A Large Esophageal Leiomyoma: Thoraco-Laparoscopic Enucleation or Esophagectomy and Reconstruction?

Background

Esophageal leiomyoma is a rare condition, with an estimated incidence rate of 0.4% of all esophageal neoplasms [1]. It is more commonly diagnosed in men than in women, with a male-to-female ratio of approximately 2: 1, and in patients between the ages of 20 and 50 years [2]. These tumors are typically small, rarely more than 5 cm, slow-growing, and often do not cause any symptoms [2]. Treatment depends on the symptoms and size and location of the tumor. Indications for surgery include constant symptoms, tumor size, mucosal ulceration, and histopathological diagnosis [3]. To date, the standard surgical technique is enucleation without damaging the mucosa, which is faster and safer than esophagectomy. Choosing treatment methods depends on the surgeon’s experience, the size and shape of the tumor, and the extent of adhesions between the tumor and the esophageal mucosa [3]. Indications for esophagectomy are given when the tumors are large, have a horseshoe shape or circumference, or the tumor is inextricably adhering to the esophageal mucosa [4]. The access route is generally determined by the location of the lesions and whether they are easily exposed. Currently, most esophageal leiomyomas can be removed by thoracoscopic or laparoscopic procedures, except for extensive adhesions in the pleural cavity, adhesions intolerant of unilateral pulmonary ventilation, huge leiomyomas of the esophagus or difficult tumor stripping, or intraoperative frozen pathology showing malignancy.

Case Report

A 59-year-old man with no significant medical record presented with a chief concern of progressive difficulty of swallowing over the past 12 months. Physical examinations revealed level 2 dysphagia (Saeed classification [5]). The results of endoscopy and endoscopic ultrasound presented an abnormal thickening of the esophagus at the lower third around the entire circumference, approximately 8 cm long, with smooth esophageal mucosa. Biopsies during endoscopic ultrasound with fine needle aspiration 3 months before surgery showed that all lesions were benign. Images of a thoracoabdominal computed tomography scan revealed a thickening of esophageal wall, about 8 cm long, extending to the cardia, with the maximum thickness of 1.5 cm. Moreover, this lesion surrounded the entire circumference of the esophagus, which was partly calcified (Figure 1).

With the possibility of a leiomyoma (smooth muscle benign tumor) of the esophagus, which had not ruled out the possibility of malignant lesions, we planned a thoraco-laparoscopic esophagectomy to remove the section of the esophagus which contained the tumor. Then, we planned to reconstruct the esophagus with bypass using a gastric tube with the junction in the mediastinum. The patient was provided good preoperative care. Examinations through the thoracoscopy found the tumor located at the distal esophagus, with a length of 8 cm, encircling around the perimeter of the lower third, extending to the end of the esophagus, and rising high. Macroscopically, we identified it as a leiomyoma with uniform density and pinkish color (Figure 2).

As predicted, frozen section biopsy gave us a result of a benign smooth muscle tumor. We decided to open 6 cm along the esophagus and enucleate the tumor within, not to damage the esophageal mucosa, and we stitched the muscle layers. In the stage of laparoscopy, we noticed that the tumor extended 2 cm through the cardia, and it was directly related to the leiomyoma in the thorax (the total length of the tumor was about 8 cm, as shown in Figure 3). We continued to perform an incision to the muscle layers along the abdominal esophagus. However, owing to the large size of the tumor surrounding the whole esophagus, as well as its location proximal to the mucosa layer, we encountered many difficulties during the enucleation. There were 2 lesions of mucosa at the anterior of the abdominal part of the esophagus, with maximum diameters about 1 cm and 0.8 cm (Figure 4). After successfully removing the tumor, we decided to stitch these injured mucosae, performed a Dor fundoplication, and placed a feeding jejunostomy tube. The patient’s postoperative state was stable. Follow-ups 1 month and 3 months after surgery showed no appearance of reflux or dysphagia. The esophago-gram 1 week and 1 month after surgery visualized no obstruction or leakage (Figure 5). Postoperative histopathological results gave us concrete evidence of a leiomyoma: elongated cells with eosinophilic cytoplasm and rhomboid nuclei with uniform size (Figure 6).

Discussion

Esophageal leiomyomas are typically small and slow-growing and often do not cause any symptoms. In terms of location, esophageal leiomyomas can occur anywhere along the length masses that are located within the wall of the esophagus. The tumors are usually homogenous and have a similar density to that of the surrounding muscle tissue. In our patient, the tumor was found in the lower third, as in most cases. However, with the large size of about 8 cm, multi-lobes, complete encirclement of the esophagus, and calcification, ours was a remarkably uncommon case.

Today, conventional treatment for esophageal leiomyoma is thoraco-laparoscopic enucleation. Submucosal tunneling endoscopic resection can also be indicated, but only for tumors with a diameter less than 3 cm and that are believed to be benign leiomyomas [9]. Esophagectomy is performed when the tumor is excessively complicated, has a horseshoe shape or circumference, or the tumor inextricably adheres to the esophageal mucosa [4]. However, esophagectomy with reconstruction takes many risks, including reflux, anastomotic stenosis, leakage that leads to mediastinal abscess, infection, and death, compared with enucleation. In our patient, the tumor was evaluated as a large, multi-lobed, completely encircled esophageal leiomyoma growing along the length and having calcifications. The first treatment plan was esophagectomy and reconstruction with an esophagogastric anastomosis, as in the case of Thakut et al [3], since the prognosis of esophageal leiomyoma enucleation was difficult. We decided to perform thoracos-copy first to control the upper margin of the tumor, with the of the esophagus, but the most common site for these tumors is the distal third [6]. The most prevalent size of an esophageal leiomyoma is less than 5 cm in diameter. A study by Yang et al [7] showed that most of the tumors range between 2 and 5 cm in size, more than 97% of them are a single tumor, and only 8% of leiomyomas are calcified. A study by Nguyen et al [8] on 36 cases indicated that the largest leiomyoma was 6 cm. On a computed tomography scan, esophageal leiomyomas typically appear as well-defined round or oval-shaped hope to enucleate even though the tumor was complicated. As expected, we found a position to open and get access to the mucosa and decided to enucleate and preserve the esophagus, similar to the case of Beji et al [10]. However, our case was more complicated because the tumor invaded the entire circumference of the esophagus and extended to the abdominal esophagus. Preservation of the esophagus and avoidance of an esophagectomy and reconstruction helps patients recover quickly and reduces complications and mortality rates.

During the laparoscopy, there were 2 lesions of the mucosa at the anterior of the abdominal part of the esophagus, and these lacerations were controlled by sutures. We used longitudinal sutures to avoid esophageal strictures at the lower-third section and performed Dor fundoplication. The fundoplication has also been used by some authors [11,12], especially in patients who have symptoms of gastroesophageal reflux or in cases in which insufficient smooth muscle remains around the cardia after enucleation. Furthermore, Ben-David et al showed that tumors located near the gastroesophageal junction have the potential to narrow the esophagus or destroy the acid protective barrier. These authors have since performed laparoscopic Nissen fundoplication in a case 4 months after an enucleation [13]. The use of Nissen or Dor fundoplication has been shown by Raue et al to have no difference in quality of life, physiological function, or reflux control [14]. In fact, at follow-up 3 months after surgery, our patient had no symptoms of reflux, leakage, or stricture.

Since the enucleation of this large tumor damaged the muscle layer of the esophagus and caused 2 lacerations in the mucosa, we decided to place a Witzel jejunostomy tube for early feeding to keep the patient well nourished. This jejunostomy was removed after 2 weeks, when the risk of leakage or stricture of the esophagus had passed and the esophagogram expressed an acceptable flow of the contrast agent through the lesion.

Conclusions

Esophageal leiomyomas are rarely observed in tumors of the esophagus. Thoraco-laparoscopic enucleation is the standard in the treatment of esophageal leiomyomas, even with tumors that are larger and occupy the entire circumference or spread to the abdominal section of the esophagus. Esophagectomy is performed in only a very few cases, such as when the tumor is too large or there are multiple esophageal leiomyomas.