Biography
Interests
Osama Helmy1*, Fouad Haddad1 , Soukaina Banani1 , Fatima Zahra EL Rhaoussi1 , Mohamed Tahiri1 , Wafaa Hliwa1 , Ahmed Bellabah1 , Wafaa Badre1 , Najat Id El Haj2 , Sara Hafidi2 , Mohamed Ridai2 ,Ghizlane Lembarki3 , Fatiha Essodegui3 , Sara Moukhlis4 & Mehdi Karkouri4
1Hepatology and Gastroenterology Department, Ibn Rochd UHC, Casablanca, Morocco
2
Thoracic Surgery Department, Ibn Rochd UHC, Casablanca, Morocco
3Central Unit of Radiology, Ibn Rochd UHC, Casablanca, Morocco
4Histopathology Department, Ibn Rochd UHC, Casablanca, Morocco
*Correspondence to: Dr. Osama Helmy, Hepatology and Gastroenterology Department, Ibn Rochd UHC, Casablanca, Morocco.
Copyright © 2023 Dr. Osama Helmy, et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Abstract
Abbreviations
CT: Computed Tomography
ECG: Electrocardiogram
Introduction
Leiomyomas are the most common mesenchymal tumors of the esophagus. They are of benign origin occurring
mostly in the lower two-thirds of the esophagus. Symptoms and signs are similar to other esophageal lesions
such as dysphagia, retrosternal chest pain, or regurgitation, but they may rarely present with bleeding from
esophageal varices which can be caused by compression of blood vessels at the lower esophagus leading to
blockage of blood flow as described in this case report.
Case Report
A 65-year-old female patient presented with recurrent hematemesis, chronic dry cough, and dyspnea on
exertion. No chest pain, hemoptysis, abdominal pain, jaundice, abdominal distension, fever, weight loss, or
anorexia were reported. Otherwise, the patient was healthy and there was no history of respiratory diseases,
including asthma or allergies. She never smoked or drank alcohol. Her drug, family, and social history were
unremarkable.
On examination, the vital signs were within normal limits. An abdominal examination showed no tenderness, mass, or ascites. A chest examination showed bilateral vesicular breathing sounds with no crepitations or wheezes. Heart sounds were normal with no murmurs.
Upper endoscopy showed esophageal varices grade 2 (with no red wales), hiatal hernia, and lower esophageal diverticulum (figure 1). However, basic laboratory investigations showed no signs of chronic liver disease or portal hypertension as there was neither anemia nor thrombopenia. In addition, there were no liver abnormalities, no portal vein dilatation, no collateral veins, nor splenomegaly in the abdominal ultrasonography.
Contrast-enhanced computed tomography (CT) revealed the presence of a large soft-tissue mass in the posterior inferior mediastinum, arising from the esophagogastric junction (figure 2). Stippled intralesional calcifications were associated. The descending aorta and esophagus were displaced posteriorly. The esophagus was not dilated and contained small parietal varices. There were no signs of blood vessel invasion nor signs of pleural and pericardial effusion. The patient was discharged and asked to return a week later to present her case to the surgery department but she didn’t attend.
Six months later, she presented with the same symptoms and upper endoscopy showed the same findings. A computed tomography scan with gastrografin swallow was performed, showing that there was no progression of the mass nor direct or distant metastasis or invasion of blood vessels. Accordingly, malignant degeneration of the mass was not highly suspected.
Bronchoscopy was performed to eliminate any lung lesions because of the chronic cough. ECG and Echocardiography showed no anomalies.
A diagnostic thoracoscopy was done and revealed a fixed large paraesophageal mediastinal mass.
The biopsy of the lesion revealed fusocellular growth with no signs of malignancy or active mitotic activity. These findings were consistent with esophageal leiomyoma (figure 3).
Discussion
Leiomyomas are the most common benign mesenchymal tumors of the esophagus [1-4]. These tumors
originate from smooth muscle cells. It usually occurs between the ages of 20 to 50 years. The lower twothirds of the esophagus is the most typical location of these tumors
Leiomyomas of the esophagus rarely cause symptoms when they are smaller than 5 cm in diameter. Moreover, they rarely grow to be larger than 10 cm, and in this situation they are called giant leiomyoma of the esophagus. These may occur as single or multiple tumors [5,6].
When these tumors grow larger, they become more symptomatic in patients. The most common symptoms are dysphagia, chest pain, vague retrosternal discomfort, heartburn, and occasionally regurgitation. They can rarely cause gastrointestinal bleeding when the tumor erodes through the mucosa. Moreover, it has never been reported that it might present by bleeding from esophageal varices because it is a benign tumor which does not invade blood vessels.
In our case, the esophageal leiomyoma caused probably a blockage of blood flow by compression of blood vessels leading to formation of bleeding esophageal varices which were detected by upper endoscopy but no tumors were visualized. A malignant nature of this mass was less suspected because the patient had a good general status and there was no mass progression within six months in the CT scans or invasion of blood vessels.
Histology and immunohistochemistry studies are important for definitive diagnosis [7]. Histologically, leiomyomas comprise bundles of interlacing smooth-muscle cells well-demarcated by adjacent tissue or by a definitive connective tissue capsule [8]. Leimyoma is distinguished from esophageal gastrointestinal stromal tumors by negative CD117 and DOG1 activity on immunohistochemistry [9,10].
Enucleation via thoracotomy or thoracoscopy is the preferred surgical method for the treatment of esophageal leiomyoma, depending on the location and size of the tumor. In contrast, surgery is favored for giant esophageal leiomyoma by esophageal resection and reconstruction [11].
Since malignant degeneration is exceedingly rare [12], surgical treatment of small asymptomatic leiomyomas is controversial, as some authors recommend resection to rule out malignancy, while others advocate for observation [11].
Conclusion
Esophageal leiomyoma is rarely revealed by upper gastrointestinal bleeding from esophageal varices. This
presentation can be associated with respiratory symptoms. It can be misdiagnosed due to atypical symptoms
or imaging features. Endoscopic and radiologic tests are essential to differentiate esophageal leiomyomas
from other esophageal and mediastinal lesions.
Conflicts of Interest
No conflicts of interest are to be declared.
Bibliography
Hi!
We're here to answer your questions!
Send us a message via Whatsapp, and we'll reply the moment we're available!