|Year : 2015 | Volume
| Issue : 3 | Page : 651
Esophageal resection for giant leiomyoma
Vikas Gupta1, Saroj K Sinha2, Kim Vaiphei3, Anupam Lal4
1 Department of Surgery, Postgraduate Institute of Medical Education and Research, Chandigarh, India
2 Department of Gastroenterology, Postgraduate Institute of Medical Education and Research, Chandigarh, India
3 Department of Histopathology, Postgraduate Institute of Medical Education and Research, Chandigarh, India
4 Department of Radiology, Postgraduate Institute of Medical Education and Research, Chandigarh, India
|Date of Web Publication||9-Oct-2015|
Department of Surgery, Postgraduate Institute of Medical Education and Research, Chandigarh - 160 012
Source of Support: None, Conflict of Interest: None
A 55-year-old Indian female presented with chest discomfort at swallowing and occasional dyspnea. Esophagogastroduodenoscopy revealed an extramucosal mass in the upper and mid esophagus. Computerized tomogram visualized a mass 7 × 11 cm in diameter in the middle esophagus with eccentric lumen and tracheal compression. Transthoracic esophagectomy was performed. She made an uneventful recovery. Diagnosis of leiomyoma was established on histopathology and immunohistochemistry staining.
Keywords: Esophagectomy, eesophagus, giant, large, leiomyoma, stromal tumour
|How to cite this article:|
Gupta V, Sinha SK, Vaiphei K, Lal A. Esophageal resection for giant leiomyoma. J Can Res Ther 2015;11:651
| > Introduction|| |
Leiomyoma, though rare, is the most common benign neoplasm of the esophagus. It is usually located in the lower and middle thirds of the esophagus. The lesion most often than not tends to be solitary.  It can occur at any age, but peak incidence is found between 3 rd and 5 th decades of life.  The disease has male preponderance. About half of the patients with leiomyoma are asymptomatic. Smaller lesions are detected incidentally, while the larger ones can cause compressive symptoms. , A lesion larger than 10 cm is termed as giant.  The lesion can be detected on cross-sectional imaging. , Variety of surgical techniques have been described to tackle these lesions. ,,, Successful surgical resection of the giant leiomyoma forms the basis of present report.
| > Case report|| |
A 55-year-old Indian female, had 6 months history of non-progressive dysphagia and occasional dyspnea, which used to get aggravated on exertion. Her physical and systemic examination was normal. A chest radiograph revealed an upper mediastinal mass with a radiolucent shadow in the periphery of the mass. However, the trachea was central in position.
A diagnostic endoscopy showed an extramucosal lesion occupying upper and middle third of the esophagus. A computerized tomogram of the chest revealed a 7 × 7 × 11 cm homogenous solid mass in the posterior mediastinum extending from the thoracic inlet to 2 cm below the carina causing chinking of the trachea and displacing the esophagus laterally [Figure 1]a. Distal esophagus and gastroesophageal junction was normal. A barium contrast esophagogram demonstrated laterally displaced thoracic esophagus to the right suggestive of an intraluminal mass. The proximal esophagus had a dilated lumen [Figure 1]b. An image-guided needle aspiration showed a stromal tumor. Fiberoptic bronchoscopy revealed an extrinsic tracheal compression with normal overlying mucosa.
|Figure 1: (a) Contrast-enhanced computed tomography (CECT) chest axial image at the level of carina showing a homogenous large soft tissue mass displacing the esophagus to right. Note the airway compression by the mass. (b) Barium swallow image shows displaced thoracic esophagus to right with suggestion of a large intraluminal mass. (c) Resected specimen, advential aspect, shows a large lesion occupying the upper and mid part of the esophagus. Note the proximal and distal esophagus is normal. (d) Low power photomicrograph of the mass showing the stretched out overlying esophageal mucosa and the well-defined smooth muscle tumor (hematoxylin and eosin (H and E), ×140)|
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A transthoracic approach (right posterolateral) was used to perform subtotal esophagectomy with gastric advancement and cervical esophagogastric anastomosis. An 8 × 8 × 12 cm mass was occupying the upper and middle thoracic esophagus. The esophagus could be easily separated from the surrounding structures.
Grossly, 8 × 8 × 12 cm tumor could be identified in the upper and middle thoracic esophagus with normal overlying mucosa [Figure 1]c. Cut-section of the tumor was fleshy and homogenous with focal areas of hemorrhage. On microscopic examination of the tumor, there was no cellular atypia or areas of necrosis, no mitotic activity. Few foci showed areas of hyalinization and focal lymphocytic collection. The overlying stratified squamous lining epithelium was seen stretched over the mass. Overall morphology was consistent with that of a giant leiomyoma [Figure 1]d. The immunohistochemical staining was strongly positive for smooth muscle actin (SMA) and S-100, focally positive for C-kit, and negative for CD34 [Figure 2].
|Figure 2: (Clockwise) Photomicrograph of a section stained with C-Kit shows focal cytoplasmic positivity, CD 34 is negative, diffuse cytoplasmic positivity for S-100 and focal intracytoplasmic positivity with smooth muscle actin (SMA) (Papanicolaou (PAP), ×240)|
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She made an uneventful recovery. She remained well for 36 months and subsequently died of an unrelated cause.
| > Discussion|| |
Benign tumors account for fewer than 10%, out of which only 4% are leiomyomas. , The exact incidence of a giant leiomyoma is difficult to determine as there are only anecdotal reports describing these lesions. They are located mainly in the lower and middle thirds of the esophagus. Tracheal compression as was there in the present case, has been reported to be a rare event. , In a recent series, Cheng et al.,  found one out of the eight giant lesions to be located in the upper esophagus. However, in the present case mass effect by an upper thoracic lesion caused tracheal compression.
As the leiomyoma grows in size it may undergo cystic degeneration. Calcification and malignant change of these tumors occur infrequently but have also been reported. ,, These are slow-growing tumors and the size of the lesions may not change for many years. , Only 5% of the leiomyomas may grow more than 10 cm in size. ,, Giant leiomyomas warrant surgical treatment once diagnosed. ,,,,,,,
Contrast studies can demonstrate an extramucosal filling defect with or without luminal obstruction or deviation of the axis of esophagus. Computerized tomogram shows a mass lesion displacing the adjacent structures. Magnetic resonance has also been used to demonstrate the lesion.  On endoscopy, the mucosa is usually normal in appearance or sometimes may show ulceration as a result of necrosis. Coexisting malignancy can also be picked up.  Endosonogram can localize the mass to be arising from the muscular layer. ,,
Leiomyomas is a different entity from gastrointestinal stromal tumor (GIST) of esophagus in contrast to the rest of gastrointestinal tract, probably because of the good prognosis associated with the former. Esophageal leiomyoma and GISTs are difficult to differentiate preoperatively since there are no specific clinical, endoscopic, endoscopic ultrasound, or computed tomography (CT) scan findings specific to them. ,, Both GISTs and leiomyomas are hypoechoic lesions originating from the muscularis propria or muscularis mucosa on endoscopic ultrasound. ,,,, Definitive diagnosis of GIST/leiomyoma can be made by fine-needle aspiration; however, it should not be performed for esophageal submucosal lesions, as it makes subsequent safe enucleation difficult.  Shinagare et al.,  have demonstrated 18-fluorodeoxyglucose positron emission tomography (FDG PET) uptake in GIST, which can help to differentiate the two conditions. A selective approach to biopsy based on tumor size (>5 cm), suspicious radiological appearance, and FDG uptake is warranted. Immunohistochemical staining can conclusively differentiate the two conditions. Diagnosis of GIST can be established by the presence of c-kit mutations and positivity for CD117 and CD34; on the contrary, leiomyomas are CD34 and CD117 negative, and do not have c-kit mutations. More so, leiomyomas are positive for desmin and SMA, in contrast to GISTs which are usually (but not always) negative, as was in the present case.  It is imperative to differentiate the two conditions as the extent and nature of surgery can be different in both the situations. Whereas, GIST requires resection with negative margins, simple enucleation is preferred for leiomyomas. ,,, However, for a larger lesion where organ preservation is not possible, as in the present case, esophageal resection and replacement is suitable in both the situations. ,,,
Enucleation is an accepted treatment for tumors up to 8 cm in size, provided the overlying mucosa remains intact and the myotomy can be reapproximated in an appropriate manner. , However, for lesions more than 8 cm, esophageal resection is an acceptable treatment.  Recently, enucleation of giant leiomyomas has also been reported. , There are anecdotal reports of thoracoscopic ,, or robot  assisted enucleation of giant leiomyomas. Functional achalasia as a result of long myotomy remains a concern. Following enucleation of a large lesion, the muscular defect is usually too large for tension-free suturing. Cheng et al.,  described a pedicled greater omentum flap to circumvent this problem. The use of latissimus dorsi flap has also been described to adequately approximate the myotomy. 
A left posterolateral thoracotomy is preferred as most of the lesions are located in the lower third of the esophagus.  However, as in the present case, a right posterolateral thoracotomy is preferred for tumors located in the upper thirds.  Similar approach had also been used by Karagulle et al. As the tumor enlarges, it increases its vascularity and tend to displace the adjacent structures. So, a careful attention should be paid to hemostasis and adjacent viscera.
Concluding, giant leiomyoma of the esophagus is a rare condition. Tracheal compression is an unusual event. Preoperative differentiation from GIST is difficult. Although, enucleation and buttressing the myotomy with a vascularized tissue has been performed, but esophageal resection and replacement is the appropriate treatment for giant leiomyoma. The diagnosis is confirmed on immunohistochemistry.
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[Figure 1], [Figure 2]