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GENERAL THORACIC

Esophageal Leiomyoma: A 40-Year Experience


Christopher J. Mutrie, MD, Dean M. Donahue, MD, John C. Wain, MD,
Cameron D. Wright, MD, Henning A. Gaissert, MD, Hermes C. Grillo, MD,
Douglas J. Mathisen, MD, and James S. Allan, MD
Division of Thoracic Surgery, Department of Surgery, Massachusetts General Hospital, Harvard Medical School, Boston,
Massachusetts

Background. Esophageal leiomyomas, although infre- leiomyomas. Eighty-four percent of the lesions in symp-
quent, are the most common benign intramural tumors of tomatic patients occurred in the lower two-thirds of the
the esophagus. They represent 10% of all gastrointestinal esophagus, with epigastric discomfort being the most
leiomyomas and frequently cause symptoms, necessitat- common presenting symptom. Among patients operated
ing resection. on solely for leiomyoma, 97% were enucleated without
Methods. The Massachusetts General Hospital Patho- an esophageal resection. None of the leiomyomas
logic Database was reviewed over a 40-year period for showed malignant transformation or recurrence. All
patients who underwent surgical resection of esophageal symptomatic patients had relief of symptoms, with no
leiomyomas. Data analyzed included demographic infor- perioperative morbidity or mortality.
mation, presenting symptoms, tumor location, tumor Conclusions. In a large pathologic series, over half of all
characteristics and histology, diagnostic procedures, and patients with esophageal leiomyomas were symptomatic.
treatment modalities/outcomes. Fifty-three patients were Larger tumors were significantly more likely to be symp-
identified; 31 patients were symptomatic from their tomatic. Local enucleation by a variety of surgical ap-
leiomyomas. proaches was accomplished in most patients. All symptom-
Results. Symptomatic patients presented at a mean age atic patients had relief of symptoms, with no perioperative
of 44 years old and exhibited a twofold male predomi- morbidity or mortality. There was no observed tendency for
nance. Mean tumor diameter among symptomatic pa- malignant transformation or recurrence.
tients was 5.3 cm, as compared to 1.5 cm in asymptomatic
patients (p < 0.0001). Thirty of the symptomatic patients (Ann Thorac Surg 2005;79:1122–5)
had solitary leiomyomas, and 1 patient had five separate © 2005 by The Society of Thoracic Surgeons

E sophageal leiomyomas are the most common benign


intramural tumors of the esophagus. Still, these
tumors are relatively rare, with autopsy series data show-
less than 5 cm in diameter are asymptomatic [1]. In a
comprehensive review of more than 800 cases reported
in the world literature, only two (0.2%) were docu-
ing an incidence of 0.006 to 0.1% [1]. By comparison, mented to show malignant transformation from
esophageal carcinoma is approximately 50 times more leiomyoma to leiomyosarcoma [2].
common than leiomyoma [2]. Esophageal leiomyomas
are typically found in patients between 20 and 50 years
old, and represent 10% of all gastrointestinal leiomyo-
Patients and Methods
mas. They are multiple in 3% to 10% of patients and are A search of the Massachusetts General Hospital Patho-
found more often in men by a ratio of 2:1 [3–5]. Eighty logic Database was performed to identify all cases of
percent occur in the middle to lower third of the esoph- esophageal leiomyoma that were surgically resected be-
agus [6]. Historically, dysphagia and odynophagia are the tween January 1962 and January 2002. Office records,
most common complaints of those patients who are hospital records, or both from all identified patients were
symptomatic [7]. also reviewed. The data extracted from the medical
Leiomyomas are most commonly diagnosed on con- records included demographic information, the present-
trast esophagography, where they appear as a smooth ing symptoms, the location of the tumor in the esopha-
concave filling defect within the esophageal lumen [1]. gus, the size and shape of the tumor, the diagnostic
They can also be seen on chest radiography, computed procedure, the treatment modality, the incidence of re-
tomography, and magnetic resonance imaging. currence, surgical complications and deaths. Information
Pathologically, leiomyomas consist of interlacing was cataloged and analyzed.
bundles of smooth muscle cells and occasionally can Fifty-three patients were identified. Of these, 31 pa-
grow as large as 20 cm in diameter. Generally, tumors tients (58%) underwent surgery solely for the resection of
symptomatic leiomyomas. One patient (2%) underwent
Accepted for publication Aug 13, 2004. resection of an asymptomatic leiomyoma. The remaining
Address reprint requests to Dr Allan, Massachusetts General Hospital, Blake 21 patients (41%) had thoracic procedures for other
1570, 55 Fruit St, Boston, MA 02114; e-mail: jallan@partners.org. indications (eg, lung cancer, esophageal cancer, hiatal

© 2005 by The Society of Thoracic Surgeons 0003-4975/05/$30.00


Published by Elsevier Inc doi:10.1016/j.athoracsur.2004.08.029
Ann Thorac Surg MUTRIE ET AL 1123

GENERAL THORACIC
2005;79:1122–5 ESOPHAGEAL LEIOMYOMA

Table 1. Presenting Complaint(s) Among Symptomatic


Patients (n ⫽ 31)
Symptom Percentage

Epigastric discomfort 68
Dysphagia 52
Regurgitation 23
GI bleed 10
Diarrhea 3
Weight loss 3

GI ⫽ gastrointestinal.

operated on solely for leiomyoma (n ⫽ 31), the mean age


was 43.8 years old (range, 19 to 76 years old).

Size
Fig 1. Anatomical location of leiomyoma.  ⫽ upper third; o ⫽
middle third; s ⫽ lower third. The mean tumor diameter of all patients studied was 3.7
cm (range, 0.2 to 17.0 cm). Among symptomatic patients
operated on solely for leiomyoma (n ⫽ 31), the mean
hernias, etc.), and resection of an incidentally discovered tumor diameter was 5.3 cm (range, 0.8 to 17.0 cm).
leiomyoma was concurrently performed. Follow-up data Asymptomatic patients had a significantly lower mean
that adequately described the patient’s health status was tumor diameter (1.5 cm [range, 0.2 to 6.0 cm]; p ⬍ 0.00001,
available on 27 of 53 patients in this series, with a mean Student’s t test).
follow-up interval of 6.3 years.
Location
Among all patients studied, the majority of the tumors
Results arose in the lower (53%) and middle (43%) thirds of the
Gender esophagus (Fig 1). Only 4% were in the upper third of the
Sixty-four percent (n ⫽ 34) of all patients studied were esophagus. Among symptomatic patients operated on
male; 36% (n ⫽ 19) were female, yielding a male predom- solely for leiomyoma (n ⫽ 31), the tumors were distrib-
inance of 1.8:1. Among symptomatic patients operated on uted as follows: lower third (39%), middle third (55%),
solely for leiomyoma (n ⫽ 31), 66% (n ⫽ 21) were male, and upper third (6%).
and 34% (n ⫽ 10) were female, yielding a male predom-
inance of 2.1:1.
Tumor Characteristics
Among all patients studied, 48 patients (91%) had solitary
Age tumors and 5 (9%) had multiple tumors. Three leiomyo-
The mean age of all patients studied was 51.7 years old mas (6%) were calcified. Fourteen (26%) patients had
(range, 19 to 81 years old). Among symptomatic patients lobulated masses. Three other patients (6%) had serpig-
enous leiomyomas. One leiomyoma (2%) was horseshoe-
shaped (Fig 2). Among symptomatic patients operated on
solely for leiomyoma (n ⫽ 31), 30 patients (97%) had
solitary tumors and 1 patient (3%) had multiple tumors.
Three leiomyomas (9%) were calcified. Thirteen patients
(41%) had lobulated masses. Three other patients (9%)
had serpigenous leiomyomas. One leiomyoma (3%)
was horseshoe-shaped. None of the lesions showed any
foci of leiomyosarcoma, suggestive of malignant
degeneration.

Symptoms and Associated Disorders


Among symptomatic patients operated on solely for
leiomyoma (n ⫽ 31), 21 patients (68%) reported epigas-
tric distress (including heartburn and other epigastric
symptoms commonly attributed to gastroesophageal
reflux). Sixteen patients (52%) reported dysphagia.
Other symptoms were reported less frequently (see
Fig 2. Horseshoe leiomyoma. Table 1).
1124 MUTRIE ET AL Ann Thorac Surg
GENERAL THORACIC

ESOPHAGEAL LEIOMYOMA 2005;79:1122–5

Table 2. Surgical Approach in Patients Operated on Solely 838 patients from the world literature, reported by Ser-
for Leiomyoma (n ⫽ 32) emetis and colleagues in 1971 [5]. Our data also show that
leiomyoma are preferentially found in the lower two-
Approach Number
thirds of the esophagus, which is consistent with the
Right thoracotomy 21 normal anatomical distribution of smooth muscle within
Left thoracotomy 6 the esophageal wall. These benign tumors tend to
Thoracoabdominal 1 present as solitary round masses without calcification.
Midline laparotomy 1 A slight majority of our patients were symptomatic and
Right VATS 1 presented with complaints of epigastric distress, dyspha-
Transverse cervical 1 gia, or both. Seremetis and coworkers [5] reported similar
symptomology in their review, noting that dysphagia and
VATS ⫽ video-assisted thoracic surgery. pyrosis (heartburn) were the most common presenting
symptoms. However, Seremetis noted in his own series
of 19 patients that only those with concomitant hiatal
Diagnostic Evaluation hernias presented with pyrosis. Our data also show the
All symptomatic patients underwent barium esophagog- intuitive finding that large leiomyomas are significantly
raphy. The majority of patients treated within the last more likely to be symptomatic. The mean diameter of
decade also received chest computed tomographic scan. tumors in symptomatic patients was 5.3 cm, as opposed
Esophageal ultrasounds and endoscopic biopsies were to 1.5 cm for patients with incidentally found leiomyomas
used occasionally by referring physicians, although the (p ⬍ 0.001; Student’s t test).
ultimate management of those patients so examined was The majority of leiomyomas in this series were re-
not significantly impacted. We do not routinely perform sected by open thoracotomy by enucleating the lesion
endoscopic biopsies ourselves unless there is some diag- from within the muscular wall of the esophagus, without
nostic uncertainty that would make resection unwar- entering the mucosal lumen. It has been our practice to
ranted. There is also some anecdotal experience suggest- routinely reapproximate the muscular layer of the esoph-
ing that a preoperative biopsy (and consequent mucosal agus with interrupted silk suture following enucleation,
adhesions) may impede easy enucleation of the tumor. which may be significant in the prevention of postoper-
ative complications such as diverticula. We have found
Surgical Approach and Outcomes this approach to be both safe and efficacious, and report
Among symptomatic patients operated on solely for no morbidity or mortality using these techniques. In
leiomyoma (n ⫽ 31), 21 patients (66%) were approached particular, we have not observed an increased incidence
through a right thoracotomy (limited or posterolateral). of heartburn or epigastric pain following resection. This
The remaining 10 patients (34%) were approached in is most likely due to our efforts to minimize hiatal
accordance with Table 2. In all patients except one, the dissection and mobilization during the operation.
leiomyoma was enucleated by dissection within the mus- In this series, we are reporting only 1 patient whose
cular wall of the esophagus. In all patient the muscular leiomyoma was removed by a video thoracoscopic ap-
layer was reapproximated with interrupted silk sutures. proach. We recognize that thoracoscopic removal of
One patient sustained a full-thickness injury to the these benign tumors is currently an excellent approach to
esophageal wall during enucleation, which was directly the management of most simple leiomyomas. The low
repaired and buttressed with a pedicled intercostal mus- frequency of minimally invasive resections in this series
cle flap at the time of surgery without sequelae. One is due both to its historical nature and due to individual
patient required an esophagogastrectomy for removal of surgeon preferences. Bardini and colleagues [8] first
a giant (⬎ 10 cm) leiomyoma. All symptomatic patients described video thoracoscopic enucleation of esophageal
had relief of symptoms. There were no postoperative leiomyomas in 1992, and several investigators have re-
complications or perioperative deaths in this group. No ported favorable results using combined thoracoscopic
patients represented to our care with recurrence. and endoscopic approaches [9, 10]. We believe that
minimally invasive procedures are certainly acceptable,
particularly for smaller rounded solitary lesions, where
Comment the submucosal plane can be easily identified and
We report here a large single-institution case series of developed.
esophageal leiomyoma. Our data first point to the rarity We have described a pathologic series of esophageal
of this entity. Despite being a tertiary referral center for leiomyomas in which slightly over half of the patients
surgical esophageal disease, our hospital performed less were symptomatic. Larger tumors were significantly
than one operation per year solely for the treatment of a more likely to be symptomatic. Local enucleation by a
symptomatic esophageal leiomyoma. These data are in variety of surgical approaches was accomplished in the
concordance with reported autopsy series suggesting an vast majority of patients. One patient with a giant leiomy-
overall incidence of 0.006% to 0.1% for esophageal oma required esophagogastrectomy. None of the
leiomyoma [1]. Our data also show that leiomyoma have leiomyomas showed malignant degeneration or re-
a twofold male sex predominance. This finding is in curred. All symptomatic patients had relief of symptoms,
agreement with the 1.9:1 ratio found in a compilation of with no perioperative morbidity or mortality.
Ann Thorac Surg MUTRIE ET AL 1125

GENERAL THORACIC
2005;79:1122–5 ESOPHAGEAL LEIOMYOMA

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Online Discussion Forum

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Early Experience With Activated Recombinant Factor VII
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Michael E. Halkos, MD, Jerrold H. Levy, MD, Edward
diac Surgery, Cardiac Transplantation, Lung Transplan-
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tation, and Aortic and Vascular Surgery.
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© 2005 by The Society of Thoracic Surgeons Ann Thorac Surg 2005;79:1125 • 0003-4975/05/$30.00
Published by Elsevier Inc

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