CASE REPORTS

Pseudosarcoma of the Esophagus R. W. Postlethwait, M.D., Andrew S. Wechsler, M.D., and John D. Shelburne, M.D. ABSTRACT Polypoid lesions of the esophagus occur infrequently and may be benign or malignant. One malignant polypoid tumor, pseudosarcoma, appears to be a distinct pathological entity; the polypoid portion is composed of sarcomatous spindle cells and the base of the polyp shows in situ or invasive squamous cell carcinoma. A patient with this lesion is described and the 16 previously reported patients are reviewed. The results indicate that local resection is inadequate; radical esophagectomy is required for cure.

P

olypoid lesions of the esophagus are not common but appear as a group of tumors of several histological varieties. The usual benign polyp is composed of fibrous and fatty tissue. Leiomyomas rarely assume a polypoid configuration. Of the malignant tumors, squamous cell carcinomas are polypoid occasionally and adenocarcinomas frequently. Among the rare lesions, leiomyosarcoma and melanoma may be polypoid. Most of the carcinosarcomas and all of the pseudosarcomas reported have been polypoid growths. Pseudosarcoma appears to be a distinct pathological entity, and since recognition of the pathological characteristics is important in planning proper treatment, a description of our patient and review of the 16 previously reported cases seems appropriate. A 53-year-old man was admitted to the hospital on February 21, 1973, because of progressive dysphagia for eight months. His intake was limited to liquids, and even these caused dull, aching, midsternal pain. He had lost 15 pounds. Physical examination was unremarkable. The hematocrit was 37 yo, hemoglobin 12.5 gm per 100 ml, and white cell count 14,300. All other From the Departments of Surgery, Veterans Administration Hospital and Duke University Medical Center, Durham, N.C. Accepted for publication Aug. 7, 1974. Address reprint requests to Dr. Postlethwait, Veterans Administration Hospital, Fulton St. and Erwin Rd., Durham, N.C. 27705.

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CASE REPORT:

Pseudosarcoma of the Esophagus

FIG. 1. Barium swallow shows large polypoid lesion in middle of thoracic esophagus.

laboratory studies were normal. Barium swallow examination revealed a polypoid lesion in the esophagus (Fig. 1). Esophagoscopy showed a bulky necrotic lesion 24 cm from the incisors. A biopsy was taken, and the pathologist’s impression was “undifferentiated tumor, possibly a spindle cell variant of squamous cell carcinoma.” Abdominal exploration showed no metastasis. The stomach was mobilized and a pyloroplasty performed, followed by right thoracotomy. The base of the tumor was at the level of the azygos vein. Dissection of the entire thoracic portion of the esophagus failed to demonstrate gross tumor through the muscularis propria or any abnormal nodes. Resection was followed by anastomosis of the stomach to the esophagus at the thoracic inlet. The postoperative course was uneventful, and the patient has remained well for the subsequent two years. Gross specimens (Fig. 2) showed a necrotic, grayish polypoid lesion that was foul smelling and was attached by a pedicle about 1.5 cm in diameter. Microscopically the base of the polyp showed typical squamous cell carcinoma extending into the submucosa but not into the muscularis propria. The narrow zone of carcinoma abruptly changed into the

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POSTLETHWAIT, WECHSLER, AND SHELBURNE

FIG. 2. Gross specimens of resected esophagus. T h e pedicle is better seen with the polyp elevated (right).

sarcomatous-appearing tissue that comprised the polyp (Fig. 3). These cells were pleomorphic, being mainly spindle shaped and arranged in whorls. T h e highly cellular pattern showed many mitoses and some multinucleated cells. T h e diagnosis was pseudosarcoma.

Pathology T h e pathological findings in our patient duplicated the first 2 cases described by Stout and Lattes [I51 in 1957. Tumors from subsequently reported patients have shown some variations but the basic histological pattern has remained the same, i.e., intramucosal or occasionally invasive squamous cell carcinoma at the base of the polyp and abrupt change (without intermingling) to the sarcomalike tissue making up the polyp. As well as can be determined, 8 of the 17 patients with pseudosarcoma had intraepithelial squamous cell carcinoma at the base of the polyp. T h e patient of Hay-Roe and associates [6] had local excision with recurrence and resection thirteen months later. T h e second specimen showed two polyps, one a pseudosarcoma and the other a typical squamous carcinoma. T h e first patient of Fraser and Kinley [4] had three pseudosarcomatous polyps with invasive carcinoma at the base but also a small area of invasive carcinoma between two of the polyps. Sanchez and colleagues [12] reported a somewhat similar patient: near the base of the polyp were four small

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CASE REPORT:

Pseudosarcoma of the Esophagus

superficial ulcerations that were found to be invasive carcinoma. The first patient of Akiyama [l] was actually operated upon for a typical squamous carcinoma of the distal third of the esophagus. The small pseudosarcoma was found proximal to this lesion. Stout and Lattes [15] stated that if metastases occur from a pseudosarcoma, the metastatic deposits are squamous and not sarcomatous. The patient described by Hughes and Cruickshank [7], however, differed somewhat. After local removal of the pseudosarcoma, recurrence six months later necessitated resection. This proved to be, again, typical pseudosarcoma with intraepithelial carcinoma. Another recurrence fourteen months after the second operation was unresectable. Postmortem examination showed a large mass on the gastric side of the anastomosis which was composed of the sarcomatous element. The similarity of pseudosarcoma of the esophagus to pseudosarcomas occurring in the mouth, fauces, and larynx, as originally described by Lane [8], has been noted repeatedly. The question of the cell of origin of the pseudosarcomatous tissue has apparently been settled by Lichtiger, Mackay, and Tessmer [9] based on their study of thirteen such tumors arising in the upper respiratory tract, oral cavity, and skin of the face. Electron microscopy demonstrated the presence in many tumor cells of aggregates of keratohyalin, slender bundles of tonofilaments, and occasional premelanosomes. Desmosomes were present but were few in number and poorly developed. They concluded that these findings do not support a connective tissue origin but justify the designation of spindle cell variant of squamous carcinoma.

FIG. 3. Light microscopy shows ( A ) junction of squamous carcinoma with pseudosarcomatous tissue and ( B ) pattern of pseudosarcoma. (HSE; A ~ 4 0 B , ~ 1 0 0 ;both reduced 45% for reproduction.)

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POSTLETHWAIT, WECHSLER, AND SHELBURNE Shields, Eilert, and Battifora [14] examined their specimen of pseudosarcoma of the esophagus by electron microscopy. They found desmosomes and tonofibrils, which they stated indicate the squamous origin. The specimen from our patient likewise was examined with electron microscopy. Small cubes of tumor tissue (1 mm) were rehydrated from paraffin blocks following routine formalin fixation and paraffin embedding. T h e tissue was fixed in 0.1 M cacodylate-buffered 4y0 glutaraldehyde, washed in the same buffer, postfixed in 1% osmium tetroxide in the same buffer, and embedded in Epon. Thin sections were examined on a Jeolco lOOB and a Hitachi HS8 electron microscope at 60 and 50 kilovolts, respectively. T h e demonstration of desmosomes and tonofibrils is shown in Figure 4. Carcinosarcoma of the esophagus, reviewed by Moore and associates [lo], differs from pseudosarcoma in that the carcinosarcoma shows intermingling of the two cell types. In addition, many of the more anaplastic squamous carcinomas of the esophagus feature microscopical areas that appear sarcomatous. Finally, inflammation of a squamous carcinoma may cause the occurrence of sarcomalike areas.

Diagnosis Age, sex, and site, as shown in the Table, do not aid in diagnosis. T h e age range is from 49 to 80 years. Three of the 17 patients were women. One lesion was in the cervical region; the others were about equally distributed in the thoracic part of the esophagus. Symptoms were essentially those of esophageal obstruction. T h e roentgenographic finding was that of a polypoid lesion without distinguishing characteristics. T h e exact diagnosis of pseudosarcoma obviously depends on the histological findings. Any polypoid lesions of the esophagus found on endoscopy should be suspect. Biopsy of the mass demonstrating the spindle cell variation with a second biopsy at the attachment of the pedicle showing squamous carcinoma would represent reasonably good evidence, but not necessarily proof, for the diagnosis of pseudosarcoma.

Treatment Local excision was performed in 4 of the 17 patients but was invariably followed by recurrence. Theoretically, a polypoid lesion with a small area of intraepithelial carcinoma should be curable by local excision. Unfortunately, invasive carcinoma both at the base of the pedicle and within the vicinity was found in some of the patients. Therefore, as extensive a resection as that performed for any squamous cell carcinoma of the esophagus should be performed at the original operation. All the long-term survivors were treated in this manner. As indicated in the Table, 9 patients had resection with 2 postoperative deaths. Two died more than six years

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CASE REPORT:

Pseu.dosarcoma of the Esophagus

FIG. 4. By electron microscopy, tonofibril aggregates are seen in the peripheral cytoplasm (arrows, A and B). These are examined at higher power in D. A t the top arrow in B the cytoplasm projects outward, resembling the processes seen between normal squamous cells (Shields, Eilert, and Battifora [14]). T w o desmosornes ( D ) are present in B. At higher power in C a central dense lamina is seen in another desmosome (D). ( N = nucleus; RER = rough endoplasmic reticulum.) ( A and B ~ 8 , 0 0 0 ,C ~ 1 9 , 2 5 0 , D ~30,000.) VOL. 19, NO. 2, FEBRUARY,

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KI

.e

56

E

L.

2

E! n

2

8

'

t:

E 9 3k-

4

B

2 polyps, 5 x 3 ~ 2 5 x 4.5 x 1.8 8 x 7 ~ 3 6 x 3 ~ 2 3.5 x 2.5 5.6 x 2.7 x 2.1 7x2 0.8 x 0.8 1 0 x 4 ~ 3 9X4X3 6x3

Lower 1/3 Middle 113 Lower 1/3 Middle 113 Junction mid-low 1/3 Middle 1/3 Cervical Lower 1/3 Middle 113 Lower 1/3 Middle 1/3

7. 63, M

8. 67, M

"Follow-up obtained by personal communication in 1973.

Sanchez [12], 1973 Postlethwait, 1974

10. 70, 11. 63, 12. 71, 13. 49, 14. 52, 15. 58, 16. 51, 17. 53,

Razzuk [ll], 1971a Shields [14], 1972 Ennle [3], 1973 Goodner, 19738 Akiyama [l], 1973a

M M M F M M F M

9. 57, M

Hughes [7], 1969

Sasano [IS], 196Sa

Local excision; resection 6 mo later Local excision Resection Resection None Palliative resection Resection Resection Resection

Resection

None

1 yr 2 yr 17 mo 2 mo 7 mo Well 8 mo Well 2 yr

Died Well Well Died Died

Died 6 yr, not carcinoma Postmortem finding Died 6 yr, not carcinoma Died 15 mo Resection

At thoracic inlet

6. 75, M

Local excision Resection

7 x 2.5 x 2.5 3 polyps, largest 10 x ? 7x?

Middle 1/3 Middle l j 3

4. 59, M 5. 60, M

Died after second resection Died 18 mo Postop. death

?

Postop. death

Result

Local excision

?

Resection

Treatment

lox?

DeMarco [2], 1965 Fraser [4], 196Sa

? ?

Size (cm)

Middle 113

?

Lower 1/3

Site of Lesion

Hay-Roe [6], 1960

Patient No., Age, & Sex

1. 80, F 2. 70, M 3. 50, M

Stout [15], 1957

Senior Author & Year

SUMMARY OF REPORTED CASES OF PSEUDOSARCOMA

CASE REPORT:

Pseudosarcoma of the Esophagus

after operation of other disease. One is living a t more than five years a n d the remaining 4 for shorter intervals.

References 1. Akiyama, H.

2. 3. 4. 5. 6. 7. 8.

9. 10. 11. 12. 13. 14. 15.

Pseudosarcoma of the esophagus. J J a p Bronchoesoph SOC 25:14, 1973. DeMarco, A. R., Leon, W., Coleman, W. O., Welsh, R. A., and Strug, L. H. Pseudosarcoma of the esophagus. J Thorac Cardiouasc Surg 49: 188, 1965. Enrile, F. T., DeJesus, P. O., Bakst, A. A., and Baluyot, R. Pseudosarcoma of the esophagus: Polypoid carcinoma of esophagus with pseudosarcomatous features. Cancer 31: 1197, 1973. Fraser, G. M., and Kinley, C. E. Pseudosarcoma with carcinoma of the esophagus: A report of two cases. Arch Pathol85:325, 1968. Goodner, J. T. Sarcoma of the esophagus. A m J Roentgen01 R a d i u m T h e r N u c l M e d 89: 132, 1963. Hay-Roe, V., Hill, R. L., and Civin, W. H. An unclassifiable tumor of the esophagus: A case report. J Thorac Cardiouasc Surg 40: 107, 1960. Hughes, J. H., and Cruickshank, A. H. Pseudosarcoma of the oesophagus. Br J Surg 56:72, 1969. Lane, N. Pseudosarcoma (polypoid sarcoma-like masses) associated with squamous cell carcinoma of the mouth, fauces, and larynx: Report of ten cases. Cancer 10:19, 1957. Lichtiger, B., Mackay, F., and Tessmer, C. F. Spindle-cell variant of squamous carcinoma: A light and electron microscopic study of 113 cases. Cancer 26: 1311, 1970. Moore, T. C., Battersby, J. A., Vellios, F., and Loeher, W. M. Carcinosarcoma of the esophagus. J Thorac Cardiovasc Surg 45:281, 1963. Razzuk, M. A., Urschel, H. C., Jr., Race, G. J., Nathan, M. J., and Paulson, D. L. Pseudosarcoma of the esophagus. J Thorac Cardiouasc Surg 61:650, 1971. Sanchez, R. A., Shah, I. C., Barman, A., Batiuchok, W., and Mule, J. E. Pseudosarcoma of the esophagus: A case report. J Thorac Cardiouasc Surg 66:833, 1973. Sasano, N., Takahashi, K., Takezawa, Y., Masuda, H., Kaneko, Y., and Tajima, T. Pseudosarcoma with polypous carcinoma of the esophagus. J a p J Ca Clin 14:973, 1968. Shields, T . W., Eilert, . B., and Battifora, H. Pseudosarcoma of the oesophagus. T h o r a x 27:4 2, 1972. Stout, A. P., and Lattes, A. Tumors of the Esophagus. I n Atlas of T u m o r Pathology, Sect V, Fasc 20. Washington, l3.C.: Armed Forces Institute of Pathology, 1957.

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Pseudosarcoma of the esophagus.

Polypoid lesions of the esophagus occur infrequently and may be benign or malignant. One malignant polypoid tumor, pseudosarcoma, appears to be a dist...
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