Benign tumors of the esophagus are rare cases encountered by a variety of specialists including gastroenterologists, general surgeons, and thoracic surgeons. The clinical presentation of these lesions may vary from asymptomatic, incidentally discovered tumors (most common) to large lesions resulting in important dysphagia or airway compromise. The development of minimally invasive approaches to esophageal pathology over the last decade has expanded the diagnostic and treatment options for these lesions.

Benign tumors represent less than 1% of all esophageal tumors, and less than 10% of all surgically resected esophageal lesions.^1,2 They may be classified based on either histology or location within the esophageal wall (mucosal, submucosal, or extraluminal) (Tables 29-1 and 29-2). The most common lesions include leiomyomas, esophageal cysts, fibrovascular polyps, and granular cell tumors. The development of endoscopic ultrasound (EUS) has improved the preoperative ­diagnosis of these lesions and is a helpful adjunct for clinical decision-making. Many of these lesions have a characteristic radiological appearance that eliminates the need for biopsy in many cases. Fine-needle aspiration (FNA) is often not required for diagnosis and may not be diagnostic in all lesions (e.g., in differentiating leiomyoma from leiomyosarcoma). Forceps biopsy is appropriate for mucosal lesions, but usually is unhelpful for submucosal or deeper lesions.

Benign tumors are generally asymptomatic and are discovered incidentally on imaging studies. Dysphagia is the most common presenting symptom and is more common with intraluminal tumors. Other presenting symptoms vary depending on the lesion, as described below, but may include vomiting, weight loss, gastrointestinal (GI) bleeding, substernal discomfort, cough, or regurgitation of pedunculated cervical lesions. Examples of intraluminal tumors include squamous cell papillomas, fibrovascular polyps, inflammatory pseudopolyps, and fibroneuroid tumors. Fibrovascular polyps are the most common, arise from the cervical esophagus, and generally are seen in men in the sixth or seventh decade of life. Squamous cell papillomas are small, sessile lesions found in the distal esophagus, most commonly seen in elderly patients.

Submucosal and intramural lesions are more commonly asymptomatic until they have grown to >5 cm in transverse diameter. Large tumors can cause obstructive symptoms such as dysphagia and emesis. Examples of these tumors include leiomyomas, granular cell tumors, lipomas, hamartomas, and neurofibromas. These tumors are difficult to differentiate on endoscopy, and EUS is helpful for making a diagnosis. Mucosal biopsy is not helpful for these lesions, and should be avoided in such cases because mucosal disruption may compromise subsequent enucleation of the lesions. FNA may be used in situations in which a diagnosis cannot be made by EUS alone, and does not threaten mucosal integrity.

All patients undergoing resection for a benign tumor should undergo a standard preoperative work-up and risk stratification as described in Chapter 4. The majority of benign tumors are asymptomatic, and other potential esophageal pathology should be evaluated carefully with a history, physical examination, and diagnostic studies to avoid an inappropriate operation. Evaluating for other esophageal pathology such as gastroesophageal acid reflux disease (GERD), achalasia, hiatal hernia, or a diverticulum also may help with assessing the need for additional procedures during resection of a benign tumor.

Diagnostic imaging for individual lesions is discussed in detail below, but often will involve a combination of barium swallow, endoscopy, and endoscopic ultrasound. Barium studies may help identify small lesions, especially those that are covered with normal mucosa and may easily be missed on endoscopy. Barium studies also will reveal other pathology including hiatal hernia, reflux, and diverticula that may need to be addressed at the time of surgery and may prompt additional preoperative testing. Endoscopy provides valuable information regarding the location of the tumor, whether there are multiple lesions, the continuity of the mucosa overlying the lesion, and other potential pathology. Endoscopic ultrasonography is invaluable in characterizing the size, layer of origin, and internal characteristics of lesions. EUS-directed FNA is frequently feasible as an aid to diagnosis. Intraoperative endoscopy also may be of assistance as described below.

Fibrovascular Polyp

Clinical Presentation and Diagnosis

Fibrovascular polyps commonly cause obstructive symptoms such as dysphagia, regurgitation, vomiting, and weight loss. These tumors originate near the thoracic inlet, adjacent to the cricopharyngeus muscle at C6, and grow on a pedunculated stalk. Symptoms may range from episodic regurgitation of the mass to laryngeal impaction and asphyxiation.^3,4 Large tumors are prone to ulceration at the tip and may be a source of GI bleeding.

On barium esophagram, these characteristically appear as smooth, lobulated, elongated filling defects starting at the level of the cervical esophagus (Fig. 29-1A,B). Computed tomography (CT) or magnetic resonance imaging (MRI) will show a dilated esophagus with a homogeneous, intraluminal soft tissue mass without invasion of surrounding structures. These studies can also identify the level of origin of the stalk. Endoscopy may be useful for identifying the site of origin and size of the lesion (Fig. 29-1C). However, small lesions may be missed because they are typically lined with normal-appearing mucosa. EUS is useful for identifying feeding vessels and predicting risk of significant bleeding during excision.

Management

Fibrovascular polyps are benign and may be treated with endoscopic excision; those with thin pedicles may be endoscopically ligated or cauterized.³ Factors that preclude safe endoscopic removal include vascular stalks that need to be ligated or cauterized, or tumors too large to be delivered through the upper esophageal sphincter. These lesions may require cervical esophagotomy for excision.^4,5 Lesions with features concerning for malignancy or those too large to be removed through a cervical esophagotomy may require a formal esophagectomy (see Chapters 15–22). Resection is recommended for all large fibrovascular polyps because of the risk of regurgitation and asphyxiation.

For fibrovascular polyps that are treated surgically, the location of the tumor stalk or pedicle should be evaluated preoperatively as described above. It is helpful to have endoscopy available for intraoperative localization. Consideration likewise should be given to performing a gastrotomy if necessary to facilitate removal of large lesions that extend into the stomach. The patient should be positioned and draped appropriately with these considerations in mind. The esophagus is approached through a longitudinal neck incision anterior to the sternocleidomastoid muscle on the side opposite to the tumor. Fibrovascular polyps generally originate just inferior to the cricopharyngeus muscle. A longitudinal esophagotomy is made at the level of the stalk origin. The incision must be long enough to deliver the tumor through the incision while the stalk is intact. The incision can then be extended distally to expose the entire stalk, allowing for submucosal resection of the tumor with the stalk (Fig. 29-2). The base of the stalk is then ligated, taking care to control any vessels supplying the tumor through the stalk. Complete excision of the pedicle is necessary to prevent recurrence. The esophagus is closed in two layers. Drains are not routinely placed.