortic arch. * = Ascending aorta. (B) Axial CT angiogram shows the crescentic thickening (arrows)—with a thickness of more than 15 mm and attenuation of 64 HU—that was eventually revealed to represent a neurofibroma. (C) Axial T2-weighted cardiac MRI scan shows crescentic structure (arrow) in the left anterior wall with intermediate signal intensity consistent with soft tissue. (D) Axial contrast-enhanced T1-weighted MRI scan shows focal crescentic late enhancement of the aortic wall (arrow). (E) Photograph of the surgical specimen shows the soft tissue tumor in the aortic wall (arrow indicates the extent of the tumor).Download as PowerPointWe hypothesize that the hemodynamic abnormalities caused by hypertension led to changes in structural components and remodeling of the aortic wall, thus forming the aortic dissection (1). However, the pathogenesis of the aortic neurofibroma and the relationship between hypertension and aortic dissection in this case are still uncertain. It is difficult to differentiate a tiny tumor from aortic wall, intramural hematoma, and atheromatous thrombus using CT angiography. MRI and MR angiography with gadolinium can demonstrate tumoral enhancement, while also defining the periaortic extension (2). When evaluating aortic dissection using CT angiography, careful attention should be paid to the pattern of thickening and enhancement of the aortic wall, to evaluate for this rare entity.Disclosures of conflicts of interest: L.S. No relevant relationships. W.K. No relevant relationships.References1. Choi JC, LeMaire SA. Thoracic aortic dissection: genes, molecules, and the knife. Tex Heart Inst J 2012;39(6):838–839. Medline, Google Scholar2. Mohsen NA, Haber M, Urrutia VC, Nunes LW. Intimal sarcoma of the aorta. AJR Am J Roentgenol 2000;175(5):1289–1290. Crossref, Medline, Google ScholarArticle HistoryReceived: Sept 21 2023Revision requested: Oct 31 2023Revision received: Dec 7 2023Accepted: Dec 18 2023Published online: Apr 02 2024 FiguresReferencesRelatedDetailsRecommended Articles Chest CT Angiography for Acute Aortic Pathologic Conditions: Pearls and PitfallsRadioGraphics2021Volume: 41Issue: 2pp. 399-424Aortic Dissection and Other Acute Aortic Syndromes: Diagnostic Imaging Findings from Acute to Chronic Longitudinal ProgressionRadioGraphics2021Volume: 41Issue: 2pp. 425-446Secondary Hypertension and Complications: Diagnosis and Role of ImagingRadioGraphics2019Volume: 39Issue: 4pp. 1036-1055Mimics of Complications in the Postsurgical Aorta at CTRadiology: Cardiothoracic Imaging2019Volume: 1Issue: 4Emerging Concepts in Intramural Hematoma ImagingRadioGraphics2016Volume: 36Issue: 3pp. 660-674See More RSNA Education Exhibits Root Cause Analysis: Understanding Type A Aortic Dissection EquivalentsDigital Posters2020Non-Ordinary Cases of Acute Aortic Syndromes: Do Not Miss Them Out!Digital Posters2020Aortic Calcifications As and Index to Disease: Spectrum of Pathologies & Imaging Findings in Various Conditions Causing Aortic CalcificationsDigital Posters2020 RSNA Case Collection Stanford Type A dissectionRSNA Case Collection2020Aortopulmonary fistula due to aortic dissection RSNA Case Collection2020Intramural hematomaRSNA Case Collection2020 Vol. 311, No. 1 Metrics Altmetric Score PDF download