Current Management of Esophageal Leiomyoma
Lawrence S Lee, BS, Sunil Singhal, MD, Clayton J Brinster, BS, Blair Marshall, MD,
Michael L Kochman, MD, Larry R Kaiser, MD, FACS, John C Kucharczuk, MD
Morgagni first described leiomyoma as a distinct gastro-
intestinal neoplasm in 1761, but Munro in 1797 was the
first to report a localized intramural leiomyoma of the
esophagus.1-3 Much of what is known today about the
histologic features of esophageal leiomyoma was speci-
fied by Virchow4 in 1863. Sauerbruch5 reported the first
successful surgical treatment of esophageal leiomyoma
when he performed an esophageal resection with gas-
troesophagostomy in 1932. One year later Ohsawa6 per-
formed the first successful surgical enucleation of this
type of tumor.
Leiomyoma has traditionally been classified along
with leiomyosarcoma as smooth-muscle–cell tumors.
Several years ago the term gastrointestinal stromal tumor
(GIST) was introduced as a histogenetically neutral
term to refer to all mesenchymal tumors of the GI tract,
including those of smooth-muscle–cell origin; but re-
cent evidence indicates that most GISTs comprise a
group of neoplasms distinct from true leiomyoma and
leiomyosarcoma based on immunohistochemical, ultra-
structural, and molecular genetic markers.7,8 Patholo-
gists currently classify leiomyoma and GIST as two sep-
arate discrete types of tumors. Leiomyoma is the most
common mesenchymal tumor of the esophagus but is
exceedingly rare in other parts of the GI tract. In contrast
GISTs are common in the stomach and intestines but are
rarely found in the esophagus.
Our objective in preparing this article was to report
the currently available techniques for the diagnosis and
treatment of esophageal leiomyoma. We have reviewed
the English-language literature reporting the various di-
agnostic and treatment modalities of this condition pub-
lished between 1900 and 2003. Literature searches were
No competing interests declared.
Received March 28, 2003; Revised August 20, 2003; Accepted August 20,
2003.
From the Section of General Thoracic Surgery, Division of Cardiothoracic
Surgery, Department of Surgery, University of Pennsylvania School of Med-
icine, Philadelphia, PA (Lee, Singhal, Brinster, Marshall, Kaiser, Kucharczuk);
and Division of Gastroenterology, Department of Medicine, University of
Pennsylvania School of Medicine, Philadelphia, PA (Kochman).
Correspondence address: John C Kucharczuk, MD, Division of Cardiotho-
racic Surgery, Department of Surgery, Hospital of the University of Pennsyl-
vania, 4 Silverstein Pavilion, 3400 Spruce St, Philadelphia, PA 19104.
© 2004 by the American College of Surgeons
Published by Elsevier Inc.
performed on Medline using the following search terms:
leiomyoma, esophagus, esophageal mesenchymal tu-
mor, gastrointestinal stromal tumor, and esophageal
smooth-muscle tumor.
CLINICAL FEATURES
Incidence
Leiomyoma is the most common benign esophageal
neoplasm, accounting for roughly two-thirds of all be-
nign tumors of this organ.9 The incidence of leiomyoma
reported in autopsy series ranges from 0.005% to
5.1%.10,11 This may be an underestimate, as many small
lesions are missed at autopsy unless the esophagus is
carefully examined. Studies using detailed histologic ex-
aminations have reported the frequency of esophageal
leiomyoma to be higher, at 7.9%;12 but leiomyoma is
relatively rare when compared with esophageal carci-
noma, which occurs 50 times more frequently.9 Leiomy-
oma can occur at any age but 90% of cases occur in
patients between the ages of 20 and 69 years, with peak
incidence in the third to fifth decades. The male-to-
female ratio is approximately 2:1 (in children it is re-
versed) and there are no apparent racial or geographic
predilections for the disease.
Location
Leiomyoma tumors are most frequently found in the
lower and middle thirds of the esophagus, with 56% and
33% of cases occurring in these regions, respectively
(Fig. 1).9 Lesions are rarely found in the upper third.
These findings correlate with the muscular composition
of the esophagus in each of these regions: predominantly
smooth muscle in the lower third, skeletal muscle in the
upper third, and a mixture in the middle. Approximately
80% of leiomyomas are located intramurally and origi-
nate in the muscularis propria (Fig. 2), and up to 13% of
these intramural lesions may have an annular morphol-
ogy that encircles the wall of the esophagus and compli-
cates treatment.13 Occasionally the lesion may arise from
the muscularis mucosa, which causes the tumor to pro-
trude into the lumen and pedunculate because of the
downward movement of food and peristalsis.14-16 An ex-
ISSN 1072-7515/04/$30.00
136 doi:10.1016/j.jamcollsurg.2003.08.015
Vol. 198, No. 1, January 2004
Abbreviations and Acronyms
EUS ⫽ endoscopic ultrasonography
EUS-FNA⫽ endoscopic endosonography–guided fine-needle
aspiration
GIST ⫽ gastrointestinal stromal tumor
HALS ⫽ hand-assisted laparoscopic surgery
traluminal growth pattern is observed in 7% of cases and
some studies indicate that up to 26% of all lesions can
cause compression of mediastinal structures.17
Tumor size
Leiomyoma is a slow growing tumor and the size of the
lesion may not change for many years. Approximately
one-half of all tumors are smaller than 5 cm, with 93%
being smaller than 15 cm. Small tumors are oval or
spherical; larger tumors are often elongated in a
dumbbell-like shape. Giant tumors involving the entire
esophagus and weighing up to 5 kg have been reported
but are extremely rare.9,18 Leiomyoma usually presents as
a single lesion, but cases of multiple lesions have been
reported.19,20
Histology
As noted previously there has been some confusion in
the literature about the proper histopathologic classifi-
cation of leiomyoma, particularly with respect to GISTs.
Figure 1. Location of leiomyoma lesions in the esophagus.9
Lee et al Management of Esophageal Leiomyoma 137
Figure 2. Growth patterns of esophageal leiomyoma.13
Leiomyoma was at one time considered to be a GIST,
but current understanding of their molecular biologic
properties specifies them as two distinct types of
neoplasms.
On gross inspection leiomyomas are firm, well encap-
sulated, and often have a smooth or nodular surface. In
section they appear white to gray with a whorled surface.
These tumors have low overall cellularity, appear
strongly eosinophilic on hematoxylin-eosin stain, and
are comprised of interlaced smooth-muscle cells with
hypovascularity and absent mitoses.8 On the other hand
GISTs have a soft, fish-flesh–like consistency and show
high cellularity with basophilic appearance on
hematoxylin-eosin stain.
Four immunohistochemical markers usually form the
basis for differentiation between an esophageal leiomy-
oma and GIST: typically leiomyoma is positive for
desmin and smooth-muscle actin and negative for
CD34 and CD117, while GISTs are negative for desmin
and smooth-muscle actin and positive for CD34 and
CD117. Expression of the last marker, CD117, has been
cited as being the most specific diagnostic criterion for
discriminating a GIST from leiomyoma.7,8
Symptoms
Symptoms, when present, are generally nonspecific and
of long duration. In a review of 838 cases, Seremetis and
colleagues9 found that the most common symptoms
138 Lee et al Management of Esophageal Leiomyoma J Am Coll Surg

Table 1. Common Presenting Symptoms

Symptom Frequency (%)
Dysphagia 47
Pain 47
Weight loss 15
Nausea and vomiting 12
Asymptomatic 15–50

were dysphagia, pain, and weight loss (Table 1). Pain is

often described as a dull pressure, sharp retrosternal
pain, or pyrosis. These tumors rarely ulcerate or bleed.
Most cases are diagnosed within 1 year of the onset of
symptoms, but 24% of patients report having symptoms
that lasted 5 or more years.9,13 The proportion of pa-
tients who are asymptomatic at the time of diagnosis
ranges from 15% to 50%; most of these lesions are dis-
covered incidentally during routine radiologic examina-
tions.9,13,17 Interestingly there appears to be no direct
correlation between tumor size and symptoms.13,21 Hia-
tal hernia, found in 4.5% to 23% of leiomyoma pa-
tients, is the most common coexistent lesion and its
presence often predisposes the patient to reflux esoph- Figure 3. Diagnostic management of suspected esophageal leiomy-
agitis and subsequent ulceration and bleeding.19,22 oma. CT, computed tomography; EUS, endoscopic ultrasonography.

DIAGNOSIS ative procedure for suspected cases; lesions appear as a

Definitive diagnosis can be made only by histologic
examination; but such analysis is difficult to obtain as
biopsy is contraindicated in patients with leiomyoma
because of an increased risk of intraoperative complica-
tions. Instead several diagnostic modalities can be used
in the workup of suspected esophageal leiomyoma (Fig.
3) to narrow the differential diagnosis.
rounded or lobulated, smoothly elevated filling defect
with sharp demarcation between the tumor edge and the
uninvolved esophagus.25 In profile, tumor intrusion into
the lumen causes the appearance of a step-like forma-
tion. There is usually no evidence of infiltration or ul-
ceration, and the overlying mucosal folds are often
stretched or flattened.19

Chest x-ray Endoscopy

Although plain films are not particularly sensitive or
specific for esophageal leiomyoma, abnormal findings
on routine chest films are often the first presenting sign
of this diagnosis, particularly in asymptomatic patients.
If large enough, a lesion will appear as a rounded or
lobulated homogeneous mass with lateral growth to one
or both sides in the mediastinum. Calcification within
the tumor, although rare, may be present.23
Barium swallow
The high sensitivity and noninvasive nature of barium
swallow have led many to state this to be the best and
most informative diagnostic test for esophageal leiomy-
oma.24 This is the most commonly performed preoper-
Endoscopy is invaluable in determining the presence
and location of the tumor and in assessing the condition
of the mucosa. Postlethwait23 cites four findings charac-
teristic of leiomyoma: 1) the overlying mucosa appears
normal and intact; 2) the tumor projects into the lumen
to varying degrees; 3) the tumor is freely movable, with
the overlying mucosa easily sliding over the lesion; and
4) narrowing of the lumen is common, but stenosis and
obstruction to passage are not (Figs. 4, 5). The presence
of normal mucosa overlying the tumor is most signifi-
cant and helpful in ruling out malignant pathologies
such as esophageal carcinoma, which usually exhibit in-
flamed and ulcerated mucosa.
In cases of suspected leiomyoma endoscopic biopsy is
Vol. 198, No. 1, January 2004
Figure 4. Endoscopy shows normal intact mucosa overlying a tumor
in a distal esophagus. Note that the lesion causes narrowing but not
complete obstruction of the lumen.
contraindicated, because this procedure involves disrup-
tion of the mucosal layer and risks secondary infection,
bleeding, and perforation. The incidence of intraopera-
Figure 5. Lesion located at the gastroesophageal junction with
normal overlying mucosa.
Lee et al Management of Esophageal Leiomyoma 139
Figure 6. Endoscopic ultrasonography shows a homogeneous hy-
poechoic mass with smooth outer borders in the fourth layer (mus-
cularis propria) of the esophagus.
tive mucosal tear is also significantly increased in pa-
tients who have undergone preoperative biopsy, and sev-
eral studies have reported the ineffectiveness of biopsy in
the diagnostic workup.15,22,24 The endoscopic examina-
tion provides critical information about the location of
the tumor and the condition of the mucosa but one
limitation of the procedure is its inability to distinguish
leiomyoma from other types of submucosal tumors.15,26
Endoscopic ultrasonography
Determining the layer of origin of a tumor is essential for
the diagnosis and treatment of leiomyoma. The strength
of endoscopic ultrasonography (EUS) lies in its ability to
depict the five layers of the esophageal wall, making it
possible to ascertain the layer of origin of a submucosal
mass and to differentiate between intramural and extrin-
sic lesions. On EUS leiomyoma appears as a well-
circumscribed, homogeneous, hypoechoic mass with a
smooth outer border (Fig. 6). Some investigators assert
that this procedure is the most effective diagnostic mo-
dality once suspicion has been raised by endoscopy or
other methods.14
This technique is not without its limitations. First,
EUS is not specific in distinguishing the nature of an
intramural mass; in particular the differentiation be-
tween leiomyoma and an extramucosal cyst may not be
obvious because of the high density of some cystic con-
tents. Second, EUS is unable to provide a reliable dis-
140 Lee et al Management of Esophageal Leiomyoma J Am Coll Surg

tinction between benign and malignant tumors, partic- Table 2. Sensitivity of Diagnostic Studies in Histologically
ularly for lesions larger than 3 cm.14,27,28 Despite these Diagnosed Esophageal Leiomyoma
Sensitivity14,24,28
limitations the information obtained via EUS, when Test Findings % (n)
combined with that from other diagnostic methods, of- Plain film Soft tissue mass 83 (12)
ten makes it possible for the clinician to infer the correct Barium swallow Filling defect 93 (15)
diagnosis.14,27,29 Bulge with normal
Some investigators have described the use of EUS- Endoscopy mucosa 100 (15)
guided fine-needle aspiration (EUS-FNA) as a means of Computed tomography Hypodense mass 91 (11)
attaining a more definitive diagnosis of esophageal Endoscopic Homogeneous,
ultrasonography hypoechoic mass 88 (32)
leiomyoma based on cytology.30,31 These reports show
that EUS-FNA is effective in obtaining sufficient sample
for cytologic study including immunohistochemical most important pathologic conditions to rule out are
analysis, and state that this technique enables investiga- esophageal carcinoma and leiomyosarcoma. Leiomyoma
tors to make a firm diagnosis. can be distinguished from these malignant counterparts
There are no reports that compare the accuracy of by the following: 1) the onset of symptoms is much more
EUS-FNA to that of EUS alone in diagnosing esopha- rapid in leiomyosarcoma and carcinoma than in leiomy-
geal leiomyoma, but the former has been shown to be oma; 2) dysphagia or weight loss, or both, are found in
more accurate when examining submucosal tumors of the vast majority of carcinoma and leiomyosarcoma pa-
the stomach and duodenum.32 In addition the rate of tients but in less than one-half of leiomyoma patients;
complications during surgical resection of an esophageal and 3) the mucosa is typically ulcerated and inflamed in
leiomyoma after preoperative EUS-FNA is unknown, carcinoma but it appears normal in leiomyoma. The
but in a recent case one such tumor diagnosed by this diagnostic sensitivities of the various studies are given in
method was surgically removed with no intra- or post- Table 2.
operative complications.31 EUS-FNA appears to aug-
ment the diagnostic capability of EUS, but more pub- TREATMENT
lished experience is needed to assess thoroughly the Once a clinical diagnosis of leiomyoma is established
safety and usefulness of this technique in the evaluation many factors must be taken into consideration when
of esophageal leiomyoma. determining an appropriate course of treatment. Of par-
ticular importance are tumor size, location, and mor-
Computed tomography phology and the patient’s symptoms and overall condi-
Computed tomography (CT) is able to depict the ana- tion. There is a general consensus in the literature that
tomic relationship of the lesion to other organs in addi- esophageal leiomyoma should be surgically removed in
tion to providing information about tumor size and lo- symptomatic patients.
cation. As such, CT is helpful in diagnosing invasion Treatment of asymptomatic patients remains debat-
into surrounding structures and in distinguishing able (Table 3). Surgical treatment has traditionally been
esophageal tumors from extrinsic compression. Leiomy- the therapy of choice, and many experts advocate the
oma usually appears as a smoothly marginated, round or resection of asymptomatic tumors based on the follow-
lobulated mass with homogeneously low- or iso-
attenuation.25 The surrounding mediastinal fat is usually Table 3. Reasons Commonly Cited for Surgical Versus Non-
not disrupted.18,33 CT cannot image intramural condi- operative Management of Asymptomatic Lesions
tions and is relatively inaccurate in differentiating be- Surgical removal Nonoperative management
tween cystic and solid masses.14,34,35 Possibility of malignant Malignant transformation is
transformation extremely rare
Possibility of symptomatic Slow growing and may not change
DIFFERENTIAL DIAGNOSIS transformation in size for years
Differential diagnosis includes leiomyosarcoma, esoph- To obtain a definitive
ageal carcinoma, hiatal hernia, diverticulum, other gas- histologic diagnosis Clinical course is benign
trointestinal stromal tumors, and compression of the Malignancy can be excluded Surgery may do more harm than
esophagus secondary to a mediastinal tumor or cyst. The only by removal good
Vol. 198, No. 1, January 2004
ing: 1) the possibility of malignant transformation; 2)
the possibility of symptomatic transformation; 3) the
need to obtain a definitive histologic diagnosis; and 4)
the exclusion of malignancy only by removal.13,17 On the
other hand studies have shown that asymptomatic pa-
tients do not experience complications of leiomyoma
even without tumor removal and that the risks of surgery
may outweigh the benefits in some cases. Such observa-
tions have led some experts to recommend nonoperative
management for asymptomatic patients.
Nonoperative management
Nonsurgical therapy is generally limited to expectant
management with monitoring by radiography, endos-
copy, or endosonography. The justification for this ap-
proach is fourfold: 1) malignant transformation to
leiomyosarcoma is extremely rare, with only four re-
ported cases in the literature; 2) leiomyoma is a slowly
growing tumor that may not change in size for many
years; 3) the clinical course of leiomyoma is usually be-
nign, with many patients remaining asymptomatic until
death; and 4) the trauma of surgical treatment may be
more harmful to the patient than no treatment at
all.17,36,37
Glanz and Grunebaum36 followed nine patients (six
symptomatic, three asymptomatic) for periods of 1 to 15
years and found no malignant transformation or
changes in radiologic findings. One patient underwent
surgical enucleation without resolution of symptoms
and experienced postoperative complications including
pulmonary emboli and pleural effusion. These results
led Glanz and Grunebaum to recommend that asymp-
tomatic and mildly symptomatic patients not undergo
surgery and instead be monitored regularly by radiogra-
phy and endoscopy to rule out malignant transforma-
tion. Similar conclusions are reported in other studies of
asymptomatic unoperated cases of leiomyoma.37,38 Some
investigators recommend evaluation by EUS every 1 to 2
years for asymptomatic patients who show a small lesion
with smooth borders and a homogeneous internal echo
pattern on EUS with no suspicious lymphadenopathy.39
Indications for surgical treatment
Surgical treatment has traditionally been the therapy of
choice for esophageal leiomyoma. Indications for surgi-
cal removal (Table 4) include unremitting symptoms,
progressive increase in tumor size, mucosal ulceration,
Lee et al Management of Esophageal Leiomyoma 141
Table 4. Indications for Surgical Resection
Unremitting symptoms
Increase in tumor size
Mucosal ulceration
To obtain histopathologic diagnosis
Facilitation of other procedures
the need to obtain histopathologic diagnosis, and facili-
tation of other esophageal procedures.13,39,40
As shown in Table 5 there are a variety of surgical
approaches; but the goals of any treatment modality are
to remove the tumor along with any surrounding neo-
plastic tissue and to restore the integrity of the gastroin-
testinal tract. To this end the principles of operative
management include resection of the lesion without in-
jury to the mucosa, appropriate measures to prevent
leakage or mucosal bulging, and sufficient postoperative
testing to confirm the absence of any leakage. Table 5
lists some of the published reports of various treatment
methods and their major complications.
Thoracotomy
Transthoracic extramucosal enucleation via thoracot-
omy is the most common procedure used for the treat-
ment of esophageal leiomyoma. The location of the tu-
mor dictates the approach: lesions in the middle and
upper thirds of the esophagus can be reached with a right
thoracotomy; those in the lower third can be removed
via a left thoracotomy. An upper midline laparotomy
may also be used for tumors located very low in the
esophagus or at the gastroesophageal junction. When
the esophageal muscle coat is longitudinally split, the
tumor appears as an avascular encapsulated mass. Enu-
cleation can be facilitated by blunt dissection and usually
occurs easily along a plane between the tumor and mu-
cosa unless there is inflammation or mucosal damage
caused by preoperative biopsy. If the mucosa is injured
during the procedure, the tear must be identified and
repaired.
There is some disagreement in the literature about
whether the myotomy should be sutured after enucle-
ation. Hennessy and Cuschieri41 state that a wide breach
can be left open without concern, but most experts em-
phasize the need to reapproximate the muscular wall to
prevent mucosal bulging.42,43 Several studies have re-
ported the occurrence of postoperative dysphagia due to
pseudodiverticular mucosal bulging at the enucleation
site in patients in whom the muscle edges were not ap-
142 Lee et al Management of Esophageal Leiomyoma J Am Coll Surg

Table 5. Reports of Various Treatment Modalities for Esophageal Leiomyoma

First
author Year Institution n Treatment method (n) Complications (n)
Bonavina22 1995 University of Milan, Italy 66 Thoracotomy (55) Thoracotomy: mucosal tear (7), intercostal
Thoracoscopy (8) vessel bleeding (1), postoperative
Esophageal resection (3) esophagopleural leak (1)
Thoracoscopy: mucosal tear (1),
conversion to thoracotomy (2),
postoperative dysphagia and mucosal
bulging (1)
Roviaro43 1998 University of Milan, Italy 7 Thoracoscopy Conversion to thoracotomy (1)
Bardini44 1997 University of Padua, Italy 5 Thoracoscopy Mucosal bulging and pseudodiverticulum
(2)
Gossot58 1993 Saint-Louis Hospital, 4 Thoracoscopy Mucosal tear (2)
France Conversion to thoracotomy (1)
Izumi60 1996 Tokyo Medical and Dental 3 Combined endoluminal None
University, Japan intracavitary thoracoscopy
Redan59 2001 Tyler Memorial Hospital, 1 Hand-assisted laparoscopy None
PA
Eda61 1990 Tohoku University, Japan 25 Endoscopic lumpectomy Local recurrence (1)
(20)
Endoscopic ethanol injection
(1)
Combination (4)
Kajiyama15 1995 Kyoto University, Japan 9 Endoscopic lumpectomy Incomplete resection (1)
Fountain21 1986 Royal Infirmary of 26 Thoracotomy (23) Thoracotomy: mucosal tear (1), lung
Edinburgh, United Esophageal resection (3) abcess (1)
Kingdom Resection: postoperative reflux esophagitis
(3), peptic stricture (2)
Preda24 1986 Insituto Nazionale 20 Thoracotomy (16) Thoracotomy: mucosal tear (3)
Tumori, Italy Esophagogastric resection (1)
Cardiomyotomy (1)
None (2)
Solomon46 1984 Jefferson Medical College, 9 Thoracotomy (4) Thoracotomy: esophageal reflux (1)
PA None (5)
Glanz36 1977 Tel Aviv University, Israel 9 Radiologic monitoring (8) Radiologic monitoring: none
Thoracotomy (1) Thoracotomy: pulmonary emboli, pleural
effusion, dysphagia

proximated. These symptoms and abnormalities imme-
diately resolved after reoperation to reconstruct the mus-
cle layer.22,43,44 It is thought that approximation of the
muscle edges after enucleation may preserve esophageal
propulsive activity, preventing such complications and
improving the long-term outcomes of the surgical
treatment.22
One concern after enucleation of a large leiomyoma is
that the myotomy may result in an esophagus that func-
tionally resembles achalasia. It is generally accepted that
tumors up to 8 cm can be safely enucleated without
significant postoperative dysphagia as long as the mu-
cosa is intact and the myotomy is reapproxi-
mated.9,22,42,43 Larger lesions can be removed via enucle-
ation but often result in defects too large to allow a
tension-free suture, requiring a tissue flap to prevent
mucosal bulging.22,23,45 Some investigators have used this
approach to successfully remove 10-cm tumors with no
postoperative motor alterations or dysphagia;22,45 but
there are no studies in the literature describing the lon-
gitudinal extent of a leiomyoma that can be enucleated
without significant resultant dysphagia, perhaps because
many reports to date have recommended esophageal re-
section for tumors larger than 8 cm.9,13
Enucleation via thoracotomy is both safe and effec-
tive, with the reported mortality rate ranging from 0%
to 1.3% and the vast majority of patients experiencing
complete resolution of symptoms.9,17,46-48 Long-term re-
sults are likewise excellent, with 89% to 94% of patients
remaining free of symptoms at 5 years after opera-
Vol. 198, No. 1, January 2004
tion.22,24 Complications are rare, but preoperative endo-
scopic biopsy is associated with an increased risk of in-
traoperative mucosal injury: one study found that
mucosal tear occurred in 50% of patients who under-
went preoperative biopsy (n ⫽ 6) and in only 8% of
those who did not (n ⫽ 55).22 Other potential compli-
cations include diverticula formation, fistulas, reflux,
esophagitis, stenosis, and ulceration. Some of these, par-
ticularly postoperative reflux esophagitis, may be caused
by reduced propulsive and acid-clearing abilities of the
esophagus, underscoring the need to approximate the
muscle edges after tumor removal. Recurrence after sur-
gical treatment is extremely rare, with only two such
cases reported in the literature.13
Thoracoscopic approach
In recent years a thoracoscopic approach has been in-
creasingly used to treat leiomyoma. Everitt and col-
leagues49 were the first to report a successful thoraco-
scopic approach to enucleation in 1992 using seven
thoracic ports. Currently the procedure can be per-
formed using four thoracic trocars.44,50 The mean oper-
ating time is reported to be 120 minutes. In many as-
pects the thoracoscopic approach is similar to the open
method. A right thoracoscopy is used for tumors in the
upper two-thirds of the esophagus, and a left thoracos-
copy or laparoscopy is used for those in the lower third.
Division of the azygos vein using an endoscopic stapler
may be necessary to identify and gain access to lesions
located in the upper two-thirds of the esophagus. As
with thoracotomy the objective is to enucleate the tumor
without injuring the mucosa. After initial blunt dissec-
tion the tumor is resected by grasping with forceps or by
placing a traction suture through it and pulling. Many
surgeons advocate the use of intraoperative esophagos-
copy as a source of transillumination to help visualize the
operative field and to check the integrity of the mucosa
during each step of the procedure.
A thoracoscopic approach may best be suited for use
in patients with lesions smaller than 5 cm that have been
diagnosed preoperatively as leiomyoma by endoscopy,
EUS, and CT.42 Large or annular lesions present diffi-
culty because endoscopic instrumentation often lacer-
ates and fragments the muscle fibers of the tumor, com-
plicating the enucleation.42,43 When the preoperative
workup cannot exclude malignancy, the approach
should be via thoracotomy.42 Excellent results are ob-
tained with thoracoscopy, with the majority of patients
Lee et al Management of Esophageal Leiomyoma 143
experiencing complete resolution of symptoms. Since
1992 a total of 30 cases have been reported in the
literature.22,43,44,49-58 There were no intraoperative deaths
but complications included mucosal tear (13.3%), post-
operative mucosal bulging (10%), pleural effusion
(3.3%), and epigastric pain (3.3%). There are no reports
in the literature describing long-term results, but one
study found no recurrence of symptoms at 16 months
after operation.54
Functional results are similar to thoracotomy, but a
thoracoscopic approach offers distinct advantages over
the open method because of the reduced surgical
trauma. Bonavina and colleagues22 found that patients
who underwent a thoracoscopy had a shorter hospital
stay (6.8 days versus 10.2 days) and decreased postoper-
ative pain (thoracoscopy patients did not require pain
medication after postoperative day 1) than those who
had a thoracotomy. In addition the thoracoscopic ap-
proach allows rapid, full reexpansion of the lung with
minimal adverse effects on pulmonary function, and pa-
tients are generally pleased with the cosmetic outcomes
of the surgical treatment.22,42
The first successful removal of an esophageal leiomy-
oma using hand-assisted laparoscopic surgery (HALS)
was recently reported.59 A combination of the closed and
open procedures, HALS gives the surgeon the ability to
use one hand during the procedure while maintaining
the benefits of a laparoscopic surgical procedure. Per-
haps the greatest advantage of this arrangement over the
closed approach is the surgeon’s ability to directly pal-
pate the organs and instruments in the operative field.
The report also indicates a decreased operating time
compared with the purely laparoscopic approach. One
concern about HALS is that the large incision needed for
handoscopy negates any benefit of the laparoscopy. The
authors of the report argue that the recovery time after
HALS is not significantly different from that after a pure
laparoscopic surgical procedure. This approach is prom-
ising but more studies are undoubtedly needed to deter-
mine its effectiveness.
Combined endoluminal intracavitary
thoracoscopic enucleation
Also called the balloon push-out method, this approach
was developed because of the difficulty in grasping the
tumor and the risk of tumor destruction or mucosal
injury while pulling on the tumor with forceps or sutures
during thoracoscopy. This technique is a modification of
144 Lee et al Management of Esophageal Leiomyoma
the thoracoscopic approach in which a balloon-
mounted intraluminal endoscope is used to assist in the
enucleation. Once the lesion is identified and located
with the endoscope, the attached balloon is positioned
adjacent to the tumor intraluminally. Inflation of the
balloon promotes the expulsion of the tumor from the
esophageal wall, facilitating the thoracoscopic resection
even for tumors that are difficult to grasp. In the rela-
tively few cases (n ⫽ 4) in which this method was used
the mortality rate was 0% and there were no intra- or
postoperative complications.51,60
Endoscopic approach
An endoscopic approach may be indicated in leiomyo-
mas that originate in the muscularis mucosa, which of-
ten grow in an intraluminal or polypoid pattern. This
technique is contraindicated in extraluminal lesions and
intramural tumors originating from the muscularis pro-
pria because of the risk of esophageal wall perforation.
Endoscopic treatment typically consists of either
lumpectomy or ethanol injections, or a combination of
both. Pedunculated tumors smaller than 2 cm are safely
removed via lumpectomy; larger tumors up to 4 cm are
best treated with ethanol injection followed by
lumpectomy.15,61
To perform lumpectomy a transparent cylinder and
snare-guide tube are attached to the tip of the endo-
scope. After separating the tumor from the submucosal
layer by injection of a hypertonic solution of 10% glyc-
erol, 5% fructose, and saline, the tumor is suctioned into
the cylinder and the snare wire is closed around the
lesion. The tumor and the surrounding mucosa are then
resected by means of electrocautery and aspirated into
the cylinder. The reported mean operating time for
lumpectomy is 18 minutes.15
In the ethanol injection method the mucosa is
stripped off with electrocautery or pure ethanol, the ex-
posed tumor is biopsied multiple times, and pure etha-
nol is injected into the tumor to promote necrotic exfo-
liation. Additional ethanol injections are performed during
subsequent sessions over several weeks to treat any re-
maining tumor tissue. Both methods have had excellent
results: in 34 reported cases there was a 0% mortality
rate, complete resolution of symptoms, and no compli-
cations except one instance of local recurrence.15,61
Although commonly used in other countries, partic-
ularly Japan, the endoscopic approach has not been
widely employed in the United States.
J Am Coll Surg
Esophageal resection
Up to 10% of leiomyoma cases may require esophageal
resection, which is indicated in patients in whom the
tumor is large (greater than 8 cm), has annular morphol-
ogy, is densely adherent to the mucosa, or where acci-
dental extensive damage to the esophageal wall appears
to present danger of postoperative leakage.9,13 Esopha-
geal resection for leiomyoma is typically performed
through an esophagectomy with gastric pull-up or an
esophagogastrectomy with colon interposition.22-24
These techniques often have significant postoperative
morbidity including reflux esophagitis, stricture forma-
tion, dumping, diarrhea, reduced meal capacity, and
weight loss.21,24,62,63 Many of these complications are
thought to be caused by division of the vagal nerves and
the subsequent loss of parasympathetic innervation to
the foregut.
To avoid these morbidities, some investigators have
described a vagal-sparing esophagectomy that allows for
removal of the esophagus while preserving the vagal
nerves and gastric reservoir.62-64 Banki and colleagues63
performed a vagal-sparing esophagectomy in 15 patients
with various underlying esophageal pathology and
found that this technique preserved gastric secretory,
motor, and reservoir function. Clinically, patients
treated in this manner had normal postoperative alimen-
tation and bowel regulation and were generally free of
dumping, diarrhea, and weight loss 20 months after
operation.
Although there are no reports of vagal-sparing esoph-
agectomy performed for leiomyoma, this is likely a con-
sequence of the rarity of these tumors and the small
percentage of them that require esophageal resection.
The previous results showing preserved gastrointestinal
function after vagal-sparing esophagectomy for varying
underlying disease suggest that this approach may be an
attractive alternative to more traditional forms of esoph-
ageal resection for leiomyoma.
In conclusion esophageal leiomyoma, although rare,
is the most common benign tumor of the esophagus.
The presenting symptoms are variable, and up to one-
half of all cases may be asymptomatic. When present,
symptoms often include dysphagia, pain, and weight
loss. Diagnosis is best made by barium swallow, endos-
copy, CT, and EUS. Depending on the characteristics of
each particular case, treatment may include expectant
management or surgical removal through one of several
available techniques. Prognosis is overwhelmingly posi-
Vol. 198, No. 1, January 2004
tive, with most patients fully recovering without com-
plications or recurrence.
Author Contributions
Study conception and design: Lee, Singhal, Marshall,
Kucharczuk
Acquisition of data: Lee, Brinster, Kochman
Analysis and interpretation of data: Lee, Marshall
Drafting of manuscript: Lee
Critical revision: Singhal, Kucharczuk
Obtaining funding: Kaiser
Supervision: Kaiser, Kucharczuk
Technical expertise: Singhal
REFERENCES
1. Businco A. Sui miomi esofagei [Latin]. Am Facol Med Chir R
Univ Perugia 1938;37:5.
2. Georgiades N, Chomatos I. Leiomyoma of the esophagus—cyst
of the esophagus. Hellen Cheir 1962;9:579.
3. Munro J. Disputatio medica unaugurales de dysphagia [Latin].
Edinburgh, 1797.
4. Virchow R. Die Krankhocften geschwiilste. Berlin: A Hirsch-
wald; 1867.
5. Sauerbruch F. Presentations in the field of thoracic surgery. Arch
Klin Chir 1932;173:457.
6. Ohsawa T. Surgery of the oesophagus. Arch Jpn Chir 1933;10:
605.
7. Miettinen M, Sarlomo-Rikala M, Lasota J. Gastrointestinal
stromal tumors: recent advances in understanding of their biol-
ogy. Hum Pathol 1999;30:1213–1220.
8. Miettinen M, Sarlomo-Rikala M, Sobin LH, Lasota J. Esopha-
geal stromal tumors: a clinicopathologic, immunohistochemi-
cal, and molecular genetic study of 17 cases and comparison
with esophageal leiomyomas and leiomyosarcomas. Am J Surg
Pathol 2000;24:211–222.
9. Seremetis MG, Lyons WS, deGuzman VC, Peabody JW Jr.
Leiomyomata of the esophagus. An analysis of 838 cases. Cancer
1976;38:2166–2177.
10. Kramer MD, Gibb SP, Ellis FH Jr. Giant leiomyoma of esoph-
agus. J Surg Oncol 1986;33:166–169.
11. Postlethwait RW, Musser AW. Changes in the esophagus in
1,000 autopsy specimens. J Thorac Cardiovasc Surg 1974;68:
953–956.
12. Takubo K, Nakagawa H, Tsuchiya S, et al. Seedling leiomyoma
of the esophagus and esophagogastric junction zone. Hum
Pathol 1981;12:1006–1010.
13. Hatch GF 3rd, Wertheimer-Hatch L, Hatch KF, et al. Tumors of
the esophagus. World J Surg 2000;24:401–411.
14. Massari M, De Simone M, Cioffi U, et al. Endoscopic ultra-
sonography in the evaluation of leiomyoma and extramucosal
cysts of the esophagus. Hepatogastroenterology 1998;45:938–
943.
15. Kajiyama T, Sakai M, Torii A, et al. Endoscopic aspiration
lumpectomy of esophageal leiomyomas derived from the mus-
cularis mucosae. Am J Gastroenterol 1995;90:417–422.
Lee et al Management of Esophageal Leiomyoma 145
16. Barrett NR. Benign smooth muscle tumours of the oesophagus.
Thorax 1964;19:185–194.
17. Rendina EA, Venuta F, Pescarmona EO, et al. Leiomyoma of the
esophagus. Scand J Thorac Cardiovasc Surg 1990;24:79–82.
18. Gallinger S, Steinhardt MI, Goldberg M. Giant leiomyoma of
the esophagus. Am J Gastroenterol 1983;78:708–711.
19. Shaffer HA Jr. Multiple leiomyomas of the esophagus. Radiol-
ogy 1976;118:29–34.
20. Taylor FH, Christenson W, Zollinger RW 2nd, et al. Multiple
leiomyomas of the esophagus. Ann Thorac Surg 1995;60:182–
183.
21. Fountain SW. Leiomyoma of the esophagus. Thorac Cardiovasc
Surg 1986;34:194–195.
22. Bonavina L, Segalin A, Rosati R, et al. Surgical therapy of esoph-
ageal leiomyoma. J Am Coll Surg 1995;181:257–262.
23. Postlethwait RW. Benign tumors of the esophagus. Surgery of
the esophagus. 2nd ed. Norwalk, CT: Appleton-Century-
Crofts; 1986:345–354.
24. Preda F, Alloisio M, Lequaglie C, et al. Leiomyoma of the esoph-
agus. Tumori 1986;72:503–506.
25. Yang PS, Lee KS, Lee SJ, et al. Esophageal leiomyoma: radio-
logic findings in 12 patients. Korean J Radiol 2001;2:132–137.
26. Faivre J, Bory R, Moulinier B. Benign tumors of oesophagus:
value of endoscopy. Endoscopy 1978;10:264–268.
27. Songur Y, Okai T, Fujii T, et al. Endoscopic ultrasonography as
a guide to strip biopsy removal of esophageal submucosal tu-
mors. J Clin Gastroenterol 1995;20:77–79.
28. Ando N, Goto H, Niwa Y, et al. The diagnosis of GI stromal
tumors with EUS-guided fine needle aspiration with immuno-
histochemical analysis. Gastrointest Endosc 2002;55:37–43.
29. Boyce GA, Sivak MV Jr, Rosch T, et al. Evaluation of submu-
cosal upper gastrointestinal tract lesions by endoscopic ultra-
sound. Gastrointest Endosc 1991;37:449–454.
30. Stelow EB, Stanley MW, Mallery S, et al. Endoscopic
ultrasound-guided fine-needle aspiration findings of gastroin-
testinal leiomyomas and gastrointestinal stromal tumors. Am J
Clin Pathol 2003;119:703–708.
31. Henke AC, Salomao DR, Timmerman TG, Hughes JH. Fine-
needle aspiration cytology of esophageal leiomyomatosis. Diagn
Cytopathol 1999;21:197–199.
32. Matsui M, Goto H, Niwa Y, et al. Preliminary results of fine
needle aspiration biopsy histology in upper gastrointestinal sub-
mucosal tumors. Endoscopy 1998;30:750–755.
33. Megibow AJ, Balthazar EJ, Hulnick DH, et al. CT evaluation of
gastrointestinal leiomyomas and leiomyosarcomas. Am J Roent-
genol 1985;144:727–731.
34. Van Dam J, Rice TW, Sivak MV Jr. Endoscopic ultrasonogra-
phy and endoscopically guided needle aspiration for the diagno-
sis of upper gastrointestinal tract foregut cysts. Am J Gastroen-
terol 1992;87:762–765.
35. Geller A, Wang KK, DiMagno EP. Diagnosis of foregut dupli-
cation cysts by endoscopic ultrasonography. Gastroenterology
1995;109:838–842.
36. Glanz I, Grunebaum M. The radiological approach to leiomy-
oma of the oesophagus with a long-term follow-up. Clin Radiol
1977;28:197–200.
37. van Preyss B, Maesen F. Leiomyoma of the oesophagus:
follow-up of 14 years before operation. Br Med J (Clin Res Ed)
1982;285:1166.
38. Standerfer RJ, Paneth M. Recurrent leiomyoma of the oespha-
gus. Thorax 1982;37:478–479.
39. Zuccaro G Jr, Rice TW. Tumors of the esophagus. In: Brandt
146 Lee et al Management of Esophageal Leiomyoma
LJ, ed. Clinical practice of gastroenterology. Philadelphia:
Churchill Livingstone; 1999:131–134.
40. Fleischer DE, Haddad NG. Neoplasms of the esophagus. In:
Castell DO, Richter JE, eds. The Esophagus. 3rd ed. Philadel-
phia: Lippincott, Williams & Wilkins; 1999:252.
41. Hennessy TPJ, Cuschieri A. Tumours of the oesophagus. In:
Hennessy TPJ, Cuschieri A, eds. Surgery of the oesophagus.
London: Butterworth-Heinemann; 1992:275–327.
42. Bardini R, Segalin A, Ruol A, et al. Videothoracoscopic enucle-
ation of esophageal leiomyoma. Ann Thorac Surg 1992;54:
576–577.
43. Roviaro GC, Maciocco M, Varoli F, et al. Videothoracoscopic
treatment of oesophageal leiomyoma. Thorax 1998;53:190–
192.
44. Bardini R, Asolati M. Thoracoscopic resection of benign tu-
mours of the esophagus. Int Surg 1997;82:5–6.
45. Aurea P, Grazia M, Petrella F, Bazzocchi R. Giant leiomyoma of
the esophagus. Eur J Cardiothorac Surg 2002;22:1008–1010.
46. Solomon MP, Rosenblum H, Rosato FE. Leiomyoma of the
esophagus. Ann Surg 1984;199:246–248.
47. Deren MM, Lundell D, Saieh T, Wilson G. Leiomyoma of the
esophagus. Conn Med 1979;43:483–485.
48. Berry BE. Esophageal leiomyomata: spectrum of clinical presen-
tation and management. J LA State Med Soc 1980;132:102–
104.
49. Everitt NJ, Glinatsis M, McMahon MJ. Thoracoscopic enucle-
ation of leiomyoma of the oesophagus. Br J Surg 1992;79:643.
50. Nguyen NT, Alcocer JJ, Luketich JD. Thoracoscopic enucle-
ation of an esophageal leiomyoma. J Clin Gastroenterol 2000;
31:89–90.
51. Mafune K, Tanaka Y. Thoracoscopic enucleation of an esopha-
geal leiomyoma with balloon dilator assistance. Surg Today
1997;27:189–192.
52. Tamura K, Takamori S, Tayama K, et al. Thoracoscopic resec-
tion of a giant leiomyoma of the esophagus with a mediastinal
outgrowth. Ann Thorac Cardiovasc Surg 1998;4:351–353.
J Am Coll Surg
53. Schmid RA, Schob OM, Klotz HP, et al. VATS resection of an
oesophageal leiomyoma in a patient with neurofibromatosis
Recklinghausen. Eur J Cardiothorac Surg 1997;12:659–662.
54. Taniguchi E, Kamiike W, Iwase K, et al. Thoracoscopic enucle-
ation of a large leiomyoma located on the left side of the esoph-
ageal wall. Surg Endosc 1997;11:280–282.
55. Van Eijkelenburg P, Bove T, Peters O, et al. Thoracoscopic
removal of a leiomyoma of the oesophagus: a case report. Acta
Chir Belg 1996;96:223–225.
56. Roviaro GC, Rebuffat C, Varoli F, et al. Videoendoscopic tho-
racic surgery. Int Surg 1993;78:4–9.
57. van der Peet DL, Berends FJ, Klinkenberg-Knol EC, Cuesta
MA. Endoscopic treatment of benign esophageal tumors: case
report of three patients. Surg Endosc 2001;15:1489.
58. Gossot D, Fourquier P, el Meteini M, Celerier M. Technical
aspects of endoscopic removal of benign tumors of the esopha-
gus. Surg Endosc 1993;7:102–103.
59. Redan JA, Gardner JC, Tylutki FJ. Hand-assisted laparoscopy
for the removal of an esophageal leiomyoma. JSLS 2001;5:167–
169.
60. Izumi Y, Inoue H, Endo M. Combined endoluminal-
intracavitary thoracoscopic enucleation of leiomyoma of the
esophagus. A new method. Surg Endosc 1996;10:457–458.
61. Eda Y, Asaki S, Yamagata L, et al. Endoscopic treatment for
submucosal tumors of the esophagus: studies in 25 patients.
Gastroenterol Jpn 1990;25:411–416.
62. Watson TJ, Peters JH, DeMeester TR. Esophageal replacement
for end-stage benign esophageal disease. Surg Clin North Am
1997;77:1099–1113.
63. Banki F, Mason RJ, DeMeester SR, et al. Vagal-sparing esoph-
agectomy: a more physiologic alternative. Ann Surg 2002;236:
324–335; discussion 335–336.
64. Akiyama H, Tsurumaru M, Ono Y, et al. Esophagectomy with-
out thoracotomy with vagal preservation. J Am Coll Surg 1994;
178:83–85.