Primary Liposarcoma of the Esophagus: 455

Purpose: Background: Liposarcomas are the most common malignant soft tissue tumors in adults. They are not common in the gastrointestinal tract. Liposarcomas are reported at a rate of 0.1% to 5.8% at autopsy; however, only 1.2% to 1.5% of those cases are seen in the esophagus. Primary esophageal liposarcomas grow as polypoid masses within the esophageal lumen, but are asymptomatic until they have reached a substantial size. Clinically they present with weight loss, progressive dysphagia, pain, regurgitation, hemorrhage anemia vomiting. Case report: A 75 year-old male presented with a 2 month history of progressive dysphagia, initially for solids and then for liquids, weight loss (11 pounds) and a 1 day history of melenic stool, lethargy and weakness. Physical exam showed clubbing, pallor, and hypertension. CT scan revealed a longitudinal esophageal mass containing fat and fibrous/soft tissue, favoring a giant fibrovascular polyp of the esophagus. Esophagus mass biopsy revealed granulation tissue with severe acute and chronic inflammation and marked reactive stroma atypia. C-kit, ki-67, and p53 were consistent with the diagnosis. Esophagogastrectomy was performed revealing a well encapsulated polypoid tan/yellow mass, weighting 120 grams and measuring 17x7x3 cm, with a stalk. A diagnosis of malignant neoplasm most consistent with liposarcoma was made. Immunohistochemistry showed positivity for MDM2 and vimentin, and negativity for S-100 and SMA. Cytogenetics was interpreted as a male karyotype with ring and marker chromosomes in 16 cells with supernumerary ring chromosomes and marker chromosomes of unknown origin associated with the diagnosis of atypical lipomas or well differentiated liposarcoma, which supported the diagnosis. The patient had no complications and was dischargde. Nine months later, the patient is asymptomatic and no tumor recurrence has been found. Conclusion: This is an extremely rare finding, with only 20 cases reported in the literature. The diagnosis was confirmed with immunohistochemical and cytogenetic studies, which are extremely useful when confronted with an unusual presentation of a fairly common neoplasm.