Intramural Benign Esophageal Tumors

Published on 20/07/2015 by admin

Filed under Radiology

Last modified 22/04/2025

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 Discrete mass; solitary (most common) or multiple

image Round or ovoid filling defects sharply outlined by barium on each side (en face view)
image Narrowed (tangential view) or stretched and widened (en face view) esophageal lumen
image Smooth surface lesion, with upper and lower borders of lesion forming right or slightly obtuse angles with adjacent esophageal wall (profile view)
image Overlying mucosa may ulcerate
• Leiomyoma: ± amorphous or punctate calcifications
• Esophageal gastrointestinal stromal tumor (GIST)

image May be large mass
image May ulcerate with gas ± contrast medium entering cavity
• CT: Discrete mass in wall; no signs of invasion or metastases

image Helps distinguish lipoma (fat density) and other mediastinal masses (e.g., mediastinal cyst)

TOP DIFFERENTIAL DIAGNOSES

• Mediastinal tumor
• Normal mediastinal structures
• Esophageal carcinoma
• Foreign body

CLINICAL ISSUES

• Asymptomatic: No treatment
• Large, symptomatic lesions: Enucleation or esophageal resection with gastric interposition

DIAGNOSTIC CHECKLIST

• Most intramural masses are benign (unlike gastric tumors)
• Leiomyomas are much more common than GIST in esophagus (but not in stomach)
• Calcifications suggest leiomyoma; almost never occur in other benign/malignant esophageal tumors
image
(Left) Film from a barium esophagram demonstrates a mass image causing eccentric narrowing of the distal lumen. The mass forms obtuse angles with the wall, and the esophageal folds and mucosa are intact. A leiomyoma was enucleated endoscopically.

image
(Right) Single-contrast esophagram shows an en face view of an intramural mass in the distal esophagus with central ulceration image due to leiomyoma. The traction diverticulum image is an incidental finding.
image
(Left) Coronal CECT in a 24-year-old man shows a huge, soft tissue density mass that envelops and displaces the distal esophagus image. Small foci of calcification image are noted. The mass was resected and proved to be a benign leiomyoma arising from the esophageal wall.

image
(Right) Axial CECT in a 73-year-old woman shows a huge esophageal mass image with a large central ulceration image that contains gas due to communication with the esophageal lumen. The central cavitation is typical of a GIST; the esophagus is an unusual site.

TERMINOLOGY

Definitions

• Benign mass composed of 1 or more tissue elements of esophageal wall

IMAGING

General Features

• Best diagnostic clue

image Intramural mass with smooth surface and slightly obtuse borders on barium esophagram
• Size

image 1 cm to > 10 cm
• Other

image Types include leiomyoma, GI stromal tumor, granular cell, lipoma, hemangioma, hamartoma

Radiographic Findings

• Fluoroscopic-guided barium studies

image Discrete mass; solitary (most common) or multiple
image Round or ovoid filling defects sharply outlined by barium on each side (en face view)

– Overlying mucosa may ulcerate
image Smooth surface lesion, with upper and lower borders forming right or slightly obtuse angles with adjacent esophageal wall (profile view)
image Narrowed (tangential view) or stretched and widened (en face view) esophageal lumen

– Varying degree of obstruction
image Leiomyoma ± amorphous or punctate calcifications
image Esophageal gastrointestinal stromal tumor (GIST)

– May be large mass
– May ulcerate with gas ± contrast medium entering cavity
image Granular cell: Distal 1/3 > middle 1/3; 0.5-2.0 cm
image Hamartoma

– Large and pedunculated; mimics fibrovascular polyp
– Diffuse, nodular mucosa composed of tiny, innumerable, hamartomatous polyps (hereditary)

CT Findings

• Discrete mass in wall; no signs of invasion or metastases
• Helps distinguish lipoma (fat density) and other mediastinal masses (e.g., mediastinal cyst)
• MPRs help display size and relationships of large masses

image e.g., some leiomyomas and GIST

Ultrasonographic Findings

• Endoscopic ultrasound

image Leiomyoma: Hypoechoic and homogeneous mass with sharply demarcated margins in muscular layer
image Granular cell: Hypo- to isoechoic mass in submucosa
image Lipoma: Homogeneous hyperechoic mass with smooth outer margins in submucosa
image Enteric duplication cyst: Sonolucent, spherical mass

Imaging Recommendations

• Best imaging tool

image Barium studies followed by CT for large mass

DIFFERENTIAL DIAGNOSIS

Mediastinal Tumor

• Compresses or indents esophagus with obtuse margins
• CT better shows extent, origin, and nature of mass

Normal Mediastinal Structures

• Indentation by aorta, left main bronchus, aberrant or dilated vessels

Esophageal Carcinoma

• “Apple core” or eccentric mucosal mass

Foreign Body

• Intraluminal mass, often above stricture

PATHOLOGY

General Features

• Associated abnormalities

image Leiomyoma: Uterine or vulva leiomyomas
image Hemangioma: Osler-Weber-Rendu disease
image Hamartoma: Cowden disease

Gross Pathologic & Surgical Features

• Leiomyoma: Firm, round, tan, unencapsulated
• GIST: Firm, tan mass, often with central necrosis, mucosal ulceration
• Granular cell: Broad-based, pinkish-tan mass with normal overlying mucosa and rubbery consistency
• Lipoma: Smooth, yellow, encapsulated tumor composed of well-differentiated adipose tissue
• Hemangioma: Blue to red, nodular mass
• Hamartoma: Various elements, including cartilage, bone, adipose and fibrous tissue, and muscle
• Mediastinal foregut cyst

image Thin-walled, nonenhancing contents
image Contents are water density (50%) to calcific

Microscopic Features

• GIST distinguished from leiomyoma by evidence of CD117 (C-kit) activity

CLINICAL ISSUES

Presentation

• Most common signs/symptoms

image Asymptomatic (most common) or dysphagia

– Uncommon: Retrosternal pain, pyrosis, cough, odynophagia, weight loss, and bleeding

Demographics

• Age

image > 40 years old
• Gender

image Leiomyoma: M:F = 2:1
• Epidemiology

image Leiomyomas are much more common than GIST or other intramural esophageal tumors

Natural History & Prognosis

• Complications: Hemorrhage, obstruction, ulceration
• Prognosis: Very good

Treatment

• Asymptomatic: No treatment
• Large, symptomatic lesions

image Enucleation or esophageal resection with gastric interposition

DIAGNOSTIC CHECKLIST

Consider

• Barium esophagram and CT are complementary
• Most intramural masses are benign (unlike gastric tumors)

Image Interpretation Pearls

• Calcifications suggest leiomyoma; almost never occur in other esophageal tumors
image
Axial T2WI MR shows duplication “foregut” cyst image as complex fluid intensity.

image
Axial CECT shows a water density mass image displacing the distal esophagus (duplication or foregut cyst).
image
Single-contrast esophagram shows an intramural mass (leiomyoma) with smooth surface, central ulcer image, and right angle or obtuse angle with wall.

SELECTED REFERENCES

1. Shin, S, et al. Enucleation of esophageal submucosal tumors: a single institution’s experience. Ann Thorac Surg. 2014; 97(2):454–459.

2. Levine, MS. Benign tumors of the esophagus: radiologic evaluation. Semin Thorac Cardiovasc Surg. 2003; 15(1):9–19.

Jiang, G, et al. Thoracoscopic enucleation of esophageal leiomyoma: a retrospective study on 40 cases. Dis Esophagus. 2009; 22(3):279–283.

Nguyen, NT, et al. Minimally invasive surgical enucleation or esophagogastrectomy for benign tumor of the esophagus. Surg Innov. 2008; 15(2):120–125.

Rice, TW. Benign esophageal tumors: esophagoscopy and endoscopic esophageal ultrasound. Semin Thorac Cardiovasc Surg. 2003; 15(1):20–26.

Noh, HM, et al. CT of the esophagus: spectrum of disease with emphasis on esophageal carcinoma. Radiographics. 1995; 15(5):1113–1134.