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Cardiovasc Pathol. 2016 May-Jun;25(3):247-257. doi: 10.1016/j.carpath.2016.03.002. Epub 2016 Mar 12.

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

Author information

1
Johns Hopkins University, Baltimore, MD, USA.
2
University of Padua, Padua, Italy.
3
University of Catania, Catania, Italy.
4
Russian Academy of Medical Sciences, Irkutsk, Russia.
5
Paris Descartes University, Paris, France.
6
University of Texas Health Science Center at Houston, Houston, TX, USA.
7
Toronto General Hospital, Toronto, Ontario, Canada.
8
Sapienza University of Rome, Rome, Italy.
9
New York Medical College, Valhalla, NY, USA.
10
Southampton University Hospital, Southampton, United Kingdom.
11
Leiden University, Leiden, the Netherlands.
12
Hospital Santa Cruz, Carnaxide, Portugal.
13
Department of Pathology, Fimlab Laboratories, Tampere, Finland.
14
East Carolina University, Greenville, NC, USA.
15
Sant'Orsola-Malpighi Hospital, University of Bologna, Bologna, Italy.
16
University of Alabama at Birmingham, Birmingham, AL, USA.
17
Mayo Clinic, Rochester, MN, USA.
18
University of Utah, Salt Lake City, UT, USA.
19
Brigham and Women's Hospital, Boston, MA, USA.
20
Papworth Hospital, Cambridgeshire, United Kingdom.
21
Pisa University Hospital, Pisa, Italy.
22
University of Nebraska, Lincoln, NE, USA.
23
Cleveland Clinic, Cleveland, OH, USA.
24
St. George's Medical School, University of London, London, United Kingdom.
25
Massachusetts General Hospital, Boston, MA, USA.
26
Sheffield Teaching Hospitals, Sheffield, United Kingdom.
27
University of Ottawa, Ottawa, Ontario, Canada.
28
University of Amsterdam, Amsterdam, the Netherlands.

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 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.

KEYWORDS:

Aneurysm; Aorta; Bicuspid aortic valve; Consensus document; Cystic medial degeneration; Degenerative; Dissection; Histopathology; Lamellar unit; Loeys–Dietz syndrome; Marfan syndrome; Medial degeneration; Noninflammatory

PMID:
27031798
DOI:
10.1016/j.carpath.2016.03.002
[Indexed for MEDLINE]

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