Dysphagia

, Volume 23, Issue 3, pp 327–330

Primary Pedunculated Giant Esophageal Liposarcoma: Case Report

Authors

    • Department of Thoracic SurgeryZhongshan Hospital, Fudan University
  • Zhenglang Xu
    • Department of Thoracic SurgeryZhongshan Hospital, Fudan University
  • Yingyong Hou
    • Department of PathologyZhongshan Hospital, Fudan University
  • Yunshan Tan
    • Department of PathologyZhongshan Hospital, Fudan University
Case Report

DOI: 10.1007/s00455-007-9130-3

Cite this article as:
Xu, S., Xu, Z., Hou, Y. et al. Dysphagia (2008) 23: 327. doi:10.1007/s00455-007-9130-3
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Abstract

Liposarcoma is one of the most common soft tissue sarcomas in adults, but the incidence of esophageal liposarcoma is extremely low. To our knowledge there are only 18 cases of esophageal liposarcoma reported in the English-language literature. Here we report a new case of primary esophageal liposarcoma in a 50-year-old male patient who complained of aggravating dysphagia with intermittent vomiting for 6 months. The tumor was located in the upper esophagus. Polypectomy was performed and histologic examination showed a well-differentiated liposarcoma. Diagnostic and therapeutic tools are discussed and the results of a literature review are discussed.

Keywords

LiposarcomaEsophagusSurgical treatmentPathologyDeglutitionDeglutition disorders

Liposarcoma is one of the most common soft tissue sarcomas in adults but is rarely seen in the gastrointestinal tract, especially in the esophagus. To our knowledge there are only 18 reported cases of esophageal liposarcoma in the English-language literature [118]. Here we report a new case of primary liposarcoma of the esophagus and discuss its clinical and pathologic findings.

Case Report

A 50-year-old male patient was admitted to our hospital due to 6 months of aggravating dysphagia accompanied by intermittent vomiting (initially with solid food, then progressively with difficulty in ingesting semifluid food). No abnormalities were obvious from his medical history, physical examination, and laboratory tests. Barium swallow examination revealed a very dilated esophagus with a tumor protruding into the lumen and occupying a region extending from the cervical to the middle thoracic portion of the esophagus (Fig. 1a). Esophagoscopy examination showed an endoluminal protuberance at the upper midesophagus, around 20-30 cm long from the incisors. This protuberance was covered by normal esophageal mucosa, with slight narrowing of the esophageal lumen. Upper gastrointestinal ultrasonographic endoscopy examination showed an inhomogeneous submucosal mass located at the posterior wall of upper midesophagus. CT scan showed a conspicuous thickening of the upper esophagus, indicating an endoluminal soft tissue tumor with inhomogeneous density. The transverse section of the tumor was 4 cm × 3 cm. The conjunction between the tumor and the esophageal wall was narrow and enhanced after intravenous contrast material administration. The other large area of the tumor was not enhanced (Fig. 1b). MR scan showed that the mass was heterogeneous with high signal intensity on T1-weighted and T2-weighted images (Fig. 1c).
https://static-content.springer.com/image/art%3A10.1007%2Fs00455-007-9130-3/MediaObjects/455_2007_9130_Fig1_HTML.jpg
Fig. 1

a Barium swallow examination showing a dilated esophagus with a tumor protruding into the lumen. b CT scan showing an endoluminal soft tissue tumor located at the upper esophagus. c MR scan illustrating the tumor in the mediastinum, displacing the trachea anteriorly. d The tumor was gray and gray-yellow in the center. e The tumor was covered with squamous epithelia, with adipocytes on the underside. f Protein S100 was positive in well-differentiated lipocytes and several vacuolated lipoblasts and negative in MGCs

The patient was scheduled for surgery because of suspected leiomyosarcoma. Considering the large size of the tumor and the possibility of malignancy and that the tumor was close to the ingress of the esophagus, total esophagectomy and laryngectomy initially were planned. However, after the full thickness of the esophageal wall had been cut, an 8 × 5 × 5 cm intraluminal soft mass was found located above the superior margin of the aortic arch and beneath the level of the cupula of pleura. Located close to the ingress of the esophagus, the mass had a stalk 3 cm long and 2.5 cm in diameter. Therefore, polypectomy was performed.

Pathologic Findings

The tumor had a smooth surface and was 11 × 5 × 5 cm including the stalk. It was gray-red outside and gray and gray-yellow in the center (Fig. 1d). Under the microscope the tumor was covered with squamous epithelium, with ulceration and granulation tissue in some areas. The tumor consisted of lamellarly distributed vacuolated adipocytes that were similar to the well-differentiated adipose tissue. Cell-type analysis showed that the tumor was composed of spindle cells with deep dyed nuclei, which were atypia and arranged irregularly as fasciculation. The tumor also consisted of multinucleated giant cells (MGCs) among well-differentiated adipocytes, spindle cells, and abundant interstitial collagen fibers. The giant mass had vacuolated cytoplasm, which was very similar to vacuolated lipoblasts and somewhat similar to anthoid MGCs. All these cells were in a mixed and crossed arrangement. Mitotic figures were infrequent (about 2/50, high power field, HPG) in the mass and tumor necrosis was not seen (Fig. 1e). Immunohistochemical study showed that protein S100 was positive in well-differentiated lipocytes and several vacuolated lipoblasts (Fig. 1f), CD68 was positive in MGCs, CD34 was focally positive in spindle cells, while CD117, α-SMA, MSA, desmin, Mac387, CD99, bcl-2, HMB45 and Melain-A were negative. Pathologic evaluation of the resected specimen confirmed the presence of a well-differentiated sclerosing liposarcoma of the upper part of the esophagus. The resected margin contained tumor cells.

Postoperation

Although the resection margin contained tumor cells, the patient did not receive any additional therapy such as radiotherapy or chemotherapy because of the low local recurrence and metastases rate. The patient was followed up for 36 months and no sign of recurrence after operation was found.

Discussion

Liposarcomas are the most common soft tissue tumors, but primary esophageal liposarcoma is extremely rare. Clinical symptoms are suggestive but nonspecific. CT or MRI shows an area with low density (fat tissue) and usually provides a clue for the diagnosis, as we found in our patient. Pathology findings are not uniform. Lipoma-like, sclerosing, and inflammatory subtypes, myxoid and/or round cell, and pleomorphic liposarcomas can all be found in esophageal liposarcoma [14]. Our case belongs to the well-differentiated lipoma-like tumor class.

Because of the low recurrence rate and nonmetastasis of esophageal liposarcoma [14], the treatment consists mostly of simple curettage with an excellent prognosis. In our case the patient was scheduled for total esophagectomy or total laryngectomy before surgery. Intraoperatively we found the border of the tumor was sharp with a polyp protrusion into the esophageal lumen and not adhering to the surroundings, so simple excision was performed. Postoperative follow-up was for 36 months and no signs of recurrence were seen.

The reported 18 cases and ours are summarized in the Table 1. The age of the patients ranged from 42 to 73 years. The type of lesion was mainly polyp (18 cases); only one case was transmural. Most esophageal liposarcomas (15 cases) were located in the cervical portion of the esophagus, and resected by polypectomy (12 cases). The tumor sizes ranged from 4 to 23 cm in length. Based on reviewing the reported cases, we suggest that the cervical approach may be considered optimal if the base of the tumor is located in the cervical portion of the esophagus, irrespective of the tumor size. In the previous 18 cases, two cases recurred after 6.5 and 25 years, respectively, with no documented metastasis; thus, long-term follow-up is important.
Table 1

Review of the literature for esophageal liposarcomaa

Source

Age (yr)/Sex

Type of lesion

Tumor size (cm)

Location

Diagnosis

Treatment

Follow-up

Mansour et al. [1]

53/M

Polyp

4

CE

ML

P

12 mo DF

Bak et al. [2]

49/F

Polyp

20

CE

WDL

TE

7mo DF

Yates et al. [3]

49/M

Polyp

NA

CE

ML

P

6.5 y R

Baca et al. [4]

66/F

Polyp

12

CE

ML

P

30 mo DF

Masumori et al. [5]

46/F

Polyp

11

CE

WDL

P

24 mo DF

Cooper et al. [6]

68/M

Polyp

7

DE

ML

SE

12 mo DF

Boggi et al. [7]

50/M

Polyp

NA

CE

ML

P/TGR

12 mo DF

Salis et al. [8]

73/M

Polyp

15

CE

WDL

P

NA

Mandell et al. [9]

62/F

Polyp

9

CE

WDL

P/PH

4mo DF

Rupper et al. [10]

72/F

Polyp

23

CE

WDL

P

NA

Beaudoin et al. [11]

68/F

Polyp

8.5

CE

WDL

P

25 y R

Chung et al. [12]

56/M

Polyp

21

CE

WDL

TE

NA

Brehant et al. [13]

70/M

Polyp

20

CE

WDL

P

16mo DF

Garcia et al. [14]

42/M

Transmural

10.5

DE

PL

TE

2mo DF

Liakakos et al. [15]

72/M

Polyp

5

DE

WDL

P

6mo DF

Yang et al. [16]

49/M

Polyp

8

CE

WDL

TE

NA

Di Mascio et al. [17]

44/M

Polyp

NA

DE

WDL

P

36mo DF

Maruyama et al. [18]

50/M

Polyp

18.5

CE

WDL

P

NA

Current case

50/M

Polyp

11

CE

WDL

P

36mo DF

Thirteen cases were subclassified as well differentiated and 5 were myxoid; only one case was pleomorphic. Unfortunately, microscopic illustrations were not shown in 5 cases; however, the descriptions were consistent with the diagnoses as well differentiated. The immunohistochemical profile was reported in 6 cases. Protein S100 was positive in well-differentiated liposarcoma and some lipoblasts, while CD34 was positive in spindle cells, the same as liposarcomas arising in other areas.

Copyright information

© Springer Science+Business Media, LLC 2007