Ann Thorac Surg 2010;89:610-612. doi:10.1016/j.athoracsur.2009.06.094
© 2010 The Society of Thoracic Surgeons
Case Reports
Giant Multi-Polypoid Liposarcoma of the Esophagus: An Atypical Presentation
Maurice A. Smith, MDa,*,
Emily Kluck, PA-Ca,
Sanjay Jagannath, MDb,
Stephen C. Yang, MDa
a Division of Thoracic Surgery, Johns Hopkins Hospital, Baltimore, Maryland
b Division of Gastroenterology, Johns Hopkins Hospital, Baltimore, Maryland
Accepted for publication June 22, 2009.
* Address correspondence to Dr Smith, 600 N Wolfe St, 618 Blalock, Johns Hopkins Hospital, Baltimore, MD 21287 (Email: msmit179{at}jhmi.edu).
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Abstract
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Liposarcomas of the esophagus are rare with only 19 cases reported in the English literature. We present a giant, well-differentiated liposarcoma of the esophagus with multiple pedunculated polypoid-like growths, which made it radiographically and pathologically noncharacteristic. We discuss the diagnostic dilemmas, clinical and pathologic findings, and surgical treatment.
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Introduction
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Sarcomas of the upper aerodigestive tract are rare. When all histologic types of esophageal sarcomas are combined, they comprise only 0.5% of all esophageal neoplasms. Within that group, liposarcomas are exceedingly rare with only 19 cases reported in the English literature since it was first reported by Mansour and colleagues [1] in 1983. We report a giant multi-polypoid liposarcoma of the esophagus, and we discuss the diagnostic dilemmas, clinical and pathologic findings, and surgical treatment.
A 38-year-old African American man presented with complaints of increasing shortness of breath, hoarseness, cough, chest discomfort, and progressive dysphagia for 1 month. The dysphagia began as reflux symptoms 3 years prior, which were refractory to medications. He then noted trouble swallowing solid food, had intermittent trouble with liquids, and had a constant feeling of a mass protruding in the back of his throat. In the days leading to hospitalization, the patient noted that he had difficulty breathing, with audible wheezing, and dull, heavy chest discomfort that would come and go after meals. He did not experience weight loss, and the remainder of his history was noncontributory. The complete physical examination was unremarkable, except for hoarseness and audible upper airway wheezing. A computed tomographic scan of the chest and abdomen (seen in Fig 1) showed an extensively dilated esophagus with a width of 8 cm at the thoracic inlet, 6.5 cm at the tracheal bifurcation, and a maximum width of 11 cm, causing slight tracheal compression at the level of the right main stem bronchus. The esophagus contained multiple, mixed fat and soft tissue density circumscribed masses protruding into the lumen, extending from the cervical esophagus to the gastroesophageal junction. A cine-esophagopharyngogram showed a markedly dilated esophagus measuring 3 to 6 cm in diameter, with a large, multi-lobulated filling defect throughout the esophagus from the cricopharyngeus down to the gastroesophageal junction, with individual polypoid components off the main mass measuring as much as to 2 to 3 cm each. Diagnostic flexible esophagoscopy revealed a large multi-polypoid lesion starting 3 cm below the upper esophageal sphincter and extending just superior to the gastroesophageal junction, nearly encompassing the entire esophageal lumen. Endoscopic ultrasound revealed the lesion to arise from the muscularis mucosa layer with normal and intact muscularis propria layer. Small vessels were seen within the polyp, and at the time of the ultrasound, it was difficult to discriminate between the possibility of multiple discrete stalks or one main mass with multiple polyps.
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Fig 1. Computed tomographic scan of the chest showing a large mass (A) with multiple circumscribed lobulations extending from the thoracic inlet to the gastroesophageal junction.
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With the hope of excising the entire tumor in a single procedure, the patient underwent a left neck esophagotomy. At this time it was difficult to identify the stalk and the mass seemed to be multiple polypoid tumors invading the mucosal layer. Only two tumors could be shelled out at the level of the cricopharyngeus, and no vascular pedicle was identified. The original path was read as fibrolipomas (measuring 4.5 x 2.5 x 2.0 cm and 4.0 x 3.0 x 2.5 cm). Postoperatively, the patient did well (with resolution of symptoms) and advanced to a soft diet. He was discharged on postoperative day 5 with the intent of preparing for a future esophagectomy.
Within 2 weeks the patient had progressive dysphagia, a 5-lb weight loss, and worsened audible wheezing, with a computed tomographic scan that was minimally unchanged. At this time, a three-incision esophagectomy with gastric pull up and cervical anastomosis was performed. The vascular pedicle was identified at the proximal esophageal margin and was stapled, ligated, and sent as a proximal margin. Postoperatively the patient did well with resolution of wheezing, hoarseness, chest heaviness, and dysphagia. The patient was discharged home on postoperative day 8 on a soft diet. The final pathologic report indicated that the mass found was a single, well-differentiated liposarcoma (17.5 x 11 x 5.3 cm) with multiple pedunculated polypoid-like growths, as shown in Figure 2. The proximal stalk margin, lymph nodes, stomach, and esophagus were all free of tumor. The patient will require no further treatment but will have a close follow-up.
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Fig 2. A macroscopic view of the liposarcoma mass (17.5 x 11 x 5.3 cm) with multiple pedunculated polypoid like growths (*) and a vascular stalk (A).
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Comment
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Due to the multi-polypoid nature of this liposarcoma, it caused some diagnostic and management challenges. First, the computed tomographic scan and cine-esophagopharyngogram described the possibility of multiple circumscribed masses, whereas the endoscopic ultrasound revealed only one stalk. This formed the idea that the patient had a typical polyp that could easily be excised through a left neck incision. The first operative and initial pathologic findings were consistent with multiple fibrolipomas, which led to the attempt at a thoracic esophagotomy at the second operation, which ultimately led to an esophagectomy.
This is case 20 of esophageal liposarcomas, according to our findings of reported cases in the English literature. Songtoa and colleagues [2] list a table of 19 previously described cases. The histologic types of liposarcoma are well-differentiated, myxoid, round cell, and pleomorphic [3]. Including our case, 70% of the liposarcomas were found to be well-differentiated, 25% were myxoid, and 5% were pleomorphic. With esophageal liposarcoma tumors, there is a male predominance of 70% and the average age of patients ranged from 38 to 73 years, with our case being the youngest patient. Tumor size ranged from 4 to 23 cm with 80% of the masses originating in the cervical esophagus. The predominant clinical symptoms included progressive dysphagia, weight loss, vomiting, retrosternal chest pain, respiratory distress, odonophagia anemia, cough, and sudden death [4]. Some patients presented with recurrent vomiting followed by extrusion of the mass through the mouth. A polypectomy was performed in 70% of cases, whereas a total esophagectomy was required in the other cases for removal of the mass. The most reliable prognostic factors for patients with esophageal liposarcoma tumors are grade, histologic subtype, location, and adequacy of surgical treatment. There is a 75% to 100% 5-year survival rate, and only two tumors (10%) locally recurred after 6.5 and 25 years with no documented metastasis [4, 5].
This case involved a well-differentiated liposarcoma of the esophagus and the twentieth case reported in the literature. The multiple pedunculated polypoid-like growths caused the liposarcoma to be radiographically and pathologically noncharacteristic.
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References
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- Mansour KA, Fritz RC, Jacobs DM, Vellios F. Pedunculated liposarcoma of the esophagus: a first case report J Thoracic Cardiovas Surg 1983;86:447-450.[Abstract]
- Songtoa X, Zheglang X, Yingyong H, Yunshan T. Primary pedunculated giant esophageal liposarcoma: case report Dysphagia 2008;23:327-330.[Medline]
- Temes R, Quiunn P, Davis M, Endara S, Follis F, Pett S, Wernly J. Endoscopic resection of esophageal liposarcoma J Thorac Cardiovasc Surg 1998;116:365-367.[Free Full Text]
- Liakakos T, Troupis T, Tzathas C, et al. Primary liposarcoma of esophagus: a case report World J Gastroenterol 2006;12:1149-1152.[Medline]
- Garcia M, Buitrago E, Bejarano P, Casillas J. Large esophageal liposarcoma a case report and review of the literature Arch Patol Lab Med 2004;128:922-925.
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Abstract
Introduction
