General Thoracic Surgery (General Thoracic Surgery (Shields)) [2 VOLUME SET]

Editors: Shields, Thomas W.; LoCicero, Joseph; Ponn, Ronald B.; Rusch, Valerie W.

Title: General Thoracic Surgery, 6th Edition

Copyright 2005 Lippincott Williams & Wilkins

> Table of Contents > Volume II > The Esophagus > Section XXIII - Benign Esophageal Disease > Chapter 149 - Benign Tumors, Cysts, and Duplications of the Esophagus

Chapter 149

Benign Tumors, Cysts, and Duplications of the Esophagus

Ganga Prabhakar

Gordon F. Murray

BENIGN TUMORS

Although rare, benign tumors of the esophagus are of concern because obstruction and subsequent aspiration with resultant pulmonary complications may lead to the death of the patient. Early recognition with simple excision carries a low mortality and usually is curative. The exact incidence of these rare tumors is difficult to ascertain because many of these tumors may go undetected. Examining 11,000 patients complaining of dysphagia, Moersch and Harrington (1944) found only 15 cases, an incidence of less than 0.2%. Whereas current diagnostic techniques suggest an apparently higher rate of occurrence, Plachta (1962), performing nearly 20,000 autopsies over 50 years, found only 90 benign tumors of the esophagus.

These tumors generally are classified by histologic type, as described by Nemir and associates (1976). Definition of benign tumors in terms of their location within the esophagus (i.e., intraluminal, submucosal, or intramural), however, has particular value relating to presentation, diagnosis, and management (Table 149-1). Leiomyoma is the most common benign tumor of the esophagus and is almost always intramural. The second most common lesion is the intraluminal esophageal polyp. Hemangioma and granular cell myoblastomas are less common and most often are submucosal.

The term gastrointestinal stromal tumor (GIST) is gaining popularity as a histogenetically neutral term referring to mesenchymal tumors that could be verified as neither neurogenic nor of smooth muscle cell origin. Miettinen and colleagues (1999) explain that true leiomyomas that predominate in the esophagus have elongated spindle cells with abundant eosinophilic cytoplasm. Immunohistochemically, they are usually positive for desmin and actin but negative for CD34 and CD117 (c-kit), typically expressed by GIST. This differentiation is important because the GISTs have a higher risk of malignant behavior. This was further confirmed by Hasegawa and co-workers (2002).

Clinical Features

The location of the tumor within the esophagus is most responsible for the specific symptoms that are present. Intraluminal tumors obstruct the esophageal lumen to varying degrees. Patients often present with dysphagia, vomiting, and weight loss. Bernatz and associates (1958) noted that many patients also have cough, substernal distress, aspiration pneumonitis, or gastrointestinal bleeding. The tumor can sometimes be regurgitated into or even out of the patient's mouth. Rarely, fatal aspiration of the pedunculated tumor has occurred.

Submucosal and intramural tumors frequently are asymptomatic, discovered incidentally at the time of an unrelated evaluation or autopsy. Submucosal lesions may, however, cause obstruction with associated dysphagia. Bleeding is uncommon, but hemangiomas may hemorrhage and rarely this may be fatal. More common symptoms of intramural tumors include dysphagia, vague chest pain, and pyrosis. Weight loss is an inconsistent finding. Massicot and colleagues (1997) reported the interesting association of an intramural tumor and hypertrophic osteoarthropathy. Rapid regression of osteoarthropathic symptoms followed successful excision of a localized esophageal leiomyoma.

Diagnosis

Diagnostic evaluation of symptomatic patients should include appropriate radiographic and endoscopic studies. Most lesions may only be identifiable by barium esophagography, although standard chest radiography on occasion

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Intraluminal tumors may be revealed by movement of the mass seen during a barium swallow. The normal peristaltic wave may be disrupted at a level just above the tumor pedicle attachment site. Such intraluminal tumors may appear to be ingested foreign bodies.

Schatzki and Hawes (1950) described the image of an intramural tumor as a crescentic filling defect seen in contrast to the natural contour of the esophageal lumen. The junction of the lesion with the normal esophageal wall has a characteristically sharp angle; mucosal folds over the tumor may be obscured by the barium, but they usually appear on the opposite wall. In contrast to intraluminal and intramural tumors, submucosal lesions are rarely detected by such radiographic evaluation.

Computed tomography with contrast medium infusion, reported by Ikezoe and associates (1989), and magnetic resonance (MR) imaging also should be used to ascertain tumor identity. These newer procedures have largely replaced the use of such specialized studies as angiography, described by Ben-Menachem and colleagues (1977), and pneumomediastinography, reported by Perasalo and Laustela (1955), for esophageal lesions. Only the most difficult diagnoses justify the use of these latter studies (i.e., to rule out a vascular malformation or aneurysm).

Endoscopic ultrasonography was advocated by Tio and co-workers (1990) for diagnosing these esophageal tumors. This technique was superior to other imaging methods in the detection, staging, and follow-up of tumors because of clear imaging of the intramural abnormality and adjacent lymph nodes. Endoscopic ultrasound can help in determining the best surgical option. The decision as to whether an endoscopic, a video-assisted thoracoscopic, or a combined approach should be elected is made easier with ultrasound findings.

All patients should be evaluated by esophagoscopic examination; often, repeated examination is required. Smaller tumors may be missed on the first examination, particularly the intraluminal tumors. Appropriate maneuvering of the endoscope is critical for visualization of an intraluminal tumor, its pedicle, and attachments. Submucosal tumors are identified readily by a change in the mucosal contour and color over the lesion. Intramural tumors generally appear as a bulge into the lumen that is mobile. Luminal narrowing occurs with little stenosis (unobstructed passage of the instrument), and the overlying mucosa is smooth and not disrupted. Obtaining tissue for biopsy from intraluminal and submucosal lesions usually is indicated and straightforward. Acquiring samples through the intact mucosa of an intramural tumor is contraindicated, however; it is difficult to harvest an adequate amount of tissue for proper analysis, and the procedure violates the mucosa, which can complicate subsequent treatment.

Benign tumors of the esophagus must be distinguished from a variety of conditions, which include esophageal carcinoma, vascular rings and aneurysms, tumors and cysts of the mediastinal visceral compartment, and other diseases intrinsic to the esophagus. Notably, Idenburg and colleagues (1991) described a patient with symptoms mimicking achalasia who had a large esophageal leiomyoma; three other similar cases have been reported.

Specific Benign Tumors

Leiomyoma

Seremetis and colleagues (1976) collected 838 documented examples of leiomyomas located in the esophagus. The true incidence is difficult to determine. Shields (1959) found 11 such tumors in 700 autopsies; none was found in 4,000 autopsies performed by Daniel and Williams (1950). This variability in incidence may reflect the depth of analysis used to seek these tumors.

Hatch and co-workers (2000), in an exhaustive study of all stromal tumors of the esophagus, compiled 1,679 leiomyomas from a period spanning 1875 to 1996. The peak age of occurrence was between 30 and 59.

Leiomyomas, when clinically manifest, most often are located in the middle and lower one-third of the body of the thoracic esophagus, and only a few occur in the upper one-third. The incidence is reported to be approximately 40%, 50%, and 10%, respectively. Only rarely are tumors identified in the cervical portion of the esophagus. Lewis and Maxfield (1954) emphasized that 99% of clinically evident tumors are intramural lesions, and only the remaining few are intraluminal.

In the previously cited review of Seremetis and colleagues (1976), 2.4% of leiomyomas presented in multiple sites. A report of Taylor and associates (1995) defines the clinical, radiologic, and pathologic findings in multiple

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A small but significant incidence of diffuse leiomyomatosis was described by Linder and Vogt-Moykopf (1970) and reviewed by Kabuto and colleagues (1980). Lonsdale and associates (1992) reported a rare combination of familial esophageal leiomyomatosis and an Alport-like nephropathy. Inherited diffuse esophageal leiomyomatosis associated with X-linked Alport's syndrome, a hereditary disease of type IV collagen, was confirmed by Heidet and colleagues (1997). Additional DNA evidence and immunohistochemical analysis by Ueki and co-workers (1998) supports the latter observation and provides further understanding of the pathogenesis and etiology of esophageal leiomyomatosis.

Leiomyomas occur twice as often in men as in women and may occur at any age. As noted by Bourgue and associates (1989), leiomyomas of the esophagus are extremely rare in children, representing approximately 2.6% of all documented cases of leiomyoma.

Intramural lesions are round or ovoid, of variable size, with sharp demarcations. They usually are smooth, although occasionally lobulated, and they sometimes grow circumferentially, which may partially obstruct the lumen. On gross inspection, the lesion is firm and usually nodular on cut section. Histologic examination demonstrates smooth muscle cells arranged in strands and whorls, with a tendency to palisade. Other findings may include calcified areas and hydropic, fatty, and hyaline degenerative changes. The occurrence of carcinoma in the surface epithelium over a benign intramural tumor is considered to be extremely rare, but Kuwano and co-workers (1995) reported three such patients.

Polyps

Polyps are rare lesions that usually arise singly at or near the cricoid cartilage in the cervical esophagus (Fig. 149-2). They manifest intraluminally, are long and cylindric, and may be based on a long pedicle. The polyps sometimes are so large as to cause significant esophageal dilatation. Histologically, the lesion displays heterogeneous connective tissue components underlying a mucosal layer that at times may be

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Other histologic varieties of polyps have been reported rarely. Pure lipoma, lymphangioma, and eosinophilic granuloma have been identified in the esophagus. The latter lesion should not be confused with eosinophilic granuloma of bone. As Leand and associates (1968) discussed, the esophageal lesion is regarded as an inflammatory eosinophilic polyp.

Lipoma

Lipomas usually occur as intraluminal polyps and can become quite large before diagnosis. They also have been reported as submucosal lesions by Ochsner and Ochsner (1957) and Zschiedrich and Neuhaus (1990), and as intramural tumors by Tolis and Shields (1967). They usually occur in the upper esophagus, and because of their large size and origin on a stalk, peristalsis may aid in their development. Symptoms include a mass in the throat and regurgitation of the polyp into the mouth, with its disappearance on swallowing. Fatal asphyxia results from laryngeal airway obstruction. This morbid event has been recorded by Allen and Talbot (1967) and Cochet and colleagues (1980).

Hemangioma

Hemangiomas, benign tumors of vascular origin first described in 1926, account for fewer than 3% of all nonmalignant esophageal neoplasms. The lesions typically are found in the submucosa; half of them occur in the upper segment, and the remainder occur almost equally in the middle and lower esophagus. They are variable in size and seldom are polypoidal. The endoscopic appearance may be that of a dark purple to red, submucosal nodule. Gilbert and associates (1990) described a wide range of histologic findings, from simple capillary to the more often encountered cavernous hemangioma. Konstantakos and associates (1995) detailed the successful resection of an arteriovenous malformation type of hemangioma disguised as esophageal leiomyoma.

Gastrointestinal hemangiomas occur with the lowest frequency in the esophagus. As reported by Plachta (1962), many of these benign esophageal lesions are asymptomatic. More than one-half are not discovered until postmortem examination. Symptoms, when they occur, usually include dysphagia and retrosternal discomfort. Hematemesis is rare, and endoscopic biopsy apparently does not present a hazard. Notwithstanding, Taylor and associates (1996) have described a patient who experienced sudden massive hematemesis and required rapid clinical intervention.

Management of these lesions does not differ from that of other benign esophageal lesions. Araki and colleagues (1999) reviewed recent options in the management of these lesions. Advances in endoscopic instruments and thoracoscopic techniques have led to complete endoscopic resection or combined thoracoscopic and endoscopic resection. Endoscopic injection therapy can be a useful alternative.

Granular Cell Tumor

A granular cell tumor was first described by Abrikossoff (1926) when he described histologically benign tumors in the tongue. Once regarded as arising only in skeletal muscle, these tumors were formerly known as granular cell myoblastomas. Since then, lesions have been found in many other locations, including skin, breast, respiratory tract, and gastrointestinal tract. Of the latter, one-third of the tumors occur in the esophagus, usually in the distal portion. Both immunohistochemical and ultrastructural data support neural (Schwann cell) differentiation. Tumors are histologically characterized by nests of cells with pyknotic nuclei, abundant granular cytoplasm, absence of mitotic figures, and strong positive reaction for S-100 protein.

Granular cell tumors are uncommon, with about 200 cases being reported in literature, with a predilection for women and individuals of African descent. Most tumors are asymptomatic, small, and found incidentally at upper endoscopy or autopsy. Brandimarte and co-workers (2000) reaffirm that these tumors are usually solitary, although in about 11% of patients two or more may be identified. The typical endoscopic appearance is that of an isolated submucosal nodule. Flexible esophagoscopy and biopsy are the mainstay in diagnosis and management.

Determination of the malignant potential of granular cell tumors and the requirement for resection remain unresolved issues. Fanburg and colleagues (1996) have proposed histologic criteria for prospectively diagnosing malignancy in granular cell tumors, including cell neurosis, cell spindling, increased nuclear size, large nucleoli, mitotic activity, and nuclear pleomorphism. Tumors that demonstrate these criteria were classified as malignant; the majority of these tumors metastasized and resulted in patient death. Goldblum and co-workers (1996) concluded that observation of granular cell tumors is indicated unless the patient is symptomatic or the tumor is larger than 1 cm or has atypical endoscopic ultrasonographic (Fig. 149-3) or histologic (Fig. 149-4) features. Szumilo and colleagues (2002) stress that, although rare, granulosa cell tumors and cancers can coexist; close follow-up in patients with granulosa tumor is suggested.

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Other Tumors

Neurofibromas and neurinomas rarely are encountered in the esophagus. Somewhat more common in patients with generalized neurofibromatosis, they generally are found as submucosal lesions. Definitive diagnosis, as reported by Saitoh and associates (1985), requires light and electron microscopic evaluation and immunohistochemical analysis with S-100 protein antibody. Lee and Williamson (1997) emphasized that unlike the granular cell neural tumors described earlier, this smaller group of neural tumors is often large, symptomatic, and requires resection.

Esophageal papillomas are rare, benign, sessile, lobulated lesions of varying size, sometimes pedunculated, and occasionally multiple. Mosca and co-workers (2001) report a prevalence of 0.01% to 0.43%, with fewer than 200 cases being reported in the literature. Histologically, Fernandez-Rodriguez and associates (1986) describe a squamous cell coverage over fingerlike extensions of relatively vascular connective tissue. Winkler and colleagues (1985) and Politoske (1992) suggested that infection with human papillomavirus may be responsible for their occurrence in some patients. Human papillomaviruses are double-stranded DNA viruses that replicate in the nuclei of squamous epithelial cells. The most common human papillomavirus types, 6 and 11, are associated with benign esophageal papilloma. However, Poljak and co-workers (1995) caution that the etiology and pathogenesis of esophageal squamous cell papillomas remain controversial. Their results show that human papillomavirus DNA is not frequently detectable in esophageal papillomas, even when highly sensitive methods such as polymerase chain reaction are used, and that other pathogenetic mechanisms may be more important.

Isolated cases of other tumor types have been reported. An aberrant thyroid nodule was described by Postlethwait and Detmer (1975). Kline and associates (1990) described a noninfiltrating mediastinal angiolipoma associated with multiple small ulcers of the distal esophagus. Perez and coauthors (1999) described a giant esophageal fibrolipoma originating just below the cricopharyngeus and reaching the cardia of the stomach, but which was visible in the oral cavity during coughing. Sialadenoma papilliferum is an extremely rare benign tumor reported by Su and associates (1998). Smith and colleagues (1976) documented the unusual occurrence of an intramural hamartoma mimicking a peptic esophageal stricture in a child. The remarkable embryologic feature of this entity is the presence of annular cartilage at the site of the esophageal stricture.

Treatment

The accepted treatment for all benign esophageal tumors is surgical removal. The surgical approach is determined by the location of the lesion and the extent to which normal esophagus is involved. Intraluminal tumors, especially when small and pedunculated, may be amenable to endoscopic removal, as described by Benedetti and co-workers (1990). Eberlein and colleagues (1992) warn that endoscopic removal should only be attempted in selected patients with tumors with a narrow stalk. Large lesions often have high cervical attachments that are quite vascular. Removal

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When the size or location of the lesion precludes this approach, surgical excision, through an axial esophagotomy and direct control of feeding vessels, is readily accomplished. On freeing the esophagus from the surrounding tissue, a longitudinal incision is made through the esophageal wall, opening the lumen opposite the tumor pedicle. The pedicle is ligated and divided, removing the tumor through the esophageal incision. The mucosa is closed with continuous absorbable sutures, and the muscularis is closed with interrupted nonabsorbable sutures. These tumors may be accessible through a cervical approach, which has less associated morbidity and thus should be done whenever feasible. Esophageal resection is rarely indicated. An exception would be diffuse esophageal leiomyomatoses, which frequently require esophagectomy. Kumar and associates (1997) reported esophageal leiomyomatoses involving the trachea. The tumor was resected by total esophagectomy and partial resection of the trachea and the left main-stem bronchus.

A frozen section should be made in every case; Marcial-Rojas and Suan (1959) have reported instances of finding squamous carcinoma adjacent to these benign lesions. The surgeon must also be aware of a rare carcinosarcoma. The distinguishing pathologic feature of sarcomatous tumors is their frequently pedunculated gross configuration (see Chapter 153). They may resemble benign esophageal polyps and tumors, and the surgeon must be alert to the malignant potential of the polypoid esophageal lesion.

Intramural and submucosal lesions usually require a thoracotomy for exposure. An incision of the mediastinal pleura is made, with isolation of the esophagus around the site of the tumor. The muscularis overlying the lesion is incised longitudinally, and the mass is enucleated through blunt and sharp dissection, usually without injuring the mucosa. Openings into the lumen, when they occur, can be repaired with continuous absorbable sutures. The incision in the muscularis is then closed with interrupted nonabsorbable sutures. Drainage of the pleural cavity and closure of the thoracotomy are achieved in a standard manner. Notably, a cervical approach has been advocated for leiomyomas in the upper thoracic esophagus (Fig. 149-5) by Schorlemmer and associates (1983).

Enucleation of benign intramural tumors has been carried out during video-assisted thoracoscopic surgery. Bardini and associates (1992) reported successful removal of esophageal leiomyoma in three patients, and Rice and Kerby (1993) demonstrated the technique of this new approach. Numerous international reports and the review of Canvasser and Naunheim (1996) suggest that video-assisted thoracic (VATS) surgical techniques allow for less pain and dysfunction and can result in shortened hospital stays. Thoracoscopic enucleation of an esophageal leiomyoma with novel balloon dilator assistance was described by Mafune and Tanaka (1997). Successful video-thoracoscopic

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Again, esophageal excision is rarely indicated and should be avoided whenever possible. Mortality with enucleation is close to zero, whereas mortality can be greater than 10% after esophageal resection. Even though mortality is low with enucleation, the morbidity associated with thoracotomy should be respected; small, asymptomatic tumors that are positively identified as benign may not need treatment.

In the experience of Tio and colleagues (1990), endoscopic ultrasonography is helpful in planning the strategy of treatment. Intramural tumors with a diameter of less than 4 cm without evidence of bleeding, obstruction, or malignancy at esophagoscopy and on endoscopic ultrasonography can be followed at intervals of 3 months. Some authors express concern about the differentiation of leiomyoma from leiomyosarcoma. However, Yamada and colleagues (1992) have shown that there are five endoscopic ultrasonographic features characteristic of malignant myogenetic tumors: tumor diameter of 3.0 cm or more, nodular shape, ulceration depth of 5 mm or more, a heterogenous internal echo, and the presence of an anechoic area. Asymptomatic myogenic tumors of the esophagus without these features need not be excised. If symptoms develop later or if tumor growth indicates an increased risk, the tumor may then be surgically removed.

Intraluminal endoscopic excision of submucosal and intramural esophageal lesions is a challenging frontier. Sclerotherapy as a viable endoscopic option was reported by Nagata-Narumiya and coauthors (2000) in one patient for the treatment of hemangioma, with no recurrence at 6 months. Hyun and associates (1997) reported successful endoscopic removal of 62 submucosal tumors of the esophagus, 30% of which were smaller than 1 cm, 30% between 1 and 2 cm, and 22% larger than 4 cm; the largest was 7 cm in length. The technique of endoscopic incisional enucleation consisted of complete stripping of the overlying tissue followed by tumor enucleation using an electrocautery snare and a coagulation electrode. The two most morbid endoscopic complications, perforation and severe hemorrhage, did not occur in this series of patients. Dorais and Marcon (1997) caution that these good results must be confirmed elsewhere before the technique can become widely used. Kusano and colleagues (1998) reported a patient requiring emergency subtotal esophagectomy for uncontrolled hemorrhage following an endoscopic resection of an esophageal polyp. Araki and coauthors (1999), in a literature review of treatment options for esophageal hemangiomas, suggest that the first choice of treatment should be endoscopic resection for mucosal and submucosal lesions. However, if this choice is not safe, then sclerotherapy or surgical resection should be considered.

Awareness of the progressive clinical course and obstructive liability of benign esophageal tumors should lead to early surgical intervention. The prognosis after removal is uniformly excellent.

CYSTS AND DUPLICATIONS

Cysts and duplications of the esophagus are uncommon lesions. Most are believed to be congenital lesions and are most commonly found in an intramural location (Table 149-2).

Congenital Cysts

A congenital esophageal cyst is a rare finding. Arbona and colleagues (1984) cited a review of 49,196 autopsies to identify esophageal tumors that demonstrated only 6 esophageal cysts, an incidence of 1 in 8,200. Most esophageal duplication cysts occur in children, but 25% to 30% are discovered incidentally in adults. They occur twice as often in males than in females. Nevertheless, Schmidt and associates (1961) point out that esophageal cysts are the second most common benign tumor of the esophagus, leiomyoma being by far the most common. Congenital esophageal cysts are characterized by their embryogenesis, histologic findings, and location. Congenital esophageal cysts are to be distinguished from mediastinal enterogenous (enteric) cysts and mediastinal duplication (neurenteric) cysts (see Section XXX).

Esophageal Duplication Cysts

Congenital esophageal cysts are classified as duplications when they fulfill the following criteria: (a) the cyst is within the esophageal wall, (b) it is covered by two muscle layers, and (c) it contains squamous epithelium or a lining compatible with that found in the embryonic esophagus (i.e., columnar, cuboid, pseudostratified, or ciliated).

Kiwan and associates (1973) suggested that duplication cysts arise from developmental malformation of the foregut. As the embryonic esophagus elongates, the lining epithelium proliferates and eventually obliterates the lumen. Later, the epithelium produces secretions that form vacuoles. These vacuoles coalesce to form the esophageal lumen. An isolated vacuole may persist, however, giving rise to an intramural esophageal cyst, a true duplication. Because of elongation of the esophagus and dextrorotation of the stomach, duplication cysts frequently are found in the inferior portion of the esophagus and on its right side. Multiple duplication cysts have been described, and Ruffin and Hansen (1989)

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Grossly, duplication cysts are small, rounded structures located intramurally within the esophageal wall. A clear mucoid or brown serous intracystic substance may be found. Duplication cysts occasionally are tubular, sometimes communicating with the esophagus or hypopharynx. Of note, Craig and colleagues (1998) reported a pedunculated intraluminal foregut reduplication cyst of the proximal esophagus. The authors attribute the absence of muscle coverage of the cuboidal/columnar lined cyst to its unique intraluminal development.

Symptoms

Symptoms relating to a duplication depend on its size. In the infant or child, the cyst may readily compress the adjacent airway or obstruct the esophageal lumen. Adults are often asymptomatic, with the cysts discovered through unrelated diagnostic evaluation. Patients with symptoms generally present with respiratory distress, dysphagia, and pain. In addition to reported cases of perforation, bleeding, and infection, Tapia and White (1986) reported squamous cell carcinoma arising in a duplication cyst of the esophagus. Lee and associates (1998) described a single case of adenocarcinoma arising in an esophageal cyst with diffuse soft tissue metastases.

Diagnosis

Diagnosis is a challenge, and Bondestam and associates (1990) point out that even extensive imaging does not always yield definitive identification. The chest radiograph may suggest a mediastinal mass lesion. Contrast fluoroscopic studies of the esophagus often reveal a round filling defect covered by apparently normal mucosa (Fig. 149-6). Endoscopic examination is not diagnostic, but it does confirm the absence of ulceration or malignancy. However, none of these studies allows differentiation between a cyst and a benign intramural tumor. Computed tomography (CT) may offer further evidence of the cystic nature of the lesion, and Page and associates (1989) reported the value of transesophageal ultrasonography in diagnosis. Bhutani and associates (1996) have confirmed the facility of endoscopic ultrasound in the diagnosis of an esophageal cyst. Specifically, Catalano (1997) has emphasized that endosonography can demonstrate size and layer of origin, and, by its echo texture, can accurately predict the etiology of submucosal tumors. With increased experience, Lupetin and Dash (1987) and Rafal and Markisz (1991) advise that MR imaging is becoming the diagnostic method of choice for definite identification of these cysts. Biopsy of a suspected cyst is contraindicated.

Treatment

Definitive treatment, detailed by Arbona (1984) and Holcomb (1989) and their colleagues, is surgical excision, often effectively accomplished by enucleation without injury to the mucosa. Thus, the cyst usually can be separated easily from the esophageal wall, and resection of the esophagus is rarely required. Long tubular duplications require dissection of the common septum between the duplication and the esophagus (Fig. 149-7). Mounting evidence suggests that video-assisted thoracoscopy should represent the first-line approach in these patients. Small, asymptomatic lesions in the adult may not need to be removed. In infancy, adherence of the duplication to surrounding structures and its vascularity frequently make complete removal hazardous. Haller and colleagues (1975) report the merit of partial excision with complete removal of the lining in such circumstances. Cioffi and co-workers (1998) recommended surgical excision in all patients, with a video-thoracoscopic method being the first-line approach.

Prognosis

The prognosis of patients with duplication cysts is excellent, emphasizing the desirability of their surgical removal

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Bronchogenic Esophageal Cysts

Bronchogenic cysts arising in the esophagus are the subject of a report and review by Zaunbauer and co-workers (1996). Esophageal bronchogenic cysts are characterized by intramural location and the presence of cartilage. The embryologic origin of these cysts relates to the separation of the lung buds from the esophagus by the tracheoesophageal septum. It is possible that fragments of the respiratory tract fuse with the esophagus and become cysts. Again, with elongation of the esophagus, the cysts become located in the lower portion. Consistent with the management of other benign tumors and cysts of the esophagus, thoracoscopic enucleation of this rare lesion has been successfully performed by Koizumi and colleagues (1998). Esophageal bronchogenic cysts have not been associated with vertebral abnormalities.

Esophageal Gastric Cysts

Esophageal gastric cysts probably arise when the stomach descends in the embryonic stage and gastric mucosa is retained in the esophagus. To be classified as an esophageal gastric cyst, the cyst must be intramural and lined with gastric mucosa. Most patients are identified at an early age because of bronchial compression or the development of peptic ulceration. Indeed, Peiper and associates (1995) have reported the ultrasonographic detection of a bleeding esophageal duplication in utero. Esophageal gastric cysts are not associated with the abnormalities of the vertebral column and spinal canal that are characteristic of neurenteric gastric lined cysts (the split notochord syndrome).

Inclusion Cysts

Inclusion cysts of the esophagus are intramural and contain epithelium that is of a respiratory or squamous type. They cannot be classified as duplications or esophageal bronchogenic cysts, however, because they are not covered by two muscle layers and do not contain cartilage. Their etiology

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Acquired Cysts

Superficial and deep submucosal glands constitute a part of the normal anatomy of the esophagus (see Chapter 123). For unclear reasons, these glands may develop into cystic lesions. Theories such as chronic inflammation of the esophagus, partial obstruction of the ducts, and hyperplasia of the esophageal mucosa have been offered. Chronic esophagitis, in fact, is a common factor in almost all published cases.

The acquired cysts (retention cysts) may be single or multiple. The term esophagitis cystica has been used to describe the occurrence of multiple retention cysts. Retention cysts are small and usually are located in the upper one-third of the esophagus. Although these cysts are not unusual in pathologic series, the presence of a symptomatic retention cyst in an adult is extremely rare.

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