Consensus statement on surgical pathology of the aorta from the Society For Cardiovascular Pathology and the Association For European Cardiovascular Pathology: II. non-inflammatory degenerative diseases – nomenclature and diagnostic criteria

• Published in: Cardiovascular pathology 2016
• Main Authors: Halushka, Marc K, Angelini, Annalisa, Bartoloni, Giovanni, Basso, Cristina, Batoroeva, Lubov, Bruneval, Patrick, Buja, L. Maximilian, Butany, Jagdish, d'Amati, Giulia, Fallon, John T, Gallagher, Patrick J, Gittenberger-de Groot, Adriana C, Gouveia, Rosa H, Kholova, Ivana, Kelly, Karen L, Leone, Ornella, Litovsky, Silvio H, Maleszewski, Joseph J, Miller, Dylan V, Mitchell, Richard N, Preston, Stephen D, Pucci, Angela, Radio, Stanley J, Rodriguez, E. Rene, Sheppard, Mary N, Stone, James R, Suvarna, S. Kim, Tan, Carmela D, Thiene, Gaetano, Veinot, John P, van der Wal, Allard C
• Format: Article
• Language: English
• Subjects: Pathology
• ISSN: 1054-8807
• DOI: 10.1016/j.carpath.2016.03.002

Abstract Surgical aortic specimens are usually examined in Pathology Departments as a result of treatment of aneurysms or dissections. A number of diseases, genetic syndromes (Marfan syndrome, Loeys-Dietz syndrome, etc.), and vasculopathic aging processes involved in vascular injury can cause both distinct and non-specific histopathologic changes with degeneration of the media as a common denominator. Terminology for these changes has varied over time leading to confusion and inconsistencies. This consensus document has established a revised, unified nomenclature for the variety of non-inflammatory degenerative aortic histopathologies seen in such specimens. Older terms such as cystic medial necrosis and medionecrosis are replaced by more technically accurate terms such as mucoid extracellular matrix accumulation (MEMA), elastic fiber fragmentation and/or loss and smooth muscle cell nuclei loss. A straightforward system of grading is presented to gauge the extent of medial degeneration and synoptic reporting tables are provided. Herein we present a standardized nomenclature that is accessible to general pathologists and useful for future publications describing these entities.