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Archives of the AIRP: Intimal Sarcomas of the Great VesselsMichael Morris, M.D., M.S.1*; Babak Saboury, M.D., M.P.H.1*; Gary Rose, M.D.1*; Niketh Bandla, M.D.1; Alan Ropp, M.D.1; Allen Burke, M.D.1^; Aletta Ann Frazier, M.D.1,2^
1-University of Maryland School of Medicine/Medical Center2-American Institute for Radiologic Pathology/American College of Radiology*These Authors Contributed Equally^These Authors were Co-Primary Investigators
The authors have nothing to disclose…
Disclosures
Aortic and pulmonary artery (PA) intimal sarcomas are rare diagnoses of middle age often missed at initial presentation: this delay in diagnosis impacts outcome.
Clinical symptomatology is frequently related to embolic complications of stroke, pulmonary embolism, end organ or limb ischemia (including claudication and absent pulses).
Metastases and invasion may occur in lung, pleura, pericardium, chest wall, liver, kidney, adrenal glands, brain, and bone.
Treatment is primarily surgical with poor survival rates of 28% at 5 years, 14% at 10 years. Adjuvant radiation and chemotherapy are areas of current investigation.
Introduction
Spectrum of Initial Imaging Manifestations
Ventilation/Perfusion Defects Mesenteric Ischemia
Cavitary Lesion
Distal Embolic Phenomena
Mediastinal Mass +/- Effusion
Pulmonary Embolism
Diagnostic Dilemma: Rarity & Low Suspicion
Initial Presentation
Metastatic Disease Disability
Palliative Treatment
Death
months later
Diagnostic Imaging
Misdiagnosed as Thrombus
Identify Suspicious Features Early Diagnosis
?Objective: Learn when to suspect Great Vessel Sarcomas
Common Situation
Treatment Options
With Improved Understanding
Increased Survival
Decreased Disability
Often resembles thrombus or gelatinous tumor mass (some are initially misdiagnosed as embolectomy specimens)
Entirely or predominantlyintraluminal, with focal invasion(consistent with origin in thearterial intima, rather than arterial wall)
Lobulated contours correspond to radiologic features
Pulmonary artery sarcoma (arrows) often fills the vessel lumen
Gross Appearance of Intimal Sarcoma (Aorta & PA)
Aortic intimal sarcoma (arrows) shows preferential intraluminal growth
Aorta: atypical neoplastic cells on microscopic exam differentiate the lesion from non-malignant, bland emboli
Aorta: frequently the tumor grows as a layer of neoplastic cells overlying a core of thrombus/necrosis(less commonly observed in pulmonary artery sarcomas)
Pulmonary Artery: intimal sarcomas often appear more cellular, with nearly total occlusion of vessel lumen(heterogeneous mixture of tumor & thrombus evident throughout the lesion)
Aorta
Low Power High Power
Histology of Intimal Sarcoma (Aorta vs. PA)
Low Power High Power
Layer of Pleomorphic
TumorCells
Pulmonary Artery
Two most common aortic intimal subtypes: epithelioid angiosarcoma and undifferentiated angiosarcoma
Differentiation between subtypes frequently requires immunohistochemistry
Pulmonary artery intimal sarcomas do not appear to have a predominant subtype
Compared to aorta, pulmonary artery intimal sarcomas more often contain heterologous elements: osteosarcoma and chondrosarcoma
Core of Thrombus/
NecrosisCore of
Thrombus/Necrosis
Histology of Intimal Sarcoma (Aorta vs. PA)
Aortic Intimal Sarcoma
High power view shows marked cellular atypia,
plentiful mitoses, myxoid background
Low power view shows tumor cells coating
thrombus on intimal surface of aorta
Great Vessel Intimal Sarcoma:Review Methodology
82 Literature Articles ReviewedOf these, 14 articles describe suspicious imaging features in 57 Cases: 8 Aortic & 49 PA Intimal Sarcomas
AIRP Archives & UMMC provide24 Cases: 7 Aortic & 17 PA Intimal Sarcomas
Total Analyzed: 15 Aortic & 66 PA Intimal Sarcomas
Suspicious Features: Reporting Method
Feature Cases Percent
IMAGES Placed Here
(template)
• Imaging features reviewed: combined AIRP, UMMC, and Literature Cases• Feature occurrence quantified: combined total from available material• Some features were not described for all cases reviewed• Intimal sarcoma features quantified as arising in PA, Aorta, or Both
Exemplary Images and annotations
Name of suspicious
feature
Ratio of total cases
demonstrating feature
Percent of total cases demonstrating feature
24/33Lobulated Margin 73%Feature #1, Characteristic
CTA CTA
PA Intimal Sarcoma
32/66Wall Eclipsing Sign 62%Feature #2, Characteristic
Lumen obliterated on both sides (“eclipsed”)
Appearance on axial imaging
PA Intimal Sarcoma
MRA T1 Black Blood
CTA CTA
8/13Contrast Enhancement 62%Feature #3, Characteristic
Heterogeneous Attenuation Fine Linear
More conspicuous on MRI
VIBE post gad
VIBE post gad
CTA
PA Intimal Sarcoma
22/66Unilateral Mass 33%Feature #4, Characteristic
Right PA Mass Left PA Mass Left PA Mass
MRA T1 Black Blood
MRA T1 Black Blood
Non-contrast
CTA
PA Intimal Sarcoma
CT-post
CT-post
30/48Interval Enlargement 63%Late Feature, Characteristic
Prior Follow up
PA Intimal Sarcoma
HIGHLY Suspicious, however our goal is to diagnose earlierCT postCTA
10/32Metastasis or Invasion 31%Late Feature, Characteristic
T1 post
T1 post subtraction
Paracardiaclymph node
Vena cava
Tumor thrombus
Pericardium
PA Intimal Sarcoma
HIGHLY Suspicious, however due to earlier onset of symptoms, most are diagnosed earlier
CTA
CT Post
13/15Lobulated Margin 87%Feature #1, CharacteristicAortic Intimal Sarcoma
CTA
CTA
CTA
CTA
13/15Atherosclerosis Absent to Minimal
87%
Feature #2, Our ObservationAortic Intimal Sarcoma
CTA CTA
12/15Thoracic Location 80%Feature #3, Characteristic
Most sarcomas occur in the thoracic aorta, whereas most benign intimal thrombus occurs in the abdominal aorta
Aortic Intimal Sarcoma
CTA
CTA CTA
1/8Contrast Enhancement 13%Feature #4, Characteristic
Enhancement is unusual in aortic intimal sarcomas, which are largely comprised of central necrosis/thrombus coated with a superficial layer of tumor.(Enhancement appears more common in PA sarcomas, perhaps due to the more heterogeneous composition of tumor and thrombus.)Contrast enhancement = HIGHLY SUSPICIOUS for malignancy (on CT and MRI)
Aortic Intimal Sarcoma
CTACTA
7/8Metastasis or Embolism 97%Late Feature, Characteristic
Right Adrenal
Left Adrenal
Left Temporal Lobe
Aortic Intimal Sarcoma
HIGHLY suspicious, however our goal is to diagnose earlier
37/48
PA Intimal Sarcoma
Lobulated Margin 77%Common Feature, Shared Characteristic
Aortic Intimal Sarcoma
CTACTA
Both PA & Aortic Intimal Sarcoma
27/32Eccentric Endoluminal Mass 84%Common Feature, Our Observation
VIBE post gad MRA
VIBE post gadCTA
CTA
CTA
CTA
Both PA & Aortic Intimal Sarcoma
PA Intimal Sarcoma PA Intimal Sarcoma Aortic Intimal Sarcoma
Both PA & Aortic Intimal Sarcoma
8/8
FDG PET/CT FusedNon-contrast CT
FDG PETScout
18F-FDG PET Avidity 100%Most Diagnostic Feature
PA (conspicuous) Aortic (inconspicuous)
Intimal Sarcoma on Chest Radiography
Pulmonary Artery Sarcoma (Right)
Intimal Sarcoma: Rad/Path Correlation
Intimal Sarcoma: Rad/Path Correlation
Pulmonary Artery Sarcoma (Left) VIBE post gad
VIBE post gad
VIBE post gad18-FDG-PET/CT
CTA
Intimal Sarcoma: Rad/Path Correlation
Aortic Intimal Sarcoma
• Eccentric endoluminalfilling defect (often low attenuation on CT: easily interpreted as thrombus)
• Lobulated margins
• Contrast enhancement
• Complete vessel occlusion (more typical of PA sarcoma)
Imaging Findings That Suggest Intimal Sarcoma
• Pulmonary embolism without risk factors and absence of deep vein thrombus on ultrasound Doppler (PA sarcoma)
• Central or peripheral embolism without risk factors for thromboembolic disease (Aortic intimal sarcoma)
• Absence of significant atherosclerotic disease (Aortic intimal sarcoma)
• Metastases
Imaging Findings That Suggest Intimal Sarcoma
• 18F-FDG PET avidity*
(*strongly recommended when suspicion for intimal sarcoma is high, based upon clinical and/or radiologic findings)
Imaging Findings That Suggest Intimal Sarcoma
Initial Presentation
Metastatic Disease Disability
Palliative Treatment
Death
months later
CTA or MRA
Misdiagnosed as Thrombus
Identify Suspicious Features
Early Diagnosis
Increased Survival
Identify suspicious features on CT or MRI which suggest great vessel Intimal sarcoma, then recommend 18F-FDG PET/CT.
Treatment Options
Decreased Disability
18F-FDG PET/CT
Proposed Diagnostic Algorithm
AortaPulmonary Artery Both Late Feature
PA
PA
Aortic
Intimal Sarcoma Features by Total Combined Occurrence: Literature, AIRP, & UMMC
Suspicious Features Quantification Summary
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Thank you for learning with us!
Michael Morris, M.D., M.S.Corresponding author: [email protected] of Maryland School of Medicine/Medical Center Department of Diagnostic Radiology and Nuclear Medicine22 South Greene StreetBaltimore, MD 21201410-328-5700
Aletta Ann Frazier, M.D.Senior author: [email protected]
The authors would like to acknowledge the University of Maryland Medical Center Department of Diagnostic Radiology and Nuclear Medicine, the American Institute of Radiopathology and the American College of Radiology for providing the rich academic environment to make this work possible.
