Ann Thorac Surg 2004;78:e51-e53
© 2004 The Society of Thoracic Surgeons
Case report
Lymphangioma of the esophagus: Surgical treatment
Yong Han Yoon, MDa,*,
Kwang Ho Kim, MDa,
Wan Ki Baek, MDa,
Joung Taek Kim, MDa,
Kuk Hui Son, MDa,
Jee Young Han, MDb,
Jung Il Lee, MDc
a Departments of Thoracic and Cardiovascular SurgeryCollege of Medicine, Inha University, Incheon, South Korea
b Department of PathologyCollege of Medicine, Inha University, Incheon, South Korea
c Department of Internal Medicine and Division of Gastroenterology, College of Medicine, Inha University, Incheon, South Korea
Accepted for publication February 10, 2004.
* Address reprint requests to Dr Yoon, Department of Thoracic and Cardiovascular Surgery, Inha University Hospital, 7-206 Shinheungdong 3-ka, Choonggu, Incheon 400-711, South Korea
yoonkwon{at}inha.ac.kr
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Abstract
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Lymphangioma of the esophagus is a very rare submucosal tumor, but it can usually be removed without complication by endoscopic resection because of its small size and benign nature. My colleagues and I report a case of an uncommonly large lymphangioma (5.1 x 2.3 x 1.7 cm) in the distal esophagus treated by distal esophagectomy and esophagogastrostomy for complete resection without narrowing the esophagus. The patient's immediate postoperative course was uneventful, and he remains well 6 months after the operation.
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Introduction
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Lymphangioma of the esophagus is a rare submucosal benign tumor that can usually be diagnosed and removed by endoscopy. However, my colleagues and I experienced a large esophageal lymphangioma that was treated by distal esophagectomy and esophagogastrostomy.
A 72-year-old Korean man was admitted because of vomiting immediately after meals for the previous 5 days. He had been in relatively good health before this symptom developed. His only medical problem was hypertension, which was controlled with an oral antihypertensive drug. He had smoked a pack of cigarettes a day for 40 years. He had no nausea, dysphagia, or weight loss. The standard hematologic and biochemical laboratory blood tests were within the normal limits. Esophagogastroduodenoscopy revealed a huge, longitudinally protruding mass lesion with mucosal ulceration located 32 cm distal to the upper incisor (Fig 1). Esophageal ultrasound showed a mixed hyperechoic and anechoic mosaic-patterned mass in the submucosal layer and muscularis propria (Fig 2). The endoscopic biopsy of the mass showed mild to moderate dysplasia with ulceration and acute and chronic inflammation. A computed tomogram of the chest with contrast enhancement demonstrated that the mass was localized to the esophagus (Fig 3). A left lateral thoracotomy through the seventh intercostal space was performed, and a mass (5.1 x 2.3 x 1.7 cm) was located 5 cm proximal to the esophagogastric junction. It was covered with the esophageal muscles. Esophageal myotomy over the mass was performed longitudinally, and it revealed that the tumor mass was infiltrated into the mucosal layer and that its base was broad. It was impossible to enucleate it. The tumor was resected with a 45-mm Autosuture TX (1 Proximate; Ethicon Endo-Surgery, Cincinnati, OH) stapler applied tangentially. Frozen section of the mass showed lymphangioma, and the free resection margin was also confirmed. The mucosal opening was closed with TX 45 mm, and the muscle layer was repaired with 3-0 Vicryl (Ethicon, Inc, Somerville, NJ). After the repair, the esophageal lumen was so narrowed that a Levin tube was unable to pass through the resected portion of the esophagus. A Sweet operation—distal esophagectomy (7 cm) with midthoracic esophagogastrostomy—was performed to restore the esophagogastric continuity without laparotomy. A circumferential incision of the diaphragm was performed, and the stomach was pulled through it into the left pleural cavity. The fundus of the stomach was incised 5 cm transversely, and the esophagogastrostomy was made with end-to-end anastomoses (Premium Plus CEEA; Auto Suture; Tyco Healthcare Group, Mansfield, MA) with a 28-mm stapler. Reinforcement sutures of the resected margin of the stomach were performed. The stab wound at the stomach for insertion of the end-to-end anastomoses was closed with the TX 60 mm, and reinforcement was performed. Then the distal site of the esophagus was repaired with TX 60 mm, and reinforcement was performed.
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Fig 1. Esophagoduodenogastroscopic finding. A bulging mass 5.0 cm in diameter with bridging folds was seen at the lower part of esophagus extended to the gastroesophageal junction. The mass was covered by relatively normal mucosa, but several erosive lesions were noticed on the overlying mucosa.
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Fig 2. Endoscopic ultrasonographic finding. A 5.0-cm mass with heterogeneous internal echo originated in the submucosal layer.
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The permanent pathologic diagnosis was consistent with cavernous lymphangioma (Fig 4). The immediate postoperative course was uneventful, and the patient was discharged on the 14th postoperative day. He was well 6 months after the operation.
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Fig 4. Biopsy specimen showing multiple dilated lymphatic vessels of varying sizes with lymphoid accumulation (arrow). (Hematoxylin and eosin, x100.)
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Comment
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Lymphangioma of the esophagus is very rare, and only 12 cases were found in a MEDLINE literature search. It is a mass with irregularly dilated lymphatic channels that contains chylous or serous fluid [1]. The most frequent location of lymphangioma is in the head and neck. Only 1% of these tumors have been discovered in the gastrointestinal tract [1]. Gangl and colleagues [2] found 32 patients with gastrointestinal lymphangioma in their review of the literature up to 1980. The colon was the most frequent location for development of a lymphangioma in the gastrointestinal tract, followed by the duodenum and stomach. The esophagus was the most unusual location for its development.
Symptoms may vary depending on the location of the tumor and the degree of obstruction [3]. Esophagogram and endoscopy are commonly used to establish the diagnosis [4]. The endoscopic appearance of lymphangiomas is known to be a submucosal polypoid mass with a translucent and lustrous surface; it is white or yellowish and pliable when probed by biopsy forceps [5]. Presently, esophageal ultrasound is the most useful for distinguishing a lymphangioma from leiomyoma or other submucosal tumors because it clearly shows cyst formation and intact esophageal muscles [4]. Endoscopic biopsy is necessary for definitive diagnosis, but endoscopic sampling may lead to misleading information because a normal surface epithelium covers the tumor [3].
The treatment of esophageal lymphangioma may be conservative if a definitive histologic diagnosis is obtained and the patient is asymptomatic [3]. Suwa and associates [4] reported that an esophageal lymphangioma less than 25 mm in diameter can be safely resected endoscopically. Kuramoto and associates [5] stated that lymphangiomas smaller than 20 mm in diameter can be treated by endoscopic polypectomy. However, a large tumor with symptoms or a mass suspected as a malignant lesion require surgical resection [3]. Determining the extent of the tumor through the esophageal wall may be difficult during operation because a lymphangioma is soft and compressible and pushes easily into the lumen. Yoshida and colleagues [3] suggested that a combination of surgery with operative endoscopy is useful to confirm the location of the tumor. No cases of malignant transformation have been described in the literature [4]. In the 12 reports about esophageal lymphangioma, the largest tumor size was 5 cm in diameter (Table 1). Four patients underwent open thoracotomy, and enucleation of the tumor was possible in most. In our case, we could confirm the frozen biopsy sample as a lymphangioma during the operation, so we tried to perform a limited tangential resection of the mass, including the mucosa. However, esophagectomy was unavoidable because the remaining lumen of the esophagus was very narrow. After the esophagectomy, we performed a Sweet operation to avoid laparotomy. Esophagogastric mobilization and anastomosis was easily performed with a circumferential incision of the diaphragm. In a case in which the esophagectomy is unavoidable because of the tumor size, the Sweet operation is an easy and safe technique that minimizes the invasiveness of the operation.
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References
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- Lee BI, Kim BW, Kim KM, et al. Esophageal lymphangiomatosis: a case report. Gastrointest Endosc. 2002;56:589–591[Medline]
- Gangl A, Polteraver P, Krepler R, Kumpan W. A further case of submucosal lymphangioma of the duodenum diagnosed during endoscopy. Endoscopy. 1980;12:188–190[Medline]
- Yoshida Y, Okamura T, Ezaki T, et al. Lymphangioma of the esophagus: a case report and review of the literature. Thorax. 1994;49:1267–1268[Abstract/Free Full Text]
- Suwa T, Ozawa S, Ando N, et al. Case report. Lymphangioma of the esophagus endoscopically resected. J Gastroenterol Hepatol. 1996;11:786–788[Medline]
- Kuramoto S, Sakai S, Tsuda K, et al. Lymphangioma of the large intestine. Report of a case. Dis Colon Rectum. 1988;31:900–905[Medline]
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Abstract
Introduction
