期刊
CARDIOVASCULAR PATHOLOGY
卷 25, 期 3, 页码 247-257出版社
ELSEVIER SCIENCE INC
DOI: 10.1016/j.carpath.2016.03.002
关键词
Aorta; Histopathology; Marfan syndrome; Loeys-Dietz syndrome; Bicuspid aortic valve; Medial degeneration; Cystic medial degeneration; Aneurysm; Dissection; Lamellar unit; Consensus document; Noninflammatory; Degenerative
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 nonspecific 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 noninflammatory 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. (C) 2016 Elsevier Inc. All rights reserved.
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