Esophageal Leiomyoma

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Background

Benign tumors of the esophagus are rare lesions that constitute less than 1% of esophageal neoplasms. Nearly two thirds of benign esophageal tumors are leiomyomas; the others mostly are polyps and cysts. Thus, leiomyomas are the most common benign tumors of the esophagus.[1]

Pathophysiology

Leiomyomas represent a hyperproliferation of interlacing bundles of smooth muscle cells that are well-demarcated by adjacent tissue or by a smooth connective tissue capsule. They usually arise as intramural growths, most commonly along the distal two thirds of the esophagus. They are multiple in approximately 5% of patients.

The majority of leiomyomas have been discovered incidentally during evaluation for dysphagia or during autopsy. Bleeding rarely occurs in cases of benign disease but typically is observed with leiomyosarcoma, the malignant counterpart of this tumor. The potential for malignant degeneration of leiomyomas is extremely small. In the distal esophagus, leiomyomas may reach large proportions and may encroach on the cardia of the stomach.

In a retrospective review of the Cleveland Clinic pathology database, Jiang et al investigated the clinicopathologic features of esophageal leiomyomas. Analyzing 30 cases from 28 patients, the investigators found mean tumor sizes among symptomatic and asymptomatic patients to be 5.2 cm and 0.4 cm, respectively. In symptomatic patients, the most common complaint was dysphagia (71.4%). The majority of esophageal leiomyomas (69%) were found in the distal and middle thirds of the esophagus, with most (69.6%) developing from the muscularis propria layer. For the most part, the tumors were made up of bland spindle cells with low cellularity and lacked both nuclear atypia and mitotic activity; cells were found to be positive for smooth muscle actin and negative for the CD34 and CD117 proteins. Among the 22 patients (78.6%) for whom follow-up information was available, no leiomyoma-related adverse events were found.[1]

Epidemiology

Frequency

International

Esophageal leiomyomas comprise less than 0.6% of all esophageal neoplasms, both in the United States and worldwide.

Race

No known differences

Sex

No known differences

Age

Typically occur in individuals aged 20-50 years

History

Physical

Imaging Studies

Procedures

Histologic Findings

Histologically, the tumors are comprised of bundles of interlacing smooth muscle cells, well-demarcated by adjacent tissue or by a definitive connective tissue capsule. They are composed of fascicles of spindle cells that tend to intersect with each other at varying angles. The tumor cells have blunt-ended elongated nuclei and show minimal atypia with few mitotic figures.

Surgical Care

Surgical excision is recommended for symptomatic leiomyomas and those greater than 5 cm. Asymptomatic or smaller lesions should be followed periodically with barium swallow.

Resection is the only way to confirm that a tumor is not malignant. Periodic follow-up of smaller lesions is recommended because leiomyomas have a characteristic radiographic appearance, slow growth rate, and low risk of malignant degeneration. Tumors of the middle third of the esophagus are approached using a right thoracotomy; tumors in the distal third of the esophagus are resected through a left thoracotomy.

The outer esophageal muscle is gently incised longitudinally in order to reveal the lesion. Careful dissection is performed to separate and remove the leiomyoma from the underlying submucosa.

If the mucosa has been opened during dissection, the underlying mucosa is reapproximated, followed by closure of the longitudinal muscle. Some authors have shown that large extramucosal defects may be left open without subsequent complications developing.

Segmental esophageal resection may be indicated for giant leiomyomas of the cardia.

While open surgical technique is the traditional mainstay of therapy for leiomyomas, combined esophagoscopy and video-assisted resection (thoracoscopy) or laparoscopic transhiatal resection are being increasingly performed.[6, 7, 8, 9]

Endoscopic removal of these tumors has been attempted, but this technique cannot be considered standard of care.

However, a retrospective study by Wang el al indicated that endoscopic submucosal tunnel dissection (ESTD) is a safe and effective treatment for esophageal leiomyoma and that it has advantages over endoscopic submucosal dissection (ESD). The study involved 39 patients who underwent resection for leiomyoma, either by ESTD or ESD. Compared with ESD, ESTD was associated with shorter operating times, hospital stays, and time needed for incision healing. Moreover, the ETSD and ESD groups showed no significant difference in the incidence of complications.[10]

Consultations

Thoracic surgeon

Prognosis

Following complete surgical resection, no recurrence has ever been reported.

Author

Kavitha Kumbum, MD, Associate Program Director and Attending Physician, Gastroenterology Fellowship Program, Division of Gastroenterology, Bronx Lebanon Hospital Center, Albert Einstein College of Medicine

Disclosure: Nothing to disclose.

Specialty Editors

Maurice A Cerulli, MD, FACP, FACG, FASGE, AGAF, Associate Professor of Clinical Medicine, Albert Einstein College of Medicine of Yeshiva University; Associate Professor of Clinical Medicine, Hofstra Medical School

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Alex J Mechaber, MD, FACP, Senior Associate Dean for Undergraduate Medical Education, Associate Professor of Medicine, University of Miami Miller School of Medicine

Disclosure: Nothing to disclose.

Chief Editor

Julian Katz, MD, Clinical Professor of Medicine, Drexel University College of Medicine

Disclosure: Nothing to disclose.

Additional Contributors

Simmy Bank, MD Chair, Professor, Department of Internal Medicine, Division of Gastroenterology, Long Island Jewish Hospital, Albert Einstein College of Medicine

Disclosure: Nothing to disclose.

Anastasios K Konstantakos, MD Clinical Associate Surgeon, Department of Cardiovascular Surgery, Billings Clinic

Disclosure: Nothing to disclose.

Sandeep Mukherjee, MB, BCh, MPH, FRCPC Associate Professor, Department of Internal Medicine, Section of Gastroenterology and Hepatology, University of Nebraska Medical Center; Consulting Staff, Section of Gastroenterology and Hepatology, Veteran Affairs Medical Center

Disclosure: Merck Honoraria Speaking and teaching; Ikaria Pharmaceuticals Honoraria Board membership

References

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