Giant liposarcoma of the esophagus

Centre Hospitalier Universitaire de Sherbrooke (CHUS), Sherbrooke, Québec Correspondence and reprints: Dr Alain Watier, Centre Hospitalier Universitaire de Sherbrooke (CHUS), 3001 12e avenue Nord, Sherbrooke, Québec J1H 5N4. Telephone 819-346-1110, fax 819-822-6797, e-mail awatier@abacom.com Received for publication February 11, 2002. Accepted April 1, 2002 A Beaudoin, C Journet, A Watier, C-J Mongeau, M Chagnon, R Beaudry. Giant liposarcoma of the esophagus. Can J Gastroenterol 2002;16(6):377-379.

L ipomatous tumours are rarely seen in the gastrointesti- nal tract.They have an incidence of 0.1% to 5.8% at autopsy and are predominantly found in the terminal ileum and colon (1).The esophagus is the least frequent alimentary tract location, comprising only 1.2% to 1.5% of all gastrointestinal lipomas (2).
We report a woman who developed a liposarcoma of the upper esophagus, which is presumed to be a recurrence of a primary tumour that had been resected 25 years earlier.

CASE PRESENTATION
A 68-year-old woman had a four-month history of progressive dysphagia, initially to solid food and then to liquids.An upper endoscopy demonstrated a polypoid mass of the proximal esophagus, extending from 18 to 31 cm.An endoluminal esophageal polyp was seen on upper gastrointestinal series, while computed tomography scan demonstrated that the polyp had a lipomatous component (Figures 1,2).While waiting to undergo excision surgery, the patient presented with an episode of vomiting, with extrusion of the polypoid mass from her mouth (Figure 3).Because of the fear of suffocation, the polyp was immediately excised transorally while the patient was under general anesthesia.Her preoperative symptoms resolved immediately after surgery.
The excised specimen consisted of an 8.5×4.5×3cm polypoid mass with bosselated focally ulcerated surface.Cut section demonstrated yellowish-white areas that were more or less completely circumscribed by greyish fibrousappearing tissue (Figure 4).A lobulated adipose tumour with fibrous septae was seen on microscopic examination.The cellular component was predominantly made up of mature adipocytes of irregular size and shape, as well as occasional, marked nuclear atypia.The presence of occasional bona fide lipoblasts was confirmed by nucleocytoplasmic immunostaining with S-100 protein (Figure 5).Spindle and myxoid patterns were also present.Therefore, the histological diagnosis was well differentiated, lipomalike liposarcoma extending to the margin of resection.
The patient is currently awaiting a second surgery for a more radical resection.Of note, she had presented with exactly the same symptoms 25 years earlier while working as a missionary in Africa.At that time, after a vomiting episode, a mass that hung from her mouth to her neck had been extruded.According to the patient, she had undergone a transoral excision.It was not possible to retrieve more information from the foreign hospital where this event occurred.She had no follow-up or pathology results.It is supposed that the first episode was a lipomatous tumour, and most likely a liposarcoma.

Figure 3) Extrusion of a polypoid mass from the mouth of a patient with a giant esophageal liposarcoma, after presenting with an episode of vomiting
This is the first case in the literature to report a possible recurrence of a primary liposarcoma of the esophagus 25 years after a first episode.

DISCUSSION
Liposarcomas comprise 9.8% to 16% of all soft tissue sarcomas (5).These are the most common sarcomas of the lower extremities and retroperitoneum (3).Esophageal liposarcomas are very rare, slowly growing tumours that arise from the soft tissue layers of the esophagus.Those tumours are believed to originate from primitive mesenchymal cells rather than mature adipose tissue (6).The histological types of liposarcomas are well differentiated, myxoid, round cell and pleomorphic (10).
All patients with esophageal liposarcomas reported in the literature complained of dysphagia (5,8).Other symptoms may include weight loss, foreign body sensation and, rarely, gastrointestinal bleeding or asphyxia.There are often surprisingly few symptoms, until the tumour has attained a considerable size.
Barium swallow and upper endoscopy series demonstrate nonspecific features and only define the presence of a polypoid esophageal tumour (7).The accuracy of the diagnosis is enhanced by computed tomography scan and magnetic resonance imaging because of the fatty nature of the lesion (8).However, relying solely on these methods could lead to misdiagnosis because of their inability to distinguish between lipomas and liposarcomas.Histological examination is often the most reliable method for making the correct diagnosis, although the pathological literature on this subject is sparse.
It is critical to obtain histologically free margins on surgical resection, considering the high local recurrence rate (5).Liposarcomas of all sites have been associated with other primary malignant tumours in approximately 12% of cases in Hajdu's series (11).Our patient had no other known malignant disease.A thorough, long term follow-up must be planned for patients with this type of tumour.
Giant liposarcoma of the esophagusCan J Gastroenterol Vol 16 No 6 June 2002 379

Figure 4 ) 5 )
Figure 4) Cut gross specimen of a polypoid mass excized from a patient with a giant esophageal liposarcoma Figure 5) Nucleocytoplasmic immunostaining of a section of the polypoid mass shown in Figure 4, demonstrating well differentiated liposarcoma (original magnification ×160)