Submitted by Paulo Serapio, MD and Joseph Frye, MD (Pathology), with Navid Mehrpoo, MD.
A 29-year-old previously healthy female presents to the emergency department with one week history of sore throat and productive cough. She also endorses generalized difficulty swallowing.
Pertinent Initial Physical Exam / Labs
- General: slightly raspy voice, otherwise alert and comfortable.
- HEENT: Moist mucous membranes, oropharynx clear. Nontender neck.
- Pulmonary: Clear to auscultation bilaterally
- Labs: CBC & CMP: Within normal limits.
- Vital signs stable.
Initial Chest X-Ray: Frontal radiography of the chest reveals a dilated air-filled structure, likely esophagus, with possible air-fluid level (bottom arrow).
Additional Clinical History
Additional history reveals one year of progressive dysphagia with patient reporting needing to take increasing amounts of liquids in order to ingest solid food. Patient endorses intermittent regurgitation of undigested food and gradual weight loss of approximately ten pounds in the past few months.
Further evaluation with esophagogastroduodenoscopy and CT scan was performed.
Contrast enhanced axial CT image reveals an intraluminal, polypoid mass within the esophagus.
Initial EGD demonstrated an ulcerated mass in the proximal and mid esophagus which was biopsied. Pathology from the biopsy returned with nonspecific ulcerated squamous mucosa with underlying spindle cell proliferation. No evidence of a neoplastic process is seen. Pathology was thought to represent a superficial fragment of a fibrovascular polyp or reactive process to ulceration.
Coronal and sagittal reformatted contrast enhanced CT images demonstrate a smooth, intraluminal mass which extends from the cervical esophagus. Fatty component is seen in the superior aspect of the coronal image. Sagittal image demonstrates stalk-like origin arising from the anterior esophageal wall.
Given the CT findings of lobulated intraluminal mass extending from the cervical esophagus with fatty components, the suggestion of fibrovascular polyp was made. Differential diagnosis includes leiomyoma or atypical appearance of esophageal carcinoma.
A repeat rigid esophagoscopy was performed with visualization thought to be most consistent with fibrovascular polyp.
An esophagram was performed and demonstrated an intraluminal filling defect from the lower cervical esophagus extending downwards. A small amount of contrast passed around the partially obstructing mass with stasis of contrast more proximally. The patient spontaneously regurgitated this residual contrast and the study was ended at this time.
Surgical resection of the lesion was performed by thoracic surgery.
Images Courtesy Joseph Frye MD, Pathology
Surgical pathology of the resected mass demonstrated dedifferentiated liposarcoma of the esophagus.
Subsequent bone scan demonstrates no evidence of osseous metastatic disease. The patient underwent adjuvant radiation therapy and at 5 months post resection demonstrated no signs of recurrent disease.
Dedifferentiated liposarcoma is an exceedingly rare tumor of the esophagus. While liposarcomas are a more common neoplasm in the adult population, esophageal location is uncommon. Although the imaging features of this lobulated mass with smooth margins favored fibrovascular polyp, dedifferentiated esophageal liposarcoma has been rarely previously reported with overlapping imaging appearance, and should be considered in the differential diagnosis.
- Brett, C., Miller, D., Jiang, L., Wolfsen, H., Attia, S., Hintenlang, L., Jagadesh, N. and Miller, R. (2016). Dedifferentiated Liposarcoma of the Esophagus: A Case Report and Selected Review of the Literature. Rare Tumors, 8(4), pp.201-202.
- Takiguchi, G., Nakamura, T., Otowa, Y., Tomono, A., Kanaji, S., Oshikiri, T., Suzuki, S., Ishida, T. and Kakeji, Y. (2016). Successful resection of giant esophageal liposarcoma by endoscopic submucosal dissection combined with surgical retrieval: a case report and literature review. Surgical Case Reports, 2(1).