Cardiovascular Pathology 24 (2015) 267–278 Contents lists available at ScienceDirect Cardiovascular Pathology Review Article Consensus statement on surgical pathology of the aorta from the Society for Cardiovascular Pathology and the Association for European Cardio- vascular Pathology: I. Inflammatory diseases James R. Stone a,⁎,PatrickBrunevalb,⁎⁎, Annalisa Angelini c, Giovanni Bartoloni d, Cristina Basso c, Lubov Batoroeva e,L.MaximilianBujaf, Jagdish Butany g, Giulia d'Amati h, John T. Fallon i, Adriana C. Gittenberger-de Groot j, Rosa H. Gouveia k, Marc K. Halushka l, Karen L. Kelly m, Ivana Kholova n, Ornella Leone o, Silvio H. Litovsky p, Joseph J. Maleszewski q, Dylan V. Miller r, Richard N. Mitchell s, Stephen D. Preston t,AngelaPucciu, Stanley J. Radio v,E.ReneRodriguezw, Mary N. Sheppard x, S. Kim Suvarna y, Carmela D. Tan w, Gaetano Thiene c, Allard C. van der Wal z, John P. Veinot aa a Massachusetts General Hospital, Boston, MA, USA b University Paris-Descartes, France c University of Padua Medical School, Padua, Italy d University of Catania, Catania, Italy e Russian Academy of Medical Sciences, Irkutsk, Russia f University of Texas Health Science Center at Houston, Houston, TX, USA g Toronto General Hospital, Toronto, Canada h Sapienza, University of Rome, Rome, Italy i New York Medical College, Valhalla, NY, USA j Leiden University, Leiden, the Netherlands k Hospital Santa Cruz, Carnaxide, Portugal l Johns Hopkins University, Baltimore, MD, USA m East Carolina University, Greenville, NC, USA n Fimlab Laboratories, Tampere, Finland o Sant’Orsola-Malpighi Hospital, University of Bologna, Bologna, Italy p University of Alabama at Birmingham, Birmingham AL, USA q Mayo Clinic, Rochester, MN, USA r University of Utah, UT, USA s Brigham and Women’s Hospital, Boston, MA, USA t Papworth Hospital, Cambridgeshire, UK u Pisa University Hospital, Pisa, Italy v University of Nebraska, NE, USA w Cleveland Clinic, Cleveland, OH, USA x St George Medical School, University of London, London, UK y Sheffield Teaching Hospitals, Sheffield, UK z University of Amsterdam, Amsterdam, the Netherlands aa University of Ottawa, Ottawa, Ontario, Canada article info abstract Article history: Inflammatory diseases of the aorta include routine atherosclerosis, aortitis, periaortitis, and atherosclerosis with Received 23 March 2015 excessive inflammatory responses, such as inflammatory atherosclerotic aneurysms. The nomenclature and his- Received in revised form 11 May 2015 tologic features of these disorders are reviewed and discussed. In addition, diagnostic criteria are provided to dis- Accepted 11 May 2015 tinguish between these disorders in surgical pathology specimens. An initial classification scheme is provided for aortitis and periaortitis based on the pattern of the inflammatory infiltrate: granulomatous/giant cell pattern, There were no sources of external funding. ⁎ Correspondence to: J.R. Stone, Massachusetts General Hospital, Simches Building Room 8236, Boston, MA, USA 02114. Tel.: +1 617 726 8303; fax: +1 617 643 3566. ⁎⁎ Correspondence to: P. Bruneval, Department of Pathology Hôpital Européen Georges Pompidou, 20 rue Leblanc, 75015, Paris, France. Tel.: +33 1 56 09 38 60; fax: +33 1 56 09 38 89. E-mail addresses: [email protected] (J.R. Stone), [email protected] (P. Bruneval). http://dx.doi.org/10.1016/j.carpath.2015.05.001 1054-8807/© 2015 Elsevier Inc. All rights reserved. 268 J.R. Stone et al. / Cardiovascular Pathology 24 (2015) 267–278 Keywords: lymphoplasmacytic pattern, mixed inflammatory pattern, and the suppurative pattern. These inflammatory Atherosclerosis patterns are discussed in relation to specific systemic diseases including giant cell arteritis, Takayasu arteritis, Aortitis granulomatosis with polyangiitis (Wegener’s), rheumatoid arthritis, sarcoidosis, ankylosing spondylitis, Cogan Inflammatory aneurysm syndrome, Behçet’s disease, relapsing polychondritis, syphilitic aortitis, and bacterial and fungal infections. Aorta © 2015 Elsevier Inc. All rights reserved. 1. Introduction encountered by cardiovascular surgical pathology services, reviewed the literature on these conditions, and constructed consensus guidelines Aortic diseases cause significant morbidity and mortality through for nomenclature and diagnostic criteria. For this report, consensus is the development of aneurysm, aortic wall rupture, acute dissection, defined as being the majority of the membership of the committee and luminal obstruction [1,2]. These phenomena can result from many and may not necessarily, in all cases, indicate a unanimous opinion. distinct diseases that afflict the aorta. During surgical repair of aortic an- The opinions put forth here do not necessarily represent the opinions eurysms and dissections, portions of aorta resected by surgeons contain of all members of the Society for Cardiovascular Pathology or the Asso- important diagnostic information concerning these underlying diseases. ciation for European Cardiovascular Pathology. Areas with significant To promote a better understanding of these aortic diseases and to en- dissenting opinion within the committee are indicated in the text. For hance patient care, the Standards and Definitions Committee of the So- the consensus opinions, an emphasis was placed on incorporating the ciety of Cardiovascular Pathology and the Association for European results of relevant published peer-reviewed literature. Cardiovascular Pathology is issuing guidelines for diagnosing aortic dis- eases in surgical pathology specimens. These guidelines will be issued in 3. Histologic classification and diagnostic criteria of inflammatory fi two consecutive reports. This rst report on aortic diseases will cover in- aortic diseases flammatory diseases of the aorta, with other aortic diseases being ad- dressed in a second document. 3.1. Processing of aortic surgical pathology specimens This document on surgical aortic diseases will cover atherosclerosis, fl in ammatory atherosclerotic aneurysms (IAAs), aortitis, and It is recommended to examine the aortic specimen grossly and to fl periaortitis. Including atherosclerosis as an in ammatory aortic disease measure both the overall dimensions of the specimen and thickness of fl isnotadeclarationthatin ammation is the underlying driving force for the aortic wall. It is recommended to note the presence and extent of all atherosclerosis. It is acknowledged that atherosclerosis is usually as- atherosclerotic lesions as well as features suggestive of aortitis, includ- fl sociated with in ammation but has multiple precipitating etiological ing thickening of the wall and cracking/wrinkling of the intima, i.e., a fl factors in addition to in ammation including age, lipid metabolism, “tree bark” appearance. In accordance with previous consensus guide- fl and vascular cell activation. The in ux of lipid into the vessel wall is a lines, it is recommended that all segments of aorta surgically resected fl key feature in the development of atherosclerosis [3,4],andthein am- for aneurysm or dissection be examined histologically [6]. In the ab- mation present is usually a reaction to this lipid. However, in atheroscle- sence of a clinical history of vasculitis or gross findings suggestive of fl rosis, the degree of the in ammatory reaction can be substantial, and in aortitis, it is recommended that six pieces of aorta be submitted in a fl some cases, the in ammation can contribute to complications such as total of two cassettes for initial screening. If there is a clinical history aneurysm formation and surface disruption with thrombus formation, of vasculitis, gross findings worrisome for aortitis, or histologic findings fl justifying the inclusion of atherosclerosis as an in ammatory aortic dis- suggesting aortitis, then it is recommended to submit additional blocks fl ease. It is acknowledged that many primarily nonin ammatory aortic of tissue for histologic examination. In these situations, it is recom- diseases to be covered in the subsequent report are associated with mended to submit up to 12 blocks of tissue, or at least 1 block of tissue fl fl some degree of in ammation, although in those disorders, the in am- per cm of tissue, to fully characterize the nature of the inflammatory in- mation is usually mild and occurs secondarily to some other primary filtrate. Sections are recommended to be 1.0–1.5 cm in length and cut in pathologic process. Disorders of the aortic valve will not be covered in cross section, i.e., perpendicular to the longitudinal axis of the aorta. If this report. atherosclerosis is present, it is recommended to submit both areas fi fl It is understood that precise classi cation of an in ammatory aortic with and without atherosclerosis. If there is a clinical history of infection disease may require correlation of the pathologic features with clinical or a grossly suppurative appearance, i.e., pus, and tissue was not sent for fi features, other laboratory ndings, and imaging. Thus, there are two culture directly from the operating room, then it is recommended to major sections to this report. First, there is a section focusing on a send fresh sterile tissue for culture if possible. Fresh sterile tissue can fi histology-based classi cation scheme, with recommendations for no- also be frozen for potential use in identification of microorganisms by menclature and diagnostic criteria, to aid the surgical pathologist ribosomal DNA amplification and sequencing. Hematoxylin and eosin when confronted with one of these specimens, following which there (H&E) and elastic stains should