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Clinical Practices and Guidelines For Esophageal Leiomyoma

Oncology
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Introduction

Most esophageal tumors are malignant, with only 1% noted to be benign.[1] However, esophageal leiomyomas are the most common type of benign esophageal tumor, accounting for about 60-70% of these lesions.[1,2] Malignant transformation are felt to be rare, only occurring in less than 0.2% of cases.[3]
Esophageal leiomyomas are mesenchymal in origin and commonly arise in the muscularis layer within the distal two-thirds of the esophagus.[1,2,3] They have been linked to the Carney Triad, which has expanded to include gastric gastrointestinal stromal tumor (GIST), pulmonary chondroma, extra-adrenal paraganglioma, adrenal adenoma, and pheochromocytoma.[4]
Most of these leiomyomas are primary, solitary, intramural, and less than 10 cm in size. Tumor sizes greater than 10 cm are classified as giant leiomyomas of the esophagus and are more likely to have multiple primary foci of growth.[1,2,3]
Leiomyomas can occur at any age, with a peak incidence between the third to fifth decades of life and a mean age of 55.7 years. There is a 2:1 male predominance compared to women.[1,2,3]
The overall prognosis for esophageal leiomyomas is good with appropriate management due to the slow growth and benign nature of these tumors.

Symptoms

Most patients with an esophageal leiomyoma are asymptomatic. However, larger tumor size is associated with the presentation of symptoms, typically in tumors larger than 5 cm.
Symptoms are often ill-defined and non-specific but are related to compression of the esophagus and surrounding mediastinal structures, such as epigastric discomfort, dysphagia, odynophagia, regurgitation, diarrhea, gastrointestinal bleeding, and weight loss.[5,6]

Diagnostic Workup

Esophageal leiomyomas are primarily diagnosed as incidental findings on various modalities of imaging, usually during routine screening or work-up for another upper gastrointestinal pathology. Esophagogastroduodenoscopy (EGD) will often note an eccentric intraluminal narrowing in the esophagus with an overlying intact mucosa (Figure 1).
Figure 1. EGD imaging shows an eccentric intraluminal narrowing in the esophagus with an overlying intact mucosa.
A barium esophagram may show intraluminal narrowing from the compressive effects of the mass with a smoothly elevated filling defect, projecting away from the lumen and outlining the size of the tumor (Figure 2).[3,7]
Figure 2. Barium esophagram showing an intraluminal narrowing from the compressive effects of the mass with a smoothly elevated filling defect, projecting from the wall into the lumen and outlining the size of the tumor.
Endoscopic ultrasound (EUS) is becoming increasingly used as it readily delineates esophageal leiomyomas from malignancies via confirmation of the mass’ confinement to the intramural layer and the absence of nodal involvement.[1,3,7]
An IV contrast-enhanced CT scan is useful for further delineating involvement with other nearby mediastinal structures and the extent of the esophageal length involved. On CT, it is often described as a circumferential, eccentric, uniformly dense, sometimes lobulated, smooth mass around the esophageal wall (figure 3).[3,7]
CT imaging can be useful for guiding further procedural or surgical modalities if indicated.
Figure 3. IV contrast-enhancing CT imaging shows a circumferential, eccentric, uniformly dense, sometimes lobulated, smooth mass around the esophageal wall.
A needle biopsy (by EUS or other mechanism) is often not recommended for these lesions due to the risk of infection and characteristic radiologic findings; however, it may be warranted in instances where image findings are inconclusive or diagnostic uncertainty exists. Gross specimen analysis of Esophageal leiomyomas when resected enbloc usually denote a circumscribed and homogenous smooth lesion (Figure 4).
Figure 4. Gross specimen shows a circumscribed smooth (left), and homogenous (right) mass.
Histopathology of these specimens will show interlacing fascicles of spindle-shaped smooth muscle cells with abundant cytoplasm and minimal mitotic figures or cytoplasmic atypia.[3,8]
Its immunohistochemistry is usually negative for CD117 and CD34 (usually positive in GIST tumors), but positive for desmin and smooth muscle actin.[9]

Management

There is currently no consensus on the management of asymptomatic or small (<5cm) tumors. However, resection is indicated when the tumor is large (>5cm) or symptomatic via endoscopic or surgical modalities.[10]
Endoscopic submucosal resection can be used for short-segment esophageal leiomyoma, whereas surgical resection via right thoracotomy can be used for tumors of the middle third of the esophagus, and a left thoracotomy for tumors of the distal one-third.[11]
Surgical techniques are increasingly focused on video-assisted thoracoscopic or robotic-assisted techniques, though there are anecdotal concerns of increased esophageal leaks after the minimally invasive approaches. Robotic-assisted techniques may provide added advantages with ambidexterity, 3-dimensional orientation, and access to more limited/small spaces. Enucleation of the tumor with an esophageal myotomy or a resection of the tumor with the esophagus is performed.
If the esophagomyotomy crosses the gastroesophageal junction, a partial fundoplication may be needed. Esophagectomy may be indicated in giant leiomyomas of the esophagus or tumors involving long segments of the esophagus with a high risk of mucosal injury.
Key steps of the surgical procedure include the following: [10,12]
  1. Dissection of the mediastinal pleural reflections to better visualize the affected segment of the esophagus
  2. Myotomy of the outer longitudinal muscularis layer of the esophagus
  3. Dissection of the tumor from the submucosa
  4. Excision of the tumor
  5. Mucosal injury closure with 44-54Fr esophageal dilator to prevent stenosis
  6. Muscularis layer closure over any mucosal closure
  7. Buttress with pleural flap, intercostal muscle, or gastric fat pad
  8. Leak test
  9. Re-repair and/or conversion to open thoracotomy may be needed if a positive leak test is noted
  10. Placement of a nasogastric tube, and a pleural drain at the level of the esophageal repair
Postoperative management prioritizes pain management and continued monitoring for an esophageal leak. A barium esophagram is typically done in the postoperative period to evaluate for a delayed leak. Other than a 2-week postoperative follow-up, these patients do not require long-term follow-up as surgery is usually curative with total excision of the tumor, as long as it is a benign leiomyoma.
If surveillance ensues if surgery is not performed or an incomplete resection is performed, EUS is presently recommended as the primary modality; however, there is currently no consensus on the interval for follow-up. One study noted an average of 0.2 mm and 0.5 mm incremental growth in size in esophageal leiomyoma at 19-month and 70-month follow-ups with EUS imaging, respectively.[8] Therefore, long-term surveillance is typically not indicated and the overall prognosis is positive.

Summary

Esophageal leiomyomas are the most common type of benign esophageal tumor, usually linked to the Carney Triad. Most of these tumors are primary, solitary, intramural, and less than 10 cm in size.
Symptoms are nonspecific but related to compression of the esophagus and surrounding mediastinal structures when tumors are larger. Esophageal leiomyomas are primarily diagnosed as incidental findings on various modalities of imaging, usually during routine screening or work-up for another upper gastrointestinal pathology, and do not require further biopsy for diagnosis.
Intervention is indicated when the tumor is >5 cm and/or symptomatic via endoscopic submucosal resection or conventional surgical resection. Long-term surveillance is typically not indicated, with a good overall prognosis.

References

  1. Matthew G, Osueni A, Carter Y. Esophageal Leiomyoma. StatPearls Publishing. https://www.ncbi.nlm.nih.gov/books/NBK459298. 2022. Accessed September 5th, 2022.
  2. Jiang W, Rice TW, Goldblum JR. Esophageal leiomyoma: experience from a single institution. Diseases of the Esophagus. 2013;26(2):167–174.
  3. Elbawab H, Fahad AlOtaibi A, Binammar AA, et al. Giant Esophageal Leiomyoma: Diagnostic and Therapeutic Challenges. American Journal of Case Reports. 2021;22(1):e934557-1–e934557-6.
  4. Tansir G, Dash NR, Galodha S, Das P, Shamim SA, Rastogi S. Carney’s triad in an adult male from a tertiary care center in India: a case report. J Med Case Rep. 2021;15(1):559.
  5. Mutrie C, Donahue D, Wain J, et al. Esophageal Leiomyoma: A 40-Year Experience. Annals of Thoracic Surgery. 2005;179(1):1122-1125.
  6. Pham DH, Nguyen ND, Do ML, et al. Video-assisted thoracoscopy or laparoscopy for enucleation of esophageal leiomyoma: A seven-year single center experience of 75 cases. Journal of Visceral Surgery. 2022;159(2):108-113.
  7. Yang PS, Lee KS, Lee SJ, et al. Esophageal Leiomyoma: Radiologic Findings in 12 Patients. Esophageal Leiomyoma: Radiologic Findings in 12 Patients. 2001;2(3):132-137.
  8. Codipilly DC, Fang H, Alexander JA, Katzka DA, Ravi K. Subepithelial esophageal tumors: a single-center review of resected and surveilled lesions. Gastrointestinal Endoscopy. 2018:87(2):370-377.
  9. Kaveh Sharzehi, Amrita Sethi, Thomas Savides. AGA Clinical Practice Update on Management of Subepithelial Lesions Encountered During Routine Endoscopy: Expert Review. Clinical Gastroenterology and Hepatology. 2022.
  10. Ivey N, Rochester SN, Bolton W, Stephenson J, Ben-Or S. Robotic resection of an esophageal leiomyoma. Multimed Man Cardiothorac Surg. 2020.
  11. Zhu S, Lin J, Huang S. Successful en bloc endoscopic full-thickness resection of a giant cervical esophageal leiomyoma originating from muscularis propria. J Cardiothorac Surg. 2019;14:16.
  12. Gadelkarim M, Harpole B, Abdelsattar Z. Totally robotic enucleation of a mid-esophageal leiomyoma. Multimed Man Cardiothorac Surg. 2022