Disclaimer: The information contained within the Grand Rounds Archive is intended for use by doctors and other health care professionals. These documents were prepared by resident physicians for presentation and discussion at a conference held at Baylor College of Medicine in Houston, Texas. No guarantees are made with respect to accuracy or timeliness of this material. This material should not be used as a basis for treatment decisions, and is not a substitute for professional consultation and/or peer-reviewed medical literature. Benign Esophageal Neoplasms Benign esophageal neoplasms are very rare. In a large autopsy study, there were 90 cases out of almost 20,000 autopsies, for a prevalence of 0.5%. Esophageal tumors may be classified as intraluminal, intramural or extramural. Benign tumors of the esophagus are more common in men than in women, and typically present after age 40. Leiomyoma is the most common benign esophageal neoplasm. In the largest review of leiomyomata of the esophagus, the male to female ratio was 1.9 to 1, and age range was 12 years to 80 years, with a mean of 44 years. Over 50% of patients were asymptomatic; these tumors were discovered in autopsy review, or on routine radiographic examination. In symptomatic patients, dysphagia and vague pain were most common, and symptoms had been present for more than 2 years in 60% of patients. The majority of leiomyomata are found in the lower one-third of the esophagus, and 97% are intramural. Leiomyomas have a characteristic appearance on barium swallow: a smooth, crescent-shaped defect covered by smooth mucosa. Esophagoscopy should also be performed in all cases. The characteristic endoscopic appearance is a submucosal bulge without actual stenosis, and the lesion is usually movable through the endoscope. Biopsy through the intact mucosa is not recommended if radiographic and endoscopic exams are consistent with leiomyoma because of subsequent ulceration and inflammation, which complicates eventual surgical removal. The recommended treatment for esophageal leiomyoma is transthoracic enucleation of the tumor. The muscular layer of the esophagus is split, and the tumor enucleated with submucosal dissection. Violation of the mucosa carries the risk of wound contamination with digestive tract flora, as well as possible fistula formation. Esophageal resection with reanastomosis is necessary only in very large tumors, annular tumors, or those densely adherent to larger areas of mucosa. The second most commonly reported benign tumor of the esophagus is the intraluminal polyp. In contrast to leiomyomas, 80% of these occur in the upper esophagus, and are often closely associated with the cricopharyngeus muscle. Esophageal polyps occur in men 75% of the time, and typically occur in the 6th and 7th decade. There have been several pathologic diagnoses for esophageal polyps, including fibroma, fibrolipoma, pedunculated lipoma, and fibroepithelial polyp, but these are now all considered under the name "fibrovascular polyp" as they have essentially the same histologic findings. There are several reports of polyps more than 15 cm in length, and in some cases, polyps have extended down to the gastroesophageal junction. The most common symptom at presentation is dysphagia, but regurgitation of the polyp, regurgitation of recently digested food, and sensation of lump in the throat have all be reported. Weight loss and anorexia are also seen, although odynophagia is uncommon. There are several reports of asphyxia and death due to polyp regurgitation and subsequent airway obstruction. The two most common methods of diagnosis are barium swallow and esophagoscopy, and both are usually necessary. Biopsy is not recommended if the characteristic findings are present on esophagoscopy, because these polyps are quite vascular. Surgical excision of esophageal polyps using an external approach has yielded typically excellent results. Most authors recommend a transcervical approach with a vertical esophagotomy. Squamous papilloma of the esophagus is also reported. Although it is reported as a rare lesion in the Radiology literature, it is seen more commonly in some GI literature. It is seen more frequently in men (3:1), the mean age is 53.8 years, and the age range is broad (14-79). Most are asymptomatic, although they may cause dysphagia. The most frequent location is the posterior wall of the lower third of the esophagus, and the lesions are usually isolated. Endoscopically, the papilloma is a warty, polypoid mass that is firm to touch. The etiology of esophageal papilloma is unknown. It is unclear whether this represents a true neoplasm, or only reactive change. Other reported benign neoplasms of the esophagus include a recent report of a recurrent schwannoma of the upper esophagus that mimicked fibrovascular polyp in both symptoms and barium swallow findings. This lesion was treated with cervical esophagotomy and excision. Granular cell tumor is also reported in the esophagus, although it is more commonly seen in the tongue, skin, and subcutaneous tissue. Hemangioma of the esophagus is also reported. Massive blood loss after biopsy is possible, and should be considered before biopsy of any soft, vascular mass. Finally, extrinsic compression may mimic a benign esophageal neoplasm. Cervical osteophytes, lymphadenopathy, thyromegaly, aortic aneurysms, and left atrial enlargement are among the reported causes of extrinsic esophageal compression. In summary, benign neoplasms of the esophagus are rare lesions, but should be considered in the differential diagnosis of dysphagia. The most common lesion is leiomyoma, which is an intramural tumor. It is usually found in the lower third of the esophagus, and presents with dysphagia or as an incidental finding. Diagnosis is made with barium swallow and esophagoscopy, and treatment is surgical excision with enucleation of the tumor. The second most common benign neoplasm reported is fibrovascular polyp. These are usually in the upper third of the esophagus, and present with dysphagia or regurgitation of the polyp. Diagnosis is made by barium swallow and esophagoscopy, and biopsy and snare removal are not recommended. Treatment is surgical excision using the transcervical approach. Squamous papilloma and granular cell tumors are very rare, and are often incidental findings in asymptomatic patients. Case Presentations A 67-year old white male presents with a 2-year history of intermittent choking, especially while supine. He reported the sensation of "something stuck in his throat that would block his breathing." He reported that he had actually regurgitated a fleshy mass into his mouth, but had to swallow it because of respiratory distress. He was seen at a medical center in another city, where he underwent a barium swallow, esophagoscopy and laryngoscopy, which were all reported as normal. He was advised to seek psychiatric counseling for "anxiety attacks." He was referred to a gastroenterologist at The Methodist Hospital for a second opinion. He underwent a barium swallow, which revealed a smooth, polypoid, mobile lesion in the upper esophagus. He then underwent esophagoscopy, with an anesthesiologist on standby for potential airway complications. At esophagoscopy, the patient had a large mucosa-covered polypoid lesion, consistent with fibrovascular polyp, which arose from the hypopharynx. No biopsy or snare removal was attempted, and the patient was referred to the Otolaryngology service for surgical management. 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