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Benign Tumors of the Esophagus:

Radiologic Evaluation
Marc S. Levine

Benign tumors of the esophagus can be classified as mucosal or submucosal in origin. The most
common mucosal lesions include squamous papillomas, adenomas arising in Barrett’s mucosa, inflam-
matory esophagogastric polyps, and glycogenic acanthosis. These benign mucosal lesions can often be
diagnosed on double-contrast esophagrams based on their characteristic radiographic findings. Major
submucosal or intramural lesions include leiomyomas, leiomyomatosis, fibrovascular polyps, granular
cell tumors, and duplication cysts. Despite their infrequency, these lesions also can often be diagnosed
on esophagography and/or CT based on their characteristic radiographic findings. The purpose of this
article is to review in some detail the radiographic features of these various benign tumors of the
esophagus.
© 2003 Elsevier Inc. All rights reserved.

B enign tumors of the esophagus comprise only


about 20% of all esophageal neoplasms.1
Most are small, asymptomatic lesions that are
crete swallows of a low-density barium to evalu-
ate esophageal motility. Finally, the patient rap-
idly gulps a low-density barium to optimally
detected fortuitously on barium studies or endos- distend the esophagus. The double-contrast
copy. However, some patients may present with phase optimizes visualization of the mucosa for
dysphagia, bleeding, or other symptoms. Depend- detection of subtle neoplastic lesions, whereas
ing on the site of origin in the esophageal wall, the single-contrast phase optimizes esophageal
benign tumors may be classified as mucosal or distention for detection of subtle areas of narrow-
submucosal. These two types of lesions have char- ing. The double- and single-contrast components
acteristic radiographic features that are dis- of the biphasic esophagram therefore have com-
cussed separately in the following sections. plementary roles in the evaluation of patients
with suspected esophageal disease.

Technique
Mucosal Lesions
Esophagography is usually performed as a bi-
phasic examination that includes both upright Papilloma
double-contrast views of the esophagus with a Squamous papillomas are uncommon benign tu-
high-density barium suspension and prone single- mors, comprising less than 5% of all esophageal
contrast views with a low-density barium suspen- neoplasms.3 These lesions consist of a central
sion.2 The patient first ingests an effervescent fibrovascular core with multiple finger-like pro-
agent and then rapidly gulps a high-density bar- jections covered by hyperplastic squamous epi-
ium in the upright, left posterior oblique position thelium.4 They usually occur as solitary lesions,
to obtain double-contrast views of the esophagus. ranging from 0.5 to 1.5 cm in diameter. Most
The patient is then placed in the prone, right patients are asymptomatic, but some with larger
anterior oblique position and asked to take dis- polyps may present with dysphagia. Multiple pap-
illomas may be present in patients with a rare
condition known as esophageal papillomatosis.5
From the University of Pennsylvania School of Medicine, Gastro-
intestinal Radiology Section, Hospital of the University of Pennsylva-
nia, Philadelphia, PA.
Radiographic Findings
Address reprint requests to Marc S. Levine, MD, Department of Papillomas are difficult to detect on single-con-
Radiology, Hospital of the University of Pennsylvania, 3400 Spruce
trast esophagrams because of their small size. In
Street, Philadelphia, PA 19104.
© 2003 Elsevier Inc. All rights reserved. contrast, papillomas can readily be detected on
1043-0679/03/1501-00003-0$30.00/0 double-contrast esophagrams, appearing as small,
doi:10.1016/S1043-0679(03)00003-0 sessile polyps with a smooth or slightly lobulated

Seminars in Thoracic and Cardiovascular Surgery, Vol 15, No 1 ( January), 2003: pp 9-19 9
10 Levine

Adenoma
Adenomas are rarely found in the esophagus
because this structure is lined by squamous
rather than columnar epithelium. However,
esophageal adenomas may develop in metaplastic
columnar epithelium associated with Barrett’s
esophagus.8,9 These adenomas are important be-
cause they can undergo malignant transforma-
tion via an adenoma-carcinoma sequence similar
to that found in the colon.8,9 Endoscopic or sur-
gical resection is therefore warranted.

Figure 1. Squamous papilloma. Double-contrast


esophagram shows a small, slightly lobulated papil-
loma etched in white (arrow) in the midesophagus.
Early esophageal cancer could occasionally produce a
similar appearance, so endoscopic biopsy specimens
are required for a definitive diagnosis. (Courtesy of
Harry Allen III, M.D., Norfolk, VA; reproduced with
permission from Levine MS, Laufer I, Tumors of the
esophagus, in Laufer I, Levine MS (eds), Double Con-
trast Gastrointestinal Radiology (ed 2), WB Saunders,
Philadelphia, PA, 1992, pp 157-190)

contour (Fig 1).6 Occasionally, papillomas may be


larger and more lobulated, or they may have a
bubbly appearance as a result of trapping of bar-
ium between the frond-like projections of the
lesion.7 Although papillomas are always benign,
they cannot be differentiated with certainty from Figure 2. Esophageal papillomatosis. Double-contrast
early esophageal cancers on radiographic crite- esophagram shows multiple wart-like excrescences
ria. Endoscopic biopsy or resection of the lesion is throughout the esophagus. Despite the dramatic radio-
therefore required when a papilloma is suspected graphic findings, this patient had no esophageal symp-
on barium studies. Despite its rarity, esophageal toms. (Courtesy of Harvey M. Goldstein, M.D., San
Antonio, TX; reproduced with permission from Levine
papillomatosis should be suggested by the pres- MS. Benign tumors, in, Levine MS, Radiology of the
ence of multiple discrete, wart-like excrescences Esophagus, WB Saunders, Philadelphia, PA, 1989, pp
on the esophageal mucosa (Fig 2). 113-130)
Benign Tumors of the Esophagus 11

Radiographic Findings
Adenomas typically appear on esophagography
as sessile or pedunculated polyps in the distal
esophagus at or near the gastroesophageal junc-
tion (Fig 3).9 Lesions that are larger or more
lobulated have a greater risk of harboring adeno-
carcinoma. Because of their location, adenoma-
tous polyps can sometimes be mistaken for in-
flammatory esophagogastric polyps on the basis
of the radiographic findings (see Inflammatory
Esophagogastric Polyp section). When an ade-
noma is suspected on esophagography, however,
endoscopy and biopsy are required for a definitive
diagnosis.

Inflammatory Esophagogastric Polyp


Although inflammatory esophagogastric polyps
are not true neoplasms, they may be manifested

Figure 4. Inflammatory esophagogastric polyp. Prone


single-contrast esophagram shows a prominent fold
(straight arrows) extending from the gastroesophageal
junction into the distal esophagus as a smooth polypoid
protuberance (curved arrow). This lesion has the typ-
ical appearance of an inflammatory esophagogastric
polyp.

on esophagography by polypoid lesions in the


distal esophagus at or near the gastroesophageal
junction.10-12 These lesions consist of a combi-
nation of inflammatory and granulation tissue
and are presumed to develop as a sequela of
chronic reflux esophagitis.11 Because esophago-
gastric polyps have no malignant potential, rou-
tine endoscopic resection of these lesions is not
warranted.12

Radiographic Findings
Figure 3. Adenomatous polyp in Barrett’s esophagus. Inflammatory esophagogastric polyps are usually
Prone single-contrast esophagram shows a peduncu- manifested on barium studies by a single promi-
lated polyp (arrows) extending from the gastroesoph- nent fold that arises in the region of the gas-
ageal junction into the distal esophagus. The resected
specimen revealed Barrett’s mucosa, with an adeno- tric cardia and extends upward into the distal
matous polyp containing a solitary focus of adenocar- esophagus as a smooth, polypoid protuberance
cinoma. (Reproduced with permission)9 (Fig 4).10-12 These lesions are often best visualized
12 Levine

on prone, single-contrast views of the esophagus


during continuous drinking of a low-density bar-
ium suspension because the distal esophagus is
optimally distended on these views. Because of its
characteristic appearance and location, endos-
copy is unnecessary when a typical inflammatory
esophagogastric polyp is detected on barium
studies. If the polyp has a lobulated contour or
other atypical radiographic features, however,
endoscopy and biopsy should be performed to
exclude an adenomatous polyp in Barrett’s
esophagus or other malignant lesions in the
esophagus.

Glycogenic Acanthosis
Glycogenic acanthosis is a benign condition of
unknown cause in which there is accumulation of
cytoplasmic glycogen in the squamous epithelial
cells lining the esophagus, causing focal plaque-
like thickening of the mucosa.13-15 Although gly-
cogenic acanthosis is not considered to be a neo-
plastic condition, it is included in this review
because it is characterized by mucosal nodules or
plaques. Glycogenic acanthosis is a benign, de-
generative condition, occurring primarily in the
elderly.14 This condition rarely causes esophageal
symptoms and is not associated with any known Figure 5. Glycogenic acanthosis. Double-contrast
risk of malignant degeneration.16 As a result, it is esophagram shows multiple small, rounded nodules in
usually discovered as an incidental finding on the midesophagus. This was an elderly patient who had
no esophageal symptoms. (Reproduced with permis-
radiologic or endoscopic examinations. sion from Levine MS. Benign tumors, in, Levine MS,
Radiology of the Esophagus, WB Saunders, Philadel-
Radiographic Findings phia, PA, 1989, pp 113-130)

Glycogenic acanthosis is manifested on double-


contrast esophagrams by multiple small, rounded Submucosal Lesions
nodules or plaques in the middle or, less com- By definition, all submucosal lesions arising in
monly, distal third of the esophagus (Fig 5).16,17 the wall of the esophagus are intramural. Not all
The nodules usually range from 1 to 3 mm in intramural lesions are submucosal, however, as
diameter, but occasional plaques can be as large they can also arise from the muscularis propria or
as several centimeters in diameter.16,17 The major even the subserosa. Despite this distinction, the
consideration in the differential diagnosis is terms submucosal and intramural are used inter-
Candida esophagitis (Fig 6). However, the plaques changeably based on long-standing convention.
of candidiasis tend to have a more linear config-
uration and typically develop in immunocompro-
mised patients with odynophagia,18 whereas glyco- Leiomyoma (Gastrointestinal Stromal
genic acanthosis occurs in older individuals who Tumor)
are not immunocompromised and have no esoph- Leiomyomas (also known as gastrointestinal stro-
ageal symptoms. Thus, it is usually possible to mal tumors) are by far the most common be-
differentiate these conditions on the basis of the nign submucosal tumors in the esophagus.3,19
clinical and radiographic findings. These lesions consist of intersecting bands of
Benign Tumors of the Esophagus 13

smooth muscle and fibrous tissue surrounded by a


well-defined capsule. Most esophageal leiomyo-
mas are located in the thoracic esophagus below
the level of the aortic arch because of the pres-
ence of striated rather than smooth muscle in the
esophagus above this level. The vast majority of
esophageal leiomyomas occur as solitary lesions,
but multiple leiomyomas are present in 3% to 4%
of cases.20,21 Most patients with esophageal
leiomyomas are asymptomatic, but some may
present with dysphagia, depending on the size of
the lesion and how much it encroaches on the
lumen. In contrast, gastrointestinal bleeding
rarely occurs because leiomyomas in the esopha-
gus, unlike those in the stomach, are almost

Figure 7. Leiomyoma. Double-contrast esophagram


shows a smooth-surfaced mass (arrows) in the
midesophagus. Note how this lesion has the typical
radiographic features of a submucosal mass, forming
slightly obtuse angles with the adjacent esophageal
wall.

never ulcerated.22 Unlike stromal tumors else-


where in the gastrointestinal tract, esophageal
leiomyomas virtually never undergo sarcomatous
degeneration. Thus, surgical removal of small
esophageal leiomyomas in asymptomatic patients
is probably not warranted.

Radiographic Findings
When leiomyomas grow exophytically into the
mediastinum, they can sometimes be recognized
Figure 6. Candida esophagitis. Double-contrast esopha- on chest radiographs by the presence of a medi-
gram shows multiple plaque-like lesions in the esoph- astinal mass.23 Rarely, these tumors may contain
agus. Note how the plaques have discrete borders and
a predominantly linear configuration. This was an im- punctate areas of calcification.24 Leiomyomas
munocompromised patient with odynophagia. (Repro- usually appear on barium studies as discrete,
duced with permission).18 smooth-surfaced submucosal masses that form
14 Levine

right angles or slightly obtuse angles with the


adjacent esophageal wall when viewed in profile
(Fig 7).19,22 These lesions may therefore be indis-
tinguishable from other mesenchymal tumors ex-
cept that leiomyomas are more likely on empiri-
cal grounds. The lesions typically range from 2 to
8 cm in diameter but can occasionally be giant
masses as large as 20 cm in size.25 Leiomyomas
typically appear on computed tomography (CT)
as homogeneous soft tissue masses but differen-
tiation from other benign or malignant esopha-
geal tumors is difficult on the basis of the CT
findings.26 Although the vast majority of esopha-
geal leiomyomas occur as solitary submucosal
masses, barium studies may occasionally reveal
multiple submucosal masses or even annular le-
sions with varying degrees of obstruction.20,21

Esophageal Leiomyomatosis
Esophageal leiomyomatosis is a rare, benign
condition in which there is neoplastic prolifera-
tion of smooth muscle, causing marked circum-
ferential thickening of the esophageal wall, most
commonly in the distal esophagus.27-29 It usually
occurs as a familial condition with autosomal-
dominant inheritance and is sometimes associ-
ated with widespread visceral leiomyomatosis.29
This condition is found predominantly in children
or adolescents who present with long-standing
dysphagia that gradually progresses over a period
of years.29 Depending on the extent of the lesion,
an esophagectomy or esophagogastrectomy is al-
most always curative.27,29

4
Figure 8. Esophageal leiomyomatosis. (A) Double-
contrast esophagram shows smooth, tapered narrow-
ing of the distal esophagus (black arrows), resembling
the appearance of primary achalasia. However, the
narrowed segment is longer than that typically seen in
achalasia. Also note a soft tissue mass (white arrow) in
the gas-filled fundus as a result of bulging of this
thickened muscle into the proximal stomach. (B) CT
scan shows a mass of relatively low soft tissue attenu-
ation (large arrow) near the gastroesophageal junc-
tion, with a slit-like collection of contrast material
(small arrow) in the compressed esophageal lumen.
(C) More caudal CT scan shows this thickened mass of
muscle bulging into the gastric fundus (arrows) on
both sides of the cardia. (A-C reproduced with per-
mission).29
Benign Tumors of the Esophagus 15

Radiographic Findings
Esophageal leiomyomatosis may be manifested
on barium studies by smooth, tapered narrowing
of the distal esophagus with markedly decreased
or absent esophageal peristalsis, mimicking the
appearance of primary achalasia (Fig 8A).29 How-
ever, the narrowed segment tends to be longer
than that in achalasia, and leiomyomatosis is
sometimes associated with relatively symmetric
paracardiac defects in the gastric fundus as a
result of bulging of this thickened mass of muscle
into the proximal stomach (see Fig 8A).29 CT
may reveal marked circumferential thickening
of the distal esophageal wall, resembling the
findings of secondary achalasia caused by meta-
static tumor at the gastroesophageal junction
(Fig 8B and C).29,30 However, esophageal leio-
myomatosis usually occurs in children or adoles-
cents with long-standing dysphagia, whereas sec-
ondary achalasia occurs in older individuals with
recent onset of dysphagia and weight loss.31 Thus,
despite its rarity, esophageal leiomyomatosis can
sometimes be suspected on the basis of the clin-
ical and radiographic findings.

Fibrovascular Polyp
Fibrovascular polyps are rare, benign mesenchy-
mal tumors characterized by the development of
pedunculated intraluminal masses that can grow
to enormous sizes in the esophagus. These lesions
consist of varying amounts of fibrovascular and
adipose tissue covered by normal squamous epi-
thelium.32 Depending on the predominant histo-
logic components, these tumors have variously
been called hamartomas, fibromas, lipomas, and
fibrolipomas.33 More recently, however, the le-
sions have all been classified together as fibrovas-
cular polyps, a term recommended by the World
Health Organization’s international histologic
classification of tumors.34
Fibrovascular polyps almost always arise in the
cervical esophagus near the level of the crico- Figure 9. Fibrovascular polyp. (A) Double-contrast
pharyngeus.32,33 The lesions gradually elongate esophagram shows a smooth, expansile, sausage-
over a period of years because they are dragged shaped mass in the upper third of the thoracic esoph-
inferiorly by esophageal peristalsis until the infe- agus. This lesion has the classic features of a fibrovas-
cular polyp on barium studies. (B) CT scan shows an
rior tip has reached the middle or even distal
expansile intraluminal mass (arrow) in the upper third
third of the esophagus, occasionally prolapsing of the thoracic esophagus. Note a peripheral rim of
through the cardia into the gastric fundus.33 Re- contrast material surrounding the polyp, confirming its
gardless of the size of the polyp, the proximal end intraluminal location. Also note the predominant fat
is usually attached to the cervical esophagus by a density of the lesion at this level. (A and B reproduced
with permission).35
pseudo-pedicle.
16 Levine

Fibrovascular polyps most commonly occur in


elderly men who present with long-standing dys-
phagia.35 Some patients may also develop respi-
ratory symptoms such as stridor or wheezing as a
result of compression of the adjacent trachea by
the polyp.35 Rarely, these individuals may have a
spectacular clinical presentation with regurgita-
tion of a fleshy mass into the pharynx or mouth or
even asphyxia and sudden death if the regurgi-
tated polyp occludes the larynx.32,33,35,36 Although
malignant degeneration of fibrovascular polyps is
thought to be extremely rare, removal of these
lesions is recommended because of the inexora-
ble progression of symptoms and the theoretical
risk of asphyxia and sudden death.22

Radiographic Findings
Fibrovascular polyps can sometimes be recog-
nized on frontal chest radiographs by the pres-
ence of a smooth, slightly lobulated mass in the
right superior mediastinum and on lateral radio-
graphs by a retrotracheal mass displacing the
trachea anteriorly.35 Barium studies typically re-
veal a smooth, expansile, sausage-shaped mass
expanding the lumen of the upper or upper and
middle thirds of the esophagus (Fig 9A).35,37,38
These lesions therefore have a characteristic ap-
pearance on esophagography.

Figure 11. Granular cell tumors. Double-contrast


esophagram shows two discrete submucosal masses
(arrows) in the middle and distal thirds of the esoph-
agus. This patient had additional granular cell tumors
in the stomach. (Reproduced with permission from
Levine MS. Benign tumors, in, Levine MS, Radiology of
the Esophagus, WB Saunders, Philadelphia, PA, 1989,
pp 113-130)

Fibrovascular polyps that contain a consider-


able amount of adipose tissue classically appear
on CT as fat-density lesions expanding the lumen
of the esophagus, with a thin rim of contrast
Figure 10. Fibrovascular polyp. CT scan shows an surrounding the polyp, confirming its intralumi-
expansile intraluminal mass (arrows) in the upper tho- nal location (Fig 9B).35,39-41 However, polyps that
racic esophagus, with a rim of contrast material sur- contain varying amounts of fibrovascular and ad-
rounding the lesion. Note the heterogeneous attenua- ipose tissue may appear as heterogeneous lesions
tion of the polyp, with areas of soft tissue density on CT, with areas of fat juxtaposed with areas of
juxtaposed with areas of fat density. The CT findings
depend on the amount of fibrovascular and adipose soft tissue density (Fig 10), and polyps that con-
tissue in these lesions. (Reproduced with permis- tain an abundance of fibrovascular tissue may
sion).35 appear as soft tissue density lesions on CT, with
Benign Tumors of the Esophagus 17

a paucity of fat.35 Thus, fibrovascular polyps may


be manifested by a spectrum of findings on CT,
depending on the predominant histologic compo-
nents of the lesion.

Granular Cell Tumor


Granular cell tumors arise from Schwann cells of
the peripheral nervous system. About 7% of gran-
ular cell tumors involve the gastrointestinal
tract, and one third of these lesions are found in
the esophagus.42,43 Histologically, these lesions
consist of sheets of polygonal tumor cells contain-
ing an eosinophilic-staining granular cytoplasm.44
Granular cell tumors usually appear on esopha-
gography as one or more small, round or ovoid
submucosal masses that are often mistaken for
leiomyomas on the basis of the radiographic find-
ings (Fig 11).44,45 Most patients with granular cell
tumors in the esophagus are asymptomatic and

Figure 13. Duplication cyst. Single-contrast esopha-


gram shows the rare, communicating form of duplica-
tion cyst as a tubular, branching outpouching (arrows)
from the midesophagus. (Reproduced with permis-
sion).22

therefore these lesions are usually detected as


incidental findings on barium studies or endos-
copy. However, large granular cell tumors that
cause dysphagia may require local excision.

Duplication Cyst
Figure 12. Duplication cyst. Double-contrast esopha- Although duplication cysts are not true neo-
gram shows a smooth, submucosal-appearing mass plasms, they may also appear on barium studies
(black arrows) on the right lateral wall of the distal as submucosal masses. Esophageal duplication
esophagus. Note how the lateral portion of the cyst is cysts comprise about 20% of all duplication cysts
visible where it interfaces with the adjacent lung in the gastrointestinal tract.46 These cysts repre-
(white arrows). (Reproduced with permission from Le-
vine MS. Benign tumors, in, Levine MS, Radiology of sent developmental anomalies in which large
the Esophagus, WB Saunders, Philadelphia, PA, 1989, nests of cells are sequestered from the primitive
pp 113-130) foregut. Duplication cysts contain multiple layers
18 Levine

of the bowel wall, including a mucosa, submu- 5. Sandvik AK, Aase S, Kveberg KH, et al: Papillomatosis of
cosa, and muscularis propria and are lined by a the esophagus. J Clin Gastroenterol 22:35-37, 1996
6. Montesi A, Alessandro P, Graziani L, et al: Small benign
ciliated columnar epithelium.47 Affected individ- tumors of the esophagus: radiological diagnosis with
uals are usually asymptomatic, but symptoms double-contrast examinations. Gastrointest Radiol 8:207-
may occasionally be caused by bleeding or infec- 212, 1983
tion of the cyst.48,49 Although most duplication 7. Walker JH: Giant papilloma of the thoracic esophagus.
cysts are noncommunicating, tubular duplica- AJR 131:519-520, 1978
8. McDonald GB, Brand DL, Thorning DR: Multiple adeno-
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the esophageal lumen. esophagus. Gastroenterology 72:1317-1321, 1977
9. Levine MS, Caroline D, Thompson JJ, et al: Adenocarci-
Radiographic Findings noma of the esophagus: relationship to Barrett mucosa.
Radiology 150:305-309, 1984
Duplications cysts tend to be located in the right 10. Bleshman MH, Banner MP, Johnson RC, et al: The in-
lower mediastinum. As a result, they can some- flammatory esophagogastric polyp and fold. Radiology
times be recognized on frontal chest radiographs 128:589-593, 1978
by the presence of a mediastinal mass in this 11. Staples DC, Knodell RG, Johnson LF: Inflammatory
pseudotumor of the esophagus. Gastrointest Endosc 24:
location.22 The cysts typically appear on barium
175-176, 1978
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guishable from other more common mesen- polyps: Radiographic and endoscopic findings. Radiology
chymal lesions, such as leiomyomas (Fig 12).22 154:307-311, 1985
These fluid-filled cysts usually have characteristic 13. Rywlin AM, Ortega R: Glycogenic acanthosis of the
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14. Ghahremani GG, Rushovich AM: Glycogenic acanthosis
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18. Levine MS, Macones AJ, Laufer I: Candida esophagitis:
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