Well-differentiated liposarcoma of esophagus
Year: 2006
Type: article
Study design: Case Report
Abstract: Gastrointestinal liposarcoma is rare, and the esophagus is the least common location at which this kind of liposarcoma may occur. We reported a patient with a well-differentiated liposarcoma of esophagus, and discussed the diagnostic utility of these imaging techniques in patient management. CASE REPORT A 49-year-old man was admitted to our hospital due to dysphagia. He complained of one-year dysphagia, aggravating in recent 4 months and a loss of 7 kg body weight. No abnormalities were found by his medical history. Physicial examination did not reveal any positive signs. Laboratory tests revealed normal counts of blood cells. Barium swallow examination revealed a longitudinal ellipse submucosal mass at the posterior wall of upper-mid esophagus (Fig. 1). Esophagoscopy revealed an external oppression at the level of upper-mid esophagus, 20-28 cm from the incisors, and a whitish intraluminal protuberance, 28 cm from the incisors. Plain helical CT examination showed an inhomo- geneous ovoid mass of esophagus with fatty density (Fig. 2). The non-fat components of the mass were mildly enhanced after intravenous contrast material administration (Figs. 3 and 4). The mass was heterogenous with high signal intensity on T1-weighted and T2-weighted images (Figs. 5 and 6). Resection was performed surgically by a triple approach with right cervicotomy, thoracotomy and laparotomy. At surgery, we found a 8.0 cm× 5.0 cm × 4.0 cm encapsulated soft taupe mass beneath the mucosa of posterior wall of esophagus with a 1.0 cm × 1.0 cm × 1.0 cm whitish protuberance invaded into the esophageal lumen. The mass was originated from the muscle layer of esophagus with a pedicel from upper esophagus. Pathological evaluation of the resected specimen confirmed the presence of a well-differentiated liposarcoma with lipocytes in various size, scattered spindle to stellate cells and lipoblasts (Figs. 7 and 8). The resection margin contained tumor cells. Immunohistochemical study indicated that Vim and S100 were positive while CD68, CD34 and desmin were negative. No dysphagia or other complications occured during his postoperative stay in hospital.Fig. 1.: Barium swallow examination shows a longitudinal ellipse submucosal mass at the level of upper-mid esophagus.Fig. 2.: Plain CT scan shows an inhomogeneous hypoattenuation ovoid mass with fatty tissue.Fig. 3.: Ehanced CT scan shows that the non-fat component of the mass is mildly enhanced.Fig. 4.: Multi-planar reconstruction (MPR) CT shows the mass located at the posterior wall of upper-mid esophagus.Fig. 5.: MR T1 weighted image shows that the mass is heterogeneous with high signal intensity.Fig. 6.: MR T2 weighted image shows that the mass is heterogeneous with high signal intensity.Fig. 7.: Low-power photomicrograph shows lipocytes in various size with scattered spindle to stellate cells and lipoblasts (HE, original magnification×10).Fig. 8.: High-power photomicrograph shows lipoblasts with atypical nucleus deeply stained and cytoplasmic vacuolation (HE, original magnification×40).DISCUSSION Liposarcoma is the most common tumor originating from adipose tissue, which may occur anywhere in the body, and typically occur in the retroperitoneum or lower extremities. They rarely arise in the gastrointestinal tract. The incidence of gastrointestinal liposarcoma is 0.1% to 5.8% at autopsy, and the esophagus is the least common location (1.2% to 1.5% of all gastrointestinal lipoma).1 To our knowledge, there is only one case report of esophageal liposarcoma in Chinese medical literature.2 Esophageal liposarcoma is a slowly growing tumor resulting from the mucosa or submucosal soft tissue layers of the esophagus. Pathollogically, the tumor is either well-differentiated liposarcoma or myxoid histotype. The main symptoms of esophageal liposarcoma are similar to those of esophageal carcinoma, such as dysphagia, accompanied by weight loss, foreign body sensation, retrosternal pain.2,3 For this patient, esophagoscopy and imaging studies indicated that the mass was originated from submocusa of posterior wall of esophagus. A long-term follow-up was done because well-differentiated liposarcoma was a malignant tumor which had a tendency to local recurrence after inadequate resection. The presence of fat on CT or MRI may indicate a lipomatous tumor such as lipoma, atypical lipoma, and liposarcoma.4 MRI and CT can be used to evaluate fat component of the various grades of tumor. Lipoma has essentially 100% fat, whereas those with less than 75% fat were atypical lipoma or low grade liposarcoma. Medium or high grade liposarcoma typically does not contain any fat on MRI.5,6 According to literature, well-differentiated liposarcoma mainly consists of fat with septations or nodules which enhance slightly or had no enhancement on enhanced MRI.7,8 In our patient, we measured the volume of fat slice by slice on CT and MR T1-weighted images, then confirmed that the tumor consisted of less than 75% fat. The non-fat component of tumor was mildly enhanced on the enhanced CT. In summary, several imaging procedures such as barium swallow study, CT, MRI are useful tools in diagnosing esophageal liposarcoma. Barium swallow study demonstrates the presence of esophageal tumor. The presence of fat tissue on CT or MRI may indicate the existence of lipomatous tumor. On the basis of the ratio of fat component in tumor, the grade of tumor can be evaluated initially.
Source: Chinese Medical Journal
Authors: Bin YANG, Pei-zhi SHI, Xiao LI, Ru-jun XU
Language: English
Subfield: Pulmonary and Respiratory Medicine
Field: Medicine
Domain: Health Sciences
Open Access status: gold
Estimated APC paid: $2,000
OpenAlex ID: W280100829