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27 Aortoenteric Fistula (Gastric, Small Intestine, Colonic, Biliary)
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the more chronic stula patient, oral nutrition may be
resumed in these patients as they have been eating and tolerating the passage of food prior to their admission. However,
we do not feed the patient with the rst acute episode until
they are stable and treatment initiated. Since using the above
approach, the treatment of aortoenteric stula patients has
shown a remarkable improvement in patient recovery and
long-term survival results. Using this approach, each of our
patients has survived the procedure to lead a quality life
unless they had other simultaneous non-stulous health processes. These patients demonstrate the challenge of polymicrobial infections in vascular surgery. Both gram-positive
and gram-negative bacterial organisms and fungi may be
present at the time of the stula culture. We favor the in situ
or endograft approach except in the very unusual patient
where an extra anatomic graft may be utilized as some advocate [19].
If one could predict which patients would develop a stula, different surgical techniques might be utilized. When
repairing aortic aneurysms or performing aortoiliac grafts,
one needs to avoid the possibility of entering the bowel
during dissection. We have closed the abdomen and awakened the patient upon inadvertently entering the bowel in
elective patients—to return another day to perform the
vascular surgery—in hopes of avoiding graft infections.
Further, vascular grafts under tension should be avoided to
prevent erosion of the contiguous tissue—such as the ureter or bowel [20]. Also when closing, the abdomen, the
synthetic graft should be protected from direct contact
with the surface of the bowel, to prevent or reduce the possible formation of pressure necrosis and a stula. Placing
the synthetic aortic bifurcation graft limbs beneath the ureters and covering the graft with aneurysmal wall, peritoneum and omentum should reduce the chance of stula
formation. When urgency dictates, we have utilized a
transthoracic or a subdiaphragmatic approach for proximal
control of the aorta as temporizing blood-sparing
approaches in the critical patient. Each of these methods
however may add other potential complications and thus
are avoided when possible. We abandoned suture closure
of the distal aortic stump as we developed the current technique and have had no aortic or iliac vessel disruptions
since utilizing this approach.
When closing the intestinal defect, we have utilized a
two-layer absorbable suture closure when possible with all
potentially ischemic, irregular, or possibly infected material
resected or inverted into the lumen of the small bowel or
colon. If appropriate, edges of the intestinal defect may be
debrided along with any necrotic material. It has been interesting to nd that in some patients the stula is more mature
and the surrounding tissue is not necrotic but more brotic in
nature. Also depending on the stula type and the location of
the stula in reference to the graft, there may be areas of the
aortic graft with no evidence of contamination or necrosis.
The area around the stulous tract may be densely adherent
and brotic with no liqueed pus. On occasion, in these situations, when further dissection was felt to be highly risky, a
short ring (1/2–3/4 inch) of previously placed prosthetic
graft has been left in place along with prior sutures. We then
suture the new prosthesis to the previous ring of the residual
Dacron prosthesis.
In the instance of a primary infected aorta, we have
resected the necrotic infected aorta and placed a prosthetic
graft wrapped with omentum. These septic patients are critically ill and, consequently, carry a high morbidity and mortality risk. Certainly, repeat infection of the graft in these
patients is a consideration but, fortunately, we have not
encountered this concern.
Clamping of the aorta and the iliac vessels for the duration (up to 5.5hours) of the operation has uncommonly led
to limb ischemia and amputation unless previous ischemia,
occlusion, or clot were present. Blood and stula cultures
along with cultures from about the graft have all been
acquired to direct antibiotic treatment postoperatively.
Secondary infections have not been a concern in our experience. Placement of omentum between the repaired duodenum and aorta to avoid direct contact and decrease the
possibility of repeat aortoenteric stula formation is a
necessity.
We began using the endovascular stent (endograft) stabilization of the hemorrhaging stula patient in 2002 [12]. In
the selected patient, we have felt this was not only appropriate but life-saving. Some surgeons have reported long-time
use of this approach without subsequent resection of the initiating or infected graft. We have taken the approach that the
stula needs to be closed (bleeding controlled), and in the
future the previous infected graft resected, the endograft
removed and a new replacement graft inserted. Other options,
utilizing the endovascular repair for control of the bleeding,
have included insertion of an intra-aortic balloon catheter
and attempts at embolization. We have not used these techniques nor have we placed postoperative sump drains about
the graft. Necrotic material and pus have been found on these
grafts at surgery, but no large abscess has been encountered
by us. Our approach has been to use the endovascular or
EVAR graft in the select patient as a short- or possible
longer- term treatment. This may be for a few days or a few
months, but not necessarily for the duration of one’s life
unless the patient’s other medical conditions contradict further intervention.
The patient with primary aortoenteric stula presents a
different concern because of the friability of the aorta and
the cause of their original sepsis. Thus, the risk for additional complications seems to be much greater in these individuals, including mortality. Postoperative intensive care
monitoring, stabilization, and treatment will include blood

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counts, temperature checks, blood cultures, vital signs, and
frequent physical exams in these individuals.
Prognosis
The lesions herein described present the patient and the
treating team a difcult and high-risk situation. Left
untreated, the mortality is almost 100%. When treated, the
complication rate is great, but the possibility of survival
and success are present [17]. In our experience, by using
the newer techniques of endografting, proper vascular
grafting and placement techniques, appropriate antibiotics,
omental wraps and patient-centered nursing care, these
patients have a reasonable chance for survival [21]. But
reports of post-EVAR aneurysm therapy have shown that a
0.8% of the patients (32 of 3932) developed an aortoenteric
stula within 18.5 months of aneurysm treatment, especially when EVAR was utilized for pseudo- aneurysm or
emergency therapy [22]. Whether the spiral saphenous vein
reconstruction of the infected aorta by Heyligers and Vriens
will prove superior to the endograft will require further
evaluation [23]. Similarly, other research, such as lithoplasty, will require time for analysis [24]. All of the studies
will be observed for the restrictive value in blood transfusion and, in morbidity, mortality, and cost [25].
Unfortunately, many of these patients have multiple
simultaneous disease processes which require concomitant
treatment. Amputation of an extremity, renal failure, prolonged intubation, bowel concerns, and long-term respirator
care are all considerations. With the current aggressive multidisciplinary therapy programs, the mortality rate has been
reduced, and long-term survival may be available to a large
percentage of these patients [26, 27]. Infections, a challenge
of vascular surgery, and other potential complications continue to be monitored [26].
Along with medical therapy, the expense of treatment
of these patients continues to rise, unfortunately placing
an additional burden on the patient, the family, and the
treating medical team. These stulae are an uncommon
but life- threatening process requiring diagnosis and treatment on an urgent basis—many within minutes or hours.
The associated age and medical condition of these patients
have a great impact on their potential outcome. Another
possible stula might be an aortoappendiceal communication. We have never seen one of these, but literature review
has demonstrated a few articles regarding aortoappendiceal stula formation (A-A-F). Rectal bleeding seems to
be a major sign of the A-A-F.As a result, colonoscopic
examination has been used in the diagnosis of such a stula [28]. These stulas may also arise from the chronic,
infected, and contained rupture of an abdominal aortic
aneurysm [29].
Bronchobiliary and gastrobiliary stula are uncommon
but may occur in the young or the adult patient [30]. We
have not seen a patient with an aortobiliary stula, but arteriobiliary stulas do occur on the rare occasion [26].
Kawakami etal. have reported their experience, utilizing a
fully covered self-expandable metallic stent placed endoscopically to treat an arteriobiliary stula [30]. Fedakar
etal. published in 2011 an aortobiliary stula in their report
of 93 patients with an abdominal aortic aneurysm [27]. We
have not encountered another possible stula, an aortoappendiceal communication, but we have found references in
the literature. Prosthetic aortic graft intervention may also
lead to multiple aortic stulas in the same patient and
includes the aortoenteric (e.g., duodenal) and aorto-caval
stula. With the new and unusual approaches, including the
transvenous approach to the aorta and cardiac lesions (e.g.,
valvular lesions), it is assumed more such stulas will
develop.
Anesthetic Consideration
It goes without saying that a competent anesthesiologist and
OR team are very important to the provision of successful
major vascular surgery, especially when that surgery involves
the aorta and aortic complications. This capability requirement involves both the open aortic procedures and the closed
or endovascular procedures. Various techniques have been
espoused for the multiple requirements to accomplish stabilization and treatment of patients with aortic lesions. The
elective correction of aortic lesions may be approached much
differently than the emergency patient in shock or receiving
cardiopulmonary resuscitation.
Thus, in our experience, the patient in critical condition
must have respiratory and cardiac control as the primary goal
during the resuscitative efforts. Endotracheal intubation
along with venous access (usually two or more) to maintain
oxygenation, to improve or obtain a blood pressure, blood
infusion, cardiac monitoring and attempts to stabilize the
patients are primary. Additional assist considerations such as
an A-line, central venous access, antibiotics, and TEE may
all be considered when the situation is better controlled.
In the elective prior planned surgery in a patient with an
aortic lesion, preoperative planned programs progress at a
different speed and utilization. In these patients, when possible, an anesthetic consultation before procedure should
clarify some of the desired steps to be utilized. In these
patients, preoperative antibiotics, blood typing and screening, and the possible requirement for a central venous access
are all determined. In the OR or special procedure room, the
requirement for or not of an arterial line or TEE (transesophageal echo) may be assessed along with the possibility of an
epidural (including possible bleeding considerations).

27 Aortoenteric Fistula (Gastric, Small Intestine, Colonic, Biliary)
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Postoperatively, the patient is usually taken to the recovery
unit and then the intensive care unit as necessary. Nasogastric
intubation may or may not be necessary.
Of note, more recently, an increase in deaths due to
abdominal malignancy have been reported following aortic
endograft procedures for abdominal aortic aneurysm therapy. These malignancy deaths are presumably due to the
increased radiation dosage received during endograft implantation and the post procedure serial studies of the endovascular implanted aortic aneurysm grafts.
Acknowledgments We appreciate the assistance of the librarian Julie
Stielstra, Library Director of Northwestern at Central DuPage Hospital
Knowledge Resource Library in Wineld, Illinois, and that of the chapter organizer and typist Lynn Murawski. This chapter is dedicated to
Robert E.McCray, M.D., and Glen H.Asselmeier, M.D., co-founders
of the Glen Ellyn Clinic LLC and the Department of Surgery (general,
vascular, and cardiovascular surgery).
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13. Tabach TJ, Kane PN, Madjarov JM, Halleman JH, Robischele F,
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Kim YT.Minimally invasive surgical repair for congenital bronchobiliary stula in an adult. Ann Thorac Surg. 2016;101:1584–7.
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aortoenteric stulae after EVAR. London: Vascular News (BIBA
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28. Church JM, Lavery IC, Beven EG.Colonoscopic diagnosis of an
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30. Kawakami H, Okamoto M, Kumatani M, Kubota Y, Kawakuloo
K, Abe Y, Kawalata S, Kubo K.Endoscopic placement of a fully
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https://doi.

Malignant andBenign Aortic Tumors
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RaymondA.Dieter Jr., GeorgeB.Kuzycz,
RaymondA.Dieter III, JessicaGulliver,
andRobertS.Dieter
28
Malignancy represents one of the more common afictions of
the human body, but benign or malignant tumors of the aorta are
very rare. Several causes or etiologies of tumors have been
dened and related to various human habits or acquired infection. Smoking and herpes infections represent two of the etiologic agents related to tumor inducement in the adult human.
Neither of these, however, has been associated with primary vascular tumor development nor has any other dened risk factor.
Malignancy of the vascular tree is very uncommon to
rare. Rarely, if ever, are primary or secondary malignancies
of the major arterial or venous systems encountered by the
average vascular or thoracic surgeon. Certainly, as tumors
develop, nutrition for the growth and multiplication of the
malignant cells must be provided by the surrounding arterial
system. But the vascular network providing such necessities
is usually of the smaller arteriole and venous system.
Only on occasion does the neoplastic process involve the
larger vessels. When this occurs, the patient may develop
either acute or chronic symptoms related to the vessel(s)
involved. When tumor involvement occurs in the lung, hemoptysis may develop. When tumor involvement of the major vessels to the extremities occurs, either acute or chronic extremity
ischemia may create major consequences for the patient [1]. In
the latter group, major peripheral vessels, the heart, or the
R. A. Dieter Jr. (*) ∙ G. B. Kuzycz
Cardiovascular and Thoracic Surgery, Northwestern University at
Cadence Health Emeritus, Wineld, IL, USA
R. A. Dieter III
University of Tennessee Medical Center, Cardiovascular and
Thoracic Surgery, Knoxville, TN, USA
J. Gulliver
Department of Pathology, University of Wisconsin Hospital
and Clinics, Madison, WI, USA
R. S. Dieter
Interventional Cardiology, Vascular and Endovascular Medicine,
Loyola University Medical Center, Maywood, IL, USA
aorta may be the source for the ischemic process by either
embolic, obstructive, or constrictive mechanisms.
Aortic tumors, either primary or secondary in origin, are
very uncommon, and thus, many physicians, including cardiovascular surgeons, have never had the opportunity to treat
patients with these concerns. The original presentation may
be confusing and thus suggest other non-neoplastic disease
entities as the cause for the patient’s complaints. Even initial
diagnostic testing may be misinterpreted or suggestive of a
non-neoplastic etiology for the patient’s complaints, as discussed in the Critical Extremity Ischemia book [1]. However,
both primary and secondary aortic tumor involvement does
occur and, in most instances, creates major diagnostic and
therapeutic challenges.
Primary Aortic Tumors
Lesions involving the aorta are common, and their symptomatic results are familiar to both the healthcare profession and
the lay public. To mention that an individual has an aortic
process affecting their health immediately brings forth an
image of possible risks and the possible result. The aorta is
well recognized as a major structure by the medical and nonmedical community to be necessary for the development and
maintenance of one’s body and its function. The usual aortic
aneurysm rupture or occlusive concepts are generally understood. However, discussion with a pathologist of the incidence of primary or secondary aortic neoplasm involvement
is usually met with a pause and then, “I don’t believe I have
ever seen such a tumor.” They then will discuss the occasional hemangiopericytoma, and similar small vessel tumors,
or the benign/malignant endocardial tumors which may
embolize. Both of these examples are much more frequent
than the aortic neoplasm and still are not common.
Aortic tumors may be classied in a variety of manners
depending on the type of classication to be utilized. Lesions
may be categorized as malignant or benign. They may be
categorized according to their location in the aorta, or they
© Springer Nature Switzerland AG 2019
R. S. Dieter et al. (eds.), Diseases of the Aorta, https://doi.org/10.1007/978-3-030-11322-3_28
385

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may be grouped according to symptomatology. The major
pathologic tumor types that one may encounter directly
involving the aorta are as follows.
Types of Benign and Malignant Aortic Tumor
Classications
I. Primary
A. Malignant (sarcoma—most common patho-
logic diagnosis)
1. Angiosarcoma (25%)—epitheloid
2. Pleomorphic sarcoma
3. Intimal sarcoma (27%)—actually dene
location
4. Malignant brous histiocytoma (15%)
5. Leiomyosarcoma (14%)
6. Undifferentiated/high-grade sarcoma (9%)
7. Fibromyxosarcoma
8. Myobrosarcoma
9. Hemangiopericytoma
10. Chondrosarcoma
B. Benign
1. Myxoma
2. Lipoma
II. Secondary/metastatic involvement
A. Lung
1. Non-small cell
(a) Squamous cell
(b) Adenocarcinoma
(c) Large cell
2. Small cell
B. Esophageal
C. Retroperitoneal lymphoma
D. Mediastinal
1. Malignant thymoma
2. Malignant germ cell
III. Associated tumor
A. Fibrous histiocytoma
B. Cardiac embolic
IV. Benign lesions—only a small percentage of the
total aortic tumors
A. Localized polypoid lesions
B. Thrombotic mass-like lesions
The most frequently reported tumor involving the aorta
is the sarcomatous group. Primary epithelioid angiosarcoma is the most frequently reported primary malignancy
of the aorta, and non-small cell lung carcinomas (squamous cell and adenocarcinoma) are the most frequent met-
astatic lesions involving the aorta. Less frequent
malignancies and other very uncommon lesions are also
diagnosed. The primary aortic tumor may metastasize to
other locations.
Primary Aortic Tumor Metastatic Sites
I. Intraluminal—embolic
A. Legs
B. Mesentery
C. Kidneys
II. Periaortic—direct
A. Thoracic
B. Retroperitoneal
III. Hematogenous
A. Bones
B. Skin
C. Organs
IV. Liver
V. Spleen
VI. Lung
Most reports of primary aortic tumors are of single or a
small number of malignancies involving the aorta [2–19].
The paucity of series reports further demonstrates the rarity
of this disease. The sarcoma groupings (epithelioid; pleomorphic; angio-, bro-, myobro-, leiomyo-, and undifferentiated sarcoma groupings) are varied in frequency,
occurrence, and appearance. Less commonly reported are
the brous histiocytomas [18]. But, all these sarcomas are
highly malignant with a guarded prognosis. Lipomas are the
most common benign aortic tumor [6, 9].
Similarly, the secondary or metastatic malignancies of the
aorta also carry a guarded or unfavorable prognosis. These
lesions usually occur in the 50- to 70-year age group and may
result primarily from the pulmonary or bronchopulmonary
group of carcinomas (CA of the lung) or esophageal carcinomas. All cell types of the lung have been involved. These
include both the small cell and non-small cell malignancies,
including the large cell, the adenocarcinoma, and the squamous cell tumors [20–31]. Malignant thymomas and germ
cell involvement have also been known to involve the aorta.
The primary aortic tumors also metastasize in a fashion
similar to many of the other malignant tumors which an individual may develop. The primary tumor, especially the sarcomas, initially may present with both local and distant
spread. One of the more common aortic tumor presentations
is the distant embolic phenomenon of the tumor cast or secondary clot with occlusion of the iliofemoral or enteric systems, especially creating sudden secondary ischemic

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symptoms of the abdomen or legs [3]. Also, periaortic direct
local extension of the malignant thoracic or retroperitoneal
aortic lesions is a natural consequence of these malignancies.
Similar to many other malignancies, hematogenous spread to
the bones, liver, and spleen is reported [3, 7, 17, 19]. Some
patients have also developed multiple palpable skin or subcutaneous metastatic nodules [7, 10].
Aortic Tumor Symptomatology
As with so many diseases, development of one or more
symptoms may direct an individual to nding the primary
disease entity almost immediately after development of
the symptom. But as with many disease entities, the presentation, the symptom complex, and the patient’s history
may be both confusing and misleading. The literature
demonstrates that both the diagnosis and the potential suggested treatment routes by the physician have frequently
been led astray due to the lack of specicity of symptoms
and diagnostic studies. Such a dilemma occurs with both
primary and secondary tumor involvement of the aorta.
The symptomatology most commonly revolves around
four main areas: (1) embolic, (2) occlusive, (3) pain, and (4)
generalized or systemic.
Potential Aortic Tumor Symptomatology
I. Embolic symptoms
A. Acute
B. Chronic
C. Loss of function
II. Occlusive—progressive
A. Abdominal aorta
B. Coarctation/dissection type
III. Pain—sudden or progressive
A. Abdominal
B. Extremities
C. Thoracic
IV. Generalized/systemic
A. Weight loss/fatigue
B. Fever
C. Night sweats
D. Nausea
E. Anorexia
V. Renal—infarction
VI. Clubbing—hand
VII. Neural
A. Horner’s syndrome
B. Hemiplegia
VIII. Extremis
Embolic symptoms may develop in both the upper and
lower extremities and in the abdomen. Most commonly, the
embolic tumor or clot may produce acute symptoms with
sudden and total occlusion of the recipient distal artery.
Symptoms Due to Embolic Aortic Tumor
I. Extremity
A. Ischemia
B. Claudication
C. Acute or chronic pain
D. Loss of function
II. Skin
A. Pallor
B. Hematogenous lump or rash
III. Stroke
A. Unable to speak
B. Major paralysis
IV. Coronary occlusion
V. Buttock pain
VI. Gastrointestinal—ischemic bowel
Depending on the artery involved, the patient may develop
stroke, severe acute leg pain, renal concerns, or skin lesions
[2, 3, 8, 11, 18, 32, 33]. In the legs, the acute embolic pain
may be sudden and excruciating with pallor and loss of function. Or, with smaller emboli, the pain may be slow and
insidious in onset depending on the vessel occluded and size
of the clot or tumor embolus. Abdominal symptoms also
may be sudden and catastrophic with embolization to the
enteric (e.g., superior mesenteric artery) or renal vessels. The
patient who develops a sarcoma in the abdominal aorta may
have progressive and confusing discomfort similar to that of
an aortic aneurysm.
Pain due to a thoracic aortic tumor may resemble that of
an expanding aortic aneurysm. The tumor may also embolize distally—downstream—or more proximally to cause a
stroke [11, 32]. The distinction of a primary tumor-induced
cerebral infarction from a stroke due to aortic dissection
may be difcult even after diagnostic studies. Retrograde
occlusive coronary artery involvement has also been demonstrated acutely in a patient [16]. The thoracic aortic
tumor symptoms may also present with pain similar to an
acute dissection [7]. Horner’s syndrome along with hemiplegia was also reported in the patient with acute coronary
[16]. Less common symptoms may result from stulization
into the bronchus, vena cava, and esophagus along with
gastrointestinal bleeding.
Other systemic symptoms include weight loss and fatigue
in many of the patients. Nausea, unilateral clubbing, night

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sweats, and fever may suggest other maladies in these
patients.
Associated Symptoms and Risks of Aortic Tumor
I. Concommitant—atherosclerosis
II. Age—40s, 50s, 60+
III. Sex—more common in males
IV. Hypertension—resistant to diagnosis/treatment
V. Aneurysm formation
A. Leakage—descending thoracic
B. Multiple
VI. Smoker/cough
VII. Death—acute
A. 1year—most
B. 3+ years—more than 90% deceased
The patients tend to be older, over 60years of age. A
few are in the 40- to 50-year age range, and the age range
of occurrence is from 3months to 82years [5, 32]. It also
seems that the male gender predominates, with only a few
females reported with these tumors [16, 19]. Single or
multiple aneurysm formations with suspected leakage
present diagnostic concerns. Elevated blood pressure has
been found in a number of these patients as has the smoking habit.
Morbidity is great in these patients, and the aortic
tumor diagnosis suggests a highly morbid outcome. As a
group, these sarcomatous lesions are highly fatal. The
individual may present acutely in a moribund state, and
surgery in this acute state is very risky. By the end of 1
year, a large portion of the patients have passed away
despite therapy. A few patients survive up to 3 years.
However, there is 90% mortality by the end of 3years [7].
The survivors are primarily patients with low-grade
malignant or benign tumors.
Aortic Tumor Diagnosis
As already mentioned the patient’s symptomatology and
ndings may be difcult to sort out and confusing. The
patient’s history may be classical for other more common
lesions and lead the physician down the wrong diagnostic
pathway. Thus diagnostic testing options and consultants
may be led astray by the patient, the history, the physical
ndings, and the diagnostic studies. The sorting out of perti-
nent historical and testing interpretations may prove
difcult.
But, symptomatology and physical ndings may lead the
diagnostic and treatment approach in the correct direction.
Even then, the diagnosis may not be established until after
therapeutic intervention has occurred. Such is the case, for
example, when a thrombus has been removed from the lower
extremity and the pathologist, a few days later, forwards the
microscopic ndings and pathologic diagnosis [1].
The usual initial approach to the patient’s problem is to
obtain a history from the patient or of an accompanying individual regarding the patient’s complaints. This is followed
by a complete physical examination—head to toe. Having
assessed the situation, this initial assessment will guide the
number and type of studies to be obtained. Initially, a CBC
(complete blood count) and x-ray may be ordered to help
differentiate the problem. With the modern-day emergency
room programs, most of these studies will be obtained in the
emergency room (ER) along with other blood tests and
radiologic scans.
The physical exam may demonstrate a relatively stable
and less emergent situation or may dictate urgent or emergency diagnostic testing, blood typing, consultation with
potential diagnosticians, and intravenous access which may
all be required during the initial evaluation.
Laboratory testing may show a marked anemia, an elevated sed rate, an increased C-reactive protein (CRP), or an
increase in the number of white blood cells (WBCs). All of
these are nonspecic and may be misleading. The physical
diagnosis may reveal a stable nondistressed individual or a
patient in severe pain. He/she may be pale, febrile, and
tachycardic and have a heart murmur. The legs may be pale
and pulseless or immobile. The patient may have severe
abdominal or chest pain and be in extremis.
The skin may contain multiple nodules and petechiae.
The patient may be aphasic or hemiplegic. The abdomen
may be tense and distended. A Horner’s syndrome or ocular disturbance may be present. Urine may be abnormal
and the BUN and creatinine elevated. The blood pressure
may be elevated and vary between the right and the left
arm [33]. Evidence of weight loss and clubbing may be
obvious to all.
Testing will proceed along the most likely and appropriate route to establish the diagnosis and course of treatment.
Different ndings and results may lead to the best and most
expeditious course of treatment. Less complicated studies
such as an arterial Doppler, electrocardiogram (EKG), and
emergency room (ER) echocardiogram may all be obtained
with varying results.

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Diagnostic Studies to Delineate Aortic Tumors
I. Radiologic imaging
A. CT (computerized tomography)
1. May be misleading
2. CT angiogram
B. MRI (magnetic resonance imaging)
1. Gadolinium
2. MRA (magnetic resonance angiogram)
C. Angiography
1. Arterial—diagnostic
2. OR (operative room)
3. Venography
D. PET scan (positive emission)
II. Biopsy/tissue
A. Microscopic
1. Mass
2. Skin
B. Immunohistochemical (best)
C. Vascular system
D. Surgical specimen
III. Lab—CBC—nonspecic
A. Increased sed rate
B. CRP (C-reactive protein) increase
C. WBC (leukocyte count) increase
D. Video microscopy
IV. Echo
A. TEE (transesophageal echocardiography)
B. Cardiac Doppler
C. Arterial ultrasound
V. Autopsy
Many of the studies performed in these patients are presented above. A fairly frequent approach may include an
emergency CT (computerized tomographic) scan. These may
be misleading or misinterpreted as an aneurysm or thrombus
due to the rarity of a primary aortic tumor [2, 12]. A CT angiogram, with intravenous contrast, may follow due to the lack of
an established diagnosis [2]. Others have performed magnetic
resonance imaging (MRI) in an effort to delineate the patient’s
diagnosis and extent of disease. MR angiography and the use
of gadolinium have been advocated in the diagnostic “tree”
due to the inability to differentiate lumen from the arterial wall
[3, 8, 16, 32]. None of these scans have proven sufcient in all
situations, and thus, angiography including operating room
(OR) angiography has also been required [8]. Distal aortic
angiography has been utilized to rule out a Leriche syndrome
[15]. Using these techniques and transvascular angiography,
one must guard against distal embolization [16].
389
Due to the difculty in diagnosis of these patient’s problems, other studies have included a TEE (transesophageal
echo) and temporal artery biopsy [3, 16, 31]. PET (positive
emission tomography) has further been utilized to delineate
the extent of the patient’s disease—especially in suspected
metastatic disease. Skin or nodule biopsy may prove confusing and diagnostic. Obviously tissue biopsy and associated
immunohistochemical studies are the most accurate diagnostic studies available. But this requires obtaining tissue and
the recognition that tissue is necessary [17]. Even video
microscopy has been utilized.
Preoperative biopsy of aortic tumors or transarterial tumor
biopsy has been discussed in the literature. In the past, 17
gauge 7 1/2 inch translumbar aortography was a routine and
rapid testing procedure for aortic, renal, or iliofemoral disease with little morbidity in our experience of its usage in
hundreds of patients. This technique has been largely supplanted by catheter angiography for diagnosis and treatment.
Transvascular biopsy techniques have been developed for
multiple disease entities such as liver and renal disease, or
cardiac rejection [34–36]. This transvenous approach for the
biopsy of neoplasia has been utilized for cardiac tumors, retroperitoneal tumors, cavoatrial tumors, and pancreatic
tumors [37–39]. Despite the availability of transarterial and
transvenous needle and catheter techniques, the use of these
approaches is uncommon for aortic tumors. The nal tissue
diagnosis usually does not occur until the surgical exploration and removal of the tissue specimen. The nal, but unfortunate, diagnostic approach may be an autopsy in the difcult
situation. Utilization of transarterial or transaortic biopsy
techniques has not gained favor for the diagnosis of primary
aortic tumors due to the embolization concerns [16]. Also,
arterial endoscopic techniques have not been utilized for
diagnostic purposes in these patients.
Dierential Diagnosis
As already presented, diagnosing and differentiating the true
nature of an aortic neoplasm may be very difcult and the
correct pathway to the tumor denition tortuous. The
patient’s symptoms may be confusing, misleading, and
strongly suggestive of other disease entities.
Concurrent preexistent disease and treatments may confuse the issue and mask the underlying sarcoma. Depending
also on whether the aortic tumor is primary or metastatic
from a non-arterial primary tumor may cloud the diagnosis
and even be difcult to diagnose under the microscope. Also,
a benign primary aortic tumor may present differently than a
malignant aortic tumor.

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Consulting radiologists or cardiovascular surgeons may
misinterpret the history, physical, laboratory studies, or radiographic evidence as to the true etiology. Cardiac murmurs or
aortic bruits may suggest underlying vascular disease, such as
mitral regurgitation with valvular prolapse or a chronic Leriche
syndrome. A lateralizing stroke can suggest aortic dissection
or cardiac emboli. Abdominal pain with distention and bruit
may imply atherosclerotic disease—especially if previous history is compatible. Some of the disease entities that may be
misapplied to the patient’s symptoms are the following.
Dierential Diagnosis of Aortic Tumors
I. Valvular heart disease—mitral regurgitation
with prolapse
II. Aortic occlusion—abdominal/Leriche
III. Primary intra-aortic thrombus
IV. Horton’s disease
V. Infections
A. Mycotic aneurysm
B. Positive cultures
VI. Lung cancer—in association
VII. Anatomy: congenital
A. Right aortic arch
B. Confusing
VIII. Multiple malignancies
The presence of chronic symptomatology due to other
nontumor lesions may suggest superimposed acute progression of a vascular process, such as arterial or aortic atherosclerosis of the aortoiliac vessels, rather than an embolic
tumor thrombus. A suspected mycotic aneurysm may be pursued with blood cultures, angiography, and antibiotic therapy
[3, 7]. When other lesions exist, such as bronchopulmonary
malignancy, the aortic association or involvement may be
difcult to differentiate. Bronchoscopy may yield the tumor
diagnosis, but not dene the aortic concern. Similarly, congenital aortic arch and branch formation such as the right
arch and descending aorta with aneurysm formation may further cloud the diagnosis [25, 27].
Multiple malignancies may also confuse the physician
[32]. Development of primary angiosarcoma in a previous
aortic graft will be confusing [40]. Other surgical procedures, such as cholecystectomy, have been performed while
attempting to improve the patient’s condition—without benet [41]. Presence of exophytic-calcied atheroma may, but
not correctly, suggest the presence of a benign papillary
broelastoma [42]. Further, oating thrombus or polypoid
thrombus may also be found distal to the aortic valve and
produce confusing distal systemic symptoms.
Similarly, one must carefully review the CT scan, MRI, or
other studies to eliminate the possibility of another nonaortic origin site of tumor such as the left ventricle or other
cardiac locations—even in infants [43]. The diagnosis may
be further confusing when infectious complications develop
during the course of the disease.
Aortic Tumor Location/Classication
The aortic tumors (mostly of mesenchymal origin) may occur
in any location from the aortic valve to the aortic bifurcation.
The type of tumor, benign or malignant, denes to some
degree the primary location of the tumor. If the broadest tumor
denition includes a mass of any type, then the oating thrombus or polypoid aortic thrombus seen in the ascending aorta,
especially just above the aortic valve, probably represents the
most common aortic tumor [44]. The subsequent distal
embolic symptoms may also be confusing as to their origin.
Echocardiography has proven helpful in this determination as to site of origin. These lesions demonstrate one of the
growth patterns of aortic tumors, namely, the intraluminal
form. Three forms of aortic tumor growth have been dened
morphologically: (1) intraluminal, (2) intimal, and (3) mural
(to include adventitial) [41].
Location of the tumor has also been delineated anatomically as to the level of the aorta involvement. Forty-six percent of the tumors are located in the chest, 25% as
thoracoabdominal, and 27% in the abdominal aorta [7].
Further localization to the aorta is shown below. The transverse aorta and arch seem to be particularly prone to the primary aortic tumor and also may be confusing when they
create a dissection. The descending aortic lesions may be
subclassied into proximal, mid, and distal thoracic lesions
and include both primary and metastatic malignancies.
Aortic Tumor Location and Dierential Diagnosis
I. Ascending aorta
A. Lipoma—adventitial
B. Polypoid thrombus—endoluminal
II. Arch—especially transverse aorta
A. Atheroma
B. Dissection
III. Descending thoracic aorta
A. Proximal—primary aneurysm
B. Mid-thoracic
C. Distal thoracic
IV. Abdominal
A. Suprarenal
B. Aneurysm
C. Paravascular—renal occlusive
D. Infrarenal
1. Leriche
2. Intra-aneurysm
V. Metastatic sites—bone, liver, adrenal, and lung

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Intra-abdominal aortic tumors may also be subclassied according to the aortic lesion location. These tumors
may be suprarenal, paravascular, or infrarenal, and symptoms may vary according to location—for example, they
may cause renal artery occlusion. The general anatomic
locations for the tumor-originating site can thus have
some effect on the type of symptoms a patient may
develop. For example, the ascending and arch locations
may produce pain and stroke type of symptoms while the
descending or abdominal primary sites may produce
renal, gastrointestinal, systemic, or lower extremity
complaints.
Non-aortic location of aortic tumor metastases may
develop in multiple organs throughout the body. These sites
include the bones, liver, kidneys, adrenal glands, and lung.
Non-aortic primary tumors involving the aorta further confuse the issue as to the symptoms and diagnosis. The most
common of these invasive tumors causing aortic involvement include the bronchopulmonary carcinomas and esophageal and the retroperitoneal tumors, which have a direct or
contiguous contact with the aorta. This further confuses the
diagnostic and treatment attempts. To date, however, we
have not seen direct tumor invasion requiring aortic resection due to mesothelioma nor malignant thymoma.
Aortic Wall Involvement
These tumors may involve any layer of the aorta.
Microscopically, they may be difcult to diagnose and to differentiate as to the cell type and point of origin. We have
already mentioned the primary locations from which these
tumors originate. Additional classications exist as to the
type and site of initial growth.
Location of Tumor in Aortic Wall
I. Intraluminal thrombus—polypoid
A. Obstructive
B. Embolize
II. Endothelial
III. Intimal
A. Tunica intima
B. Usually descending thoracic or abdominal
C. Prone to embolize
IV. Intramural
A. Extravascular dissection
B. Media—extravascular growth may cause S
and S
C. Adventitia
V. Aortic branches—subclavian aneurysm
The most common point of origin seems to be the intimal
or tunica internal layer [3, 5, 19, 33]. Because of their origination site, many of these tumors may grow into the aortic
lumen and produce polypoid-type lesions that may further be
classied as obstructive or nonobstructive. It seems that
these lesions are more commonly located in the descending
thoracic and the abdominal aorta. This tendency may lend
itself more to the formation or creation of emboli in association with narrowing of the aorta. Primarily polypoid lesions
may also be felt to originate in the endothelium [17, 19].
The intramural-originating aortic tumors (18%) may lead
to dissection or a differential diagnostic concern resulting
from dissection like ndings as a result of their media origin.
Extravascular growth from tumors originating in the media
or adventitia usually creates greater mass-like tumors [16].
Ten percent of the malignant aortic tumors involve all three
layers of the aorta. The intraluminal benign mass lesions are
usually of thrombus or plaque in origin. Whereas the benign
lipomatous lesions usually arise in the adventitia or in the
outer layer of the aorta, major luminal encroachment may
and does occur with most of the malignant aortic tumors.
Such encroachment may lead to vascular occlusion as a
result of growth, thrombus, or embolus. When the tumor
involves the vessel media in and about the major cerebral
circulation, cerebral ischemia or infarction may then create
preoperative uncertainty as to the diagnosis.
Secondary complications due to the tumor depend in
many instances on the wall location of the primary tumor
origin. Stroke, renal infarction, and other embolic sites may
result from the intraluminal lesion embolus, intramural
growth and dissection, as well as additional adventitial mass
formation. Such mass formations may thus be primary or
secondary in origin. Nonprimary malignant growths involving the aorta primarily originate from contiguous invasive
malignancies such as the pulmonary or retroperitoneal
lesions. Periaortic contiguous tumor masses may be malignant—such as lymphoma or carcinoma of the lung—and difcult to diagnose and treat. Such lesions may be benign,
partially calcied, and delineation from an actual aortic
aneurysm, small vessel aneurysm, or benign tumor lesion
vexing (Fig.28.1).
Treatment ofAortic Tumors
Successful treatment of the patient and his/her disease usually depends on having a correct initial diagnosis. With
knowledge of the type of process aficting the patient,
appropriate therapy may then be initiated. Medical, oncologic, and surgical treatments are all utilized when the diagnosis indicates. Unfortunately, with the malignant tumors of
the aorta, diagnosis is often not established early in the process and, in a large number of the patients, only established
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