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

United States and international data

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

Age-, sex-, and race-related demographics

Esophageal leiomyomas typically occur in individuals aged 20-50 years.

No known sex or race predilection is recognized.

Prognosis

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

History

Esophageal leiomyomas rarely cause symptoms when they are smaller than 5 cm in diameter. Large tumors can cause dysphagia, vague retrosternal discomfort, chest pain, esophageal obstruction, and regurgitation. Rarely, they can cause gastrointestinal bleeding, with erosion through the mucosa.

Physical

Other than the nonspecific symptoms associated with esophageal leiomyomas, very few physical findings are ever noted. In extremely rare cases where severe esophageal obstruction is caused by a leiomyoma, weight loss and muscle wasting may be observed.

Imaging Studies

Esophageal leiomyoma can be visualized using barium swallow. The classic appearance is a smooth concave mass underlying intact mucosa. Distinct sharp angles are seen at the junction of the tumor and normal tissue. Encroachment onto the esophageal lumen usually is observed.

Esophageal ultrasonography can be helpful in demonstrating a smooth, typically round, mass located in the muscularis without encroachment into the overlying mucosa or underlying adventitia.

Esophagoscopy may reveal a nonspecific tumor in the wall of the esophagus, typically without mucosal involvement (ulceration or erosion).

Procedures

Upon endoscopy, the lesions are identified as mobile submucosal masses.

Esophagoscopy should be performed to rule out carcinoma. It is important to remember that if a leiomyoma is suspected at esophagoscopy, a biopsy should not be performed if it would cause scarring at the biopsy site, which would hamper definitive extramucosal resection at surgery. A brush cytology may be performed. A leiomyoma (especially an ulcerated one) routinely is biopsied through the endoscope without any problems occurring in the ability to resect at a later date. In fact, an ulcer in a leiomyoma may be considered much worse than a mucosal biopsy.

Esophageal ultrasonography has aided in the diagnosis of leiomyomas, which demonstrate a homogeneous region of hypoechogenicity juxtaposed with the overlying mucosa.

Endoscopic ultrasonography is a useful technique for diagnosing and reaching treatment decisions for esophageal leiomyoma.[5]

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

Consultation with a thoracic surgeon may be beneficial.

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

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

Disclosure: Received salary from Medscape for employment. for: Medscape.

Chief Editor

Praveen K Roy, MD, AGAF, Chief of Gastroenterology, Presbyterian Hospital; Medical Director of Endoscopy, Presbyterian Medical Group; Adjunct Associate Research Scientist, Lovelace Respiratory Research Institute

Disclosure: Nothing to disclose.

Additional Contributors

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.

Acknowledgements

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

  1. Jiang W, Rice TW, Goldblum JR. Esophageal leiomyoma: experience from a single institution. Dis Esophagus. 2013 Feb-Mar. 26(2):167-74. [View Abstract]
  2. Gupta V, Lal A, Sinha SK, Nada R, Gupta NM. Leiomyomatosis of the esophagus: experience over a decade. J Gastrointest Surg. 2009 Feb. 13(2):206-11. [View Abstract]
  3. Choi H, Kim S, Moon JH, et al. Multiple endocrine neoplasia type 1 with multiple leiomyomas linked to a novel mutation in the MEN1 gene. Yonsei Med J. 2008 Aug 30. 49(4):655-61. [View Abstract]
  4. Ray S, Saluja SS, Gupta R, Chattopadhyay TK. Esophageal leiomyomatosis -- an unusual cause of pseudoachalasia. Can J Gastroenterol. 2008 Feb. 22(2):187-9. [View Abstract]
  5. Xu GQ, Qian JJ, Chen MH, Ren GP, Chen HT. Endoscopic ultrasonography for the diagnosis and selecting treatment of esophageal leiomyoma. J Gastroenterol Hepatol. 2012 Mar. 27(3):521-5. [View Abstract]
  6. Jiang G, Zhao H, Yang F, et al. Thoracoscopic enucleation of esophageal leiomyoma: a retrospective study on 40 cases. Dis Esophagus. 2009. 22(3):279-83. [View Abstract]
  7. Obuchi T, Sasaki A, Nitta H, Koeda K, Ikeda K, Wakabayashi G. Minimally invasive surgical enucleation for esophageal leiomyoma: report of seven cases. Dis Esophagus. 2010 Jan. 23(1):E1-4. [View Abstract]
  8. Shimada Y, Okumura T, Nagata T, et al. Successful enucleation of a fluorine-18-fluorodeoxyglucose positron emission tomography positive esophageal leiomyoma in the prone position using sponge spacer and intra-esophageal balloon compression. Gen Thorac Cardiovasc Surg. 2012 Aug. 60(8):542-5. [View Abstract]
  9. Luh SP, Hou SM, Fang CC, Chen CY. Video-thoracoscopic enucleation of esophageal leiomyoma. World J Surg Oncol. 2012 Mar 16. 10:52. [View Abstract]
  10. Wang L, Ren W, Zhang Z, et al. Retrospective study of endoscopic submucosal tunnel dissection (ESTD) for surgical resection of esophageal leiomyoma. Surg Endosc. 2013 Nov. 27(11):4259-66. [View Abstract]
  11. Bonavina L, Segalin A, Rosati R, Pavanello M, Peracchia A. Surgical therapy of esophageal leiomyoma. J Am Coll Surg. 1995 Sep. 181(3):257-62. [View Abstract]
  12. Boran C, Sengul N, Balaban YH, Gurel S. Multinodular leiomyoma of the esophagus with internodular hydropic degeneration and bulbous serosal protrusions similar to cotyledonoid uterine leiomyoma. Dis Esophagus. 2007. 20(2):187-9. [View Abstract]
  13. Chiu PW, Lee YT, Ng EK. Resection of esophageal leiomyoma using an endoscopic submucosal dissection technique. Endoscopy. 2006. 38 Suppl 2:E4. [View Abstract]
  14. Elli E, Espat NJ, Berger R, Jacobsen G, Knoblock L, Horgan S. Robotic-assisted thoracoscopic resection of esophageal leiomyoma. Surg Endosc. 2004 Apr. 18(4):713-6. [View Abstract]
  15. Kent M, d'Amato T, Nordman C, et al. Minimally invasive resection of benign esophageal tumors. J Thorac Cardiovasc Surg. 2007 Jul. 134(1):176-81. [View Abstract]
  16. Knop S, Schupp M, Wardelmann E, et al. A new case of Carney triad: gastrointestinal stromal tumours and leiomyoma of the oesophagus do not show activating mutations of KIT and platelet-derived growth factor receptor alpha. J Clin Pathol. 2006 Oct. 59(10):1097-9. [View Abstract]
  17. Konstantakos AK, Douglas WI, Abdul-Karim FW, et al. Arteriovenous malformation of the esophagus disguised as a leiomyoma. Ann Thorac Surg. 1995. 60:1798-1800. [View Abstract]
  18. Meirelles GS, Ravizzini G, Yeung HW, Akhurst T. Esophageal leiomyoma: a rare cause of false-positive FDG scans. Clin Nucl Med. 2006 Jun. 31(6):342-4. [View Abstract]
  19. Memisoglu E, Agarwal B, Akduman I, Prather C, Collins B, Civelek AC. Multimodality diagnostic imaging of diffuse esophageal leiomyomatosis. J Comput Assist Tomogr. 2006 Jan-Feb. 30(1):100-4. [View Abstract]
  20. Nemir P Jr, Wallace HW, Fallahnejad M. Diagnosis and surgical management of benign diseases of the esophagus. Curr Probl Surg. 1976 Mar. 13(3):1-74. [View Abstract]
  21. Palanivelu C, Rangarajan M, Senthilkumar R, Annapoorni S, Jategaonkar PA. Thoracoscopic management of benign tumors of the mid-esophagus: a retrospective study. Int J Surg. 2007 Oct. 5(5):328-31. [View Abstract]
  22. Postlethwait RW, Lowe JE. Benign tumors and cysts of the esophagus. Orringer MB, Zuidema GD, eds. Shackelford's Surgery of the Alimentary Tract. 4th ed. Philadelphia, Pa: WB Saunders; 1996. Vol 1: 369-86.
  23. Prenzel KL, Schafer E, Stippel D, Beckurts KT, Holscher AH. Multiple giant leiomyomas of the esophagus and stomach. Dis Esophagus. 2006. 19(6):504-8. [View Abstract]
  24. Priego P, Lobo E, Rodriguez G, Cabanas J, Perez de Oteyza J, Fresneda V. Endoscopic treatment of oesophageal leiomyoma: four new cases. Clin Transl Oncol. 2007 Feb. 9(2):106-9. [View Abstract]
  25. Punpale A, Rangole A, Bhambhani N, et al. Leiomyoma of esophagus. Ann Thorac Cardiovasc Surg. 2007 Apr. 13(2):78-81. [View Abstract]
  26. Tio TL, Tytgat GN, eds. Atlas of Transintestinal Ultrasonography. Aalsmeer, The Netherlands: Smith Klein & French b.v; 1986.
  27. Wong NA, Pawade J. Mast cell-rich leiomyomas should not be mistaken for gastrointestinal stromal tumours. Histopathology. 2007 Aug. 51(2):273-5. [View Abstract]
  28. Yamada H, Morita T, Fujita M, Miyasaka Y, Senmaru N, Oshikiri T. Long-term results following thoracoscopic surgery for esophageal leiomyoma in four patients. Hepatogastroenterology. 2007 Sep. 54(78):1713-5. [View Abstract]
  29. Zaninotto G, Portale G, Costantini M, et al. Minimally invasive enucleation of esophageal leiomyoma. Surg Endosc. 2006 Dec. 20(12):1904-8. [View Abstract]