Ann Thorac Surg 2001;72:2117-2119
© 2001 The Society of Thoracic Surgeons
Case report
Adenocarcinoma arising in a retained esophageal remnant
Onur Genc, MDa,
Ronald K. Knight, FRCPc,
Andrew G. Nicholson, DMb,
Peter Goldstraw, FRCS*a
a Department of Thoracic Surgery, Royal Brompton Hospital, London, United Kingdom
b Department of Histopathology, Royal Brompton Hospital, London, United Kingdom
c Frimley Park Hospital, Surrey, United Kingdom
Accepted for publication March 27, 2001.
* Address reprint requests to Mr Goldstraw, Royal Brompton Hospital, Sydney St SW 3 6 NP, London, England, United Kingdom
e-mail: pgoldstraw{at}rbh.nthnames.nhs.uk
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Abstract
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Fistulation between the trachea and an infected mucocele arising from an excluded retained esophageal segment is an interesting and unusual complication of esophageal bypass surgery. We present such a case in which there was also malignant transformation in the cyst wall, manifested by invasive adenocarcinoma and widespread high-grade glandular dysplasia.
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Introduction
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Bypass of the diseased esophagus has been undertaken in the treatment of varied esophageal conditions, including benign strictures, perforations and rupture, unresectable esophageal cancer, and congenital tracheoesophageal fistula. Mucoceles may form in the excluded esophageal segment, but they reportedly remain small and asymptomatic [1]. We describe a patient who presented with malignant transformation in an excluded esophageal segment, presenting through fistulation into the trachea of an enlarged and infected mucocele.
A male patient underwent attempted closure of a congenital tracheoesophageal fistula during the first year of life, but because of complications, he eventually required a colonic conduit and subtotal esophagectomy, with exclusion of a small portion of the distal esophagus. He subsequently remained well and asymptomatic until 23 years of age, when he presented with recurrent episodes of chest infection, expectorating large quantities of pus. A computed tomographic scan showed a 90-mm x 40-mm thick-walled abscess cavity in the mediastinum, extending from the carina to the diaphragm and lying to the right of the vertebral bodies (Fig 1). On bronchoscopy, a large diverticulum was seen in the posterior wall of the trachea above the carina, although no fistula was seen at this time. A diagnosis of an infected mucocele was made. To drain the abscess cavity, a transcutaneous pigtail catheter was inserted, and the patient’s immediate condition subsequently improved.
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Fig 1. Computed tomographic scan of the thorax shows a thick-walled cystic mass in the right posterior mediastinum; the cyst is filled with fluidlike material.
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However, the patient continued to drain small amounts of pus through the pigtail catheter, and he was therefore referred for surgical excision of the infected mucocele in the excluded portion of distal esophagus. At right thoracotomy, the tracheal diverticulum was found to communicate with the distal esophageal remnant, with a blind-ending mucocele extending through the hiatus and adhering to the upper surface of stomach. The esophageal remnant was mobilized and excised, and the tracheal orifice was repaired with continuous polypropylene sutures. Adjacent soft tissues were sutured against the fistula repair. The opened sac had a focally ulcerated pale mucosal surface and a diffusely thickened fibrotic wall. A perforation in the wall, relating to the pigtail catheter, was noted (Fig 2). Microscopy revealed the majority of the epithelium to be of gastric type and to show widespread high-grade glandular dysplasia, although squamous epithelium without dysplasia was present at the fistula margin. In addition, there were several foci of invasive adenocarcinoma, one of which extended into the muscularis propria. Both invasive and dysplastic components were completely excised (Fig 3). No Helicobacter pylori organisms were seen. The patient’s postoperative course was uneventful, and he remains well and free of disease at 6 months.
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Fig 2. Opened esophageal remnant with tracheal fistula. The wall is thickened, and a pigtail catheter is noted in situ.
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Fig 3. A section from the cyst wall shows a polypoid area of glandular dysplasia with a focus of invasive adenocarcinoma extending to infiltrate the superficial aspect of the muscularis propria.
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Comment
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Primary repair of a congenital tracheoesophageal fistula can be undertaken in most cases [2], but esophageal replacement is sometimes required, either following breakdown of the primary anastomosis or in patients with associated "long-gap" esophageal atresia. In such cases, the esophagus is usually resected and restored by a colon interposition or gastric tube [3, 4]. However, this has not always been the case, as seen in this report, in that the distal esophagus had not been resected when the colonic graft was placed through the left chest to the neck. Experimental studies in dogs have shown that an excluded esophageal segment may develop into a cyst within a matter of weeks, with the growth of the cyst regulated by its internal pressure [5], and series of mucoceles arising post–esophageal replacement have been reported, in cysts lined by both squamous and glandular epithelium [1, 6]. However, infection within an esophageal mucocele is very rare, with only one previous report [6]. Furthermore, although premalignant changes have been described in an esophageal mucocele postsurgery for achalasia [6], development of an invasive adenocarcinoma is a unique finding. This may reflect the 20-year history, with unknown potential carcinogenic factors in the cyst contributing to the eventual development of neoplasia. It is also interesting that most esophageal adenocarcinomas arise on a background of metaplasia secondary to gastroesophageal reflux. In this patient, it is most unlikely that metaplasia developed within the first year of life; therefore, either the columnar epithelium from which this tumor developed was heterotopic in nature, or, more likely, it arose in association with chronic inflammation within the enclosed cyst.
The presence of an adenocarcinoma may also have contributed to the patient’s presentation through overproduction of mucus leading to increased pressure and fistulation. However, given the history of recent infection within the mucocele, it is likely that a combination of these factors led to increased pressure and recanalization of the fistula. We conclude that, if there is a risk of malignant transformation within these remnants, surgery should perhaps be considered even if patients are asymptomatic.
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References
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Olsen C.O., Hopkins R.A., Postlethwait R.W. Management of an infected mucocele occurring in a bypassed excluded esophageal segment. Ann Thorac Surg 1985;40:73-75.[Abstract/Free Full Text]
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Moores D.W.O., Campbell D.P. Congenital anomalies of the oesophagus. In: Shields T.W., ed. . General thoracic surgery. Philadelphia: Lippincott, Williams and Wilkins, 1994:1529-1540.
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Kelly J.P., Shackelford G.D., Roper C.L. Esophageal replacement with colon in children: functional results and long-term growth. Ann Thorac Surg 1983;36:634-643.[Abstract/Free Full Text]
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Richardson J.V., Heintz S.E., Rossi N.P., et al. Esophageal atresia and tracheoesophageal fistula. Ann Thorac Surg 1980;29:364-368.[Abstract/Free Full Text]
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Deaton W.R., Bradshaw H.H. The fate of an isolated segment of the oesophagus. J Thorac Surg 1952;23:570-574.
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Kamath M.V., Ellison G.R., Rubin W.J., Moore H.V., Pai G.P. Esophageal mucocele: a complication of blind loop esophagus. Ann Thorac Surg 1987;43:263-269.[Abstract/Free Full Text]
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Abstract
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