PSEUDOSARCOMA OF THE ESOPHAGUS

PSEUDOSARCOMA OF THE ESOPHAGUS

PSEUDOSARCOMA OF THE ESOPHAGUS Arnold R. DeMarco, M.D., William Leon, M.D., Warren 0. Coleman, Ronald A. Welsh, M.D.* M M.D., and Lawrence H. Strug...

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PSEUDOSARCOMA OF THE ESOPHAGUS Arnold R. DeMarco, M.D., William Leon, M.D., Warren 0. Coleman, Ronald A. Welsh, M.D.*

M

M.D.,

and Lawrence H. Strug, M.D., New Orleans, La.

lesions of the esophagus other than frank epidermoid and adenoeareinoma are remarkably uncommon. A particularly unique tumor in this respect is the pseudosarcoma of the esophagus described by Stout in 1957.10 Since this original description of the lesion, based on Stout's pathologic ex­ amination of tissue from 2 cases, only 1 additional case has been reported in the esophagus.3 It is our purpose, therefore, to add our recent experience with this tumor to the literature on the subject and to review briefly the pathologic features and what is known concerning the biological behavior of this tumor. ALIGNANT

CASE REPORT E. J., a 59-year-old Negro man, was admitted to the L.S.U. Thoracic Surgical Service at Charity Hospital on May 27, 1963. Prior to this, the patient was hospitalized at Greenwell Springs Tuberculosis Sanitorium with a diagnosis of far-advanced pulmonary tubercu­ losis and had achieved sputum conversion under therapy. Approximately 2 months prior to admission to our service he began to complain of dysphagia which was insidious in onset, painless, and progressive and culminated in the patient's inability to swallow solid foods. At the time of his admission there was an estimated twenty pound weight loss. He denied symptoms of vomiting, hematemesis, or melena, but a subtotal gastric resection had been performed for chronic duodenal ulcer in 1958. Physical findings were limited essentially to pallor of the mucous membranes and evidence of recent weight loss. Laboratory studies revealed a microcytic anemia (packed cell volume, 25 per cent) but a normal total blood volume. Stool specimens were consistently positive for occult blood. Eoentgenographic examination of the chest revealed bilateral fibronodular apical infiltrates and a small cavitary lesion of the left upper lobe consistent with the patient's previous diag­ nosis of pulmonary tuberculosis. Pulmonary ventilation studies revealed moderate obstructive dysfunction. An esophagram performed on the day following admission (Pig. 1) revealed the presence of a large ovoid filling defect in the mid-thoracic esophagus with partial ob­ struction, but no apparent mucosal destruction. Endoseopic examination was performed 7 days after admission. Bronchoscopy revealed no endobronchial disease and no evidence of external compression on the trachea or major bronchi. A 53 cm. Jessberg esophagoscope was then inserted into the esophagus without diffi­ culty and passed to the esophagogastric junction at 40 cm. Surprisingly, no abnormalities were noted. However, upon withdrawal of the instrument an intraluminal mass was ob­ served at 25 cm. from the incisor teeth. The tumor appeared to have a complete mucosal Prom the Department of Thoracic Surgery, Louisiana State University School of Medicine, and Charity Hospital of Louisiana at New Orleans, New Orleans, La. Received for publication May 14, 1964. ♦Department of Pathology, Louisiana State University School of Medicine, New Orleans, La. 188

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covering and was mobile. Biopsies taken from this lesion were reported as "sarcoma of the esophagus, type undetermined." On J u n e 17, 1963, after adequate preparation, including transfusion with 2,000 c.c. of blood, the patient underwent operation. A right posterolateral thoracotomy was per­ formed, with the chest entered through the sixth intercostal space. Exposure of the esophagus revealed a visible bulge adjacent to the right main bronchus and a firm, ovoid, mobile tumor was easily palpable through the intact wall. After adequate mobilization of the involved segment of the esophagus, a vertical esophagotomy was performed near the area that was

Fig-. 1.—Preoperative esophagram shows an intraluminal filling defect.

considered the origin of the tumor pedicle. Examination revealed a tumor much larger than had been anticipated, which arose from the posterior wall of the esophagus by a narrow pedicle. Eesection was accomplished by removing the full thickness of that portion of the wall from which the tumor originated in a longitudinal elliptical fashion. Frozen section ex­ amination of the pedicle showed it to be free of tumor. Because the lumen of the esophagus was somewhat dilated a t the resection site, it was possible to perform primary suture of the esophagotomy without compromising the lumen. The right pleural space was drained by a single intercostal catheter and the thoracotomy wound was closed in the usual manner. Convalescence was rapid and uneventful. The thoracotomy tube was removed in 48 hours and oral intake of liquids was permitted on the fifth postoperative day. By the eighth day the patient was taking a normal diet without difficulty and has had no subsequent com-

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Pig. 2.—Uncut specimen. The area noted as O is the approximate site of the intraepithelial carcinoma. The arrow points to the junction of the epithelium with the polypoid tumor mass which was devoid of epithelial covering. Fig. 3.—Cut section of pseudosarcomatous tumor mass. plaints of dysphagia. Esophagrams obtained 2 weeks, 3 months, and 7 months following surgery showed some narrowing of the mid-thoracic esophagus, but no obstruction or mueosal changes. During the period of convalescence, we received the final pathological report with the rather unexpected diagnosis of pseudosarcoma. Serious consideration was given to re-explora­ tion and resection of the involved area of the esophagus. However, our decision was to ob­ serve the patient carefully and frequently until other findings compelled a different course of action. Esophagoseopy was performed 3 months and 7 months postoperatively and nothing suggestive of recurrence of tumor was noted. In September, the patient was discharged and returned to the referring hospital for further treatment of his tuberculosis. Pahological Description: S.N. 63-7177L.—The specimen was an ovoid polypoid mass, measuring 7.0 by 2.5 by 2.5 cm., attached by a broad base to an elliptical segment of esophageal wall which measured 3.0 by 2.5 cm. The polyp itself was devoid of epithelial cover­ ing, with the esophageal epithelium beginning around the base of the polyp (Fig. 2). The

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epithelium covering the attached esophageal wall was pearly white and thickened on one side of the specimen. The cut surface of the polypoid mass was a homogeneous, whitish gray tissue of a soft, fleshy consistency (Fig. 3 ) . Microscopically, the polyp was composed of bizarre spindle cells and large giant cells often separated by an abundant intercellular fibrous stroma (Fig. 4). Nuclear anaplasia was marked, and abnormal mitotic figures were numerous. The histological picture was distinctly that of a sarcoma, and there was no evidence of any epithelial pattern of growth of the cells. Although occasionally the cytoplasm of the cells was strap-like, no cross striations were demonstrated after extensive search. Although giant cells and spindle cells dominated the pattern, there were no typical lipoblasts or lipid forma­ tions suggestive of liposarcoma. In the esophageal epithelium of the adjacent wall of one side of the specimen, there was marked hyperplasia with anaplasia of the squamous cells (Fig. 5). These cells showed lack of normal maturation to the surface, loss of polarity, irregu­ larity of nuclear size and staining intensity, and frequent mitoses, some of which were ab­ normal or tripolar. The basement membrane was intact and there was no invasion. In par­ ticular, this area of intraepithelial carcinoma was separated by a narrow zone of benign epithelium from the adjacent polypoid sarcoma, and there was no evidence of transition be­ tween the two. Final pathological diagnosis was, "Intraepithelial carcinoma in pedicle ad­ joining polypoid sarcoma of indeterminate type; the lesion resembles published reports of pseudosarcoma."

Fig. 4.—An area of tumor shows bizarre giant forms, marked anaplasia, abnormal mitotic activity, and an increase in intercellular stroma (X200; reduced Ms). Fig. 5.—Intraepithelial carcinoma in the base of the pedicle. There is complete loss of nuclear polarity. Mitotic figures are present through all levels. In this particular field there is a slight tendency toward maturation at the surface, but other areas showed complete lack of maturation (X200; reduced % ) . DISCUSSION

As' previously mentioned, Stout first described this strange tumor in 1957 as being polypoid, projecting into the lumen of the esophagus, and having a mucosal covering. Microscopically, the pedunculated part is composed of cellu­ lar agglomerations suggestive of various sarcomas. However, there also is an intramucosal, occasionally invasive, carcinoma in the vicinity of the pedicle.

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Lane, 5 in 1957, reported 10 cases of pseudosarcoma of the mouth, fauces, and larynx. He described them as being polypoid, "non-neoplastic connective tissue masses with a histologic picture that is at once bizarre, alarming and deceivingly suggestive of some truly malignant form of sarcoma capable of metastases." All of these were intimately associated with a usually inconspicu­ ous squamous cell carcinoma. In differentiating these from earcinosarcoma, the junction between carcinomatous and sarcomatous elements must be critically studied. In earcinosarcoma there is an extensive and intimate intermingling of these two elements1' "■ 7 ' 12 whereas in pseudosarcoma there is merely side by side positioning. The possibility that these are bizarre morphologic variants of carcinomas is very unlikely, but the evidence is suggestive rather than con­ clusive. The general appearance is nonepithelial; the associated carcinoma is well differentiated rather than highly anaplastic; the squamous cell carcinoma is sharply demarcated from the pseudosarcomatous tissue and reticulum stains show that in all areas the cells are enveloped by fibers. Lane postulates that these tumors start as an intramucosal carcinoma in tissue which has a loose areolar structure. This supporting tissue allows a secondary influx of fluid and cells which causes the polypoid form. The bizarre cellular proliferation is sec­ ondary to some local mechanism. In view of this feeling and in view of the fact that in none of the reported eases has the sarcomatous element been known to metastasize, Lane states that these cannot be interpreted as so-called "collision : tumors.'' Shirwin and associates9 reported 3 more cases which occurred in the oral pharynx. They contend that the polypoid nature is secondary to mucosal ulceration and granulation tissue overgrowth. The spindle cells originate from the carcinomatous epithelium and their proliferation, in conjunction with the granulation tissue, forms the stroma of the polyp. Hay-Roe and colleagues3 reported a case of pseudosarcoma involving the esophagus. They performed tissue cultures of the tumor and were able to propa­ gate both polygonal and spindle cells, depending on the site of the tissue. The ability of a tumor to have autonomous growth indicates malignancy to many investigators. Spindling is probably derived from the epithelial elements in the tumor. In all of the previously reported pseudosarcomas, regardless of their loca­ tion, emphasis is made that only the carcinomatous elements tend to metastasize, not the stromal or sarcomatous elements; in earcinosarcoma the sarcomatous elements do metastasize.2 In Hay-Roe's case, operation more than one year after the original diagnosis revealed no metastases. Saphir and Vass,8 in reviewing the literature of earcinosarcoma up to 1938, found 153 cases of which 15 were in the upper gastrointestinal tract (esophagus, 14; hypopharynx, 1), and 13 in the respiratory tract (larynx, 6; lung, 7). In these locations the lesions were most often polypoid with a small carcinoma in the base. The greater portion of the mass was "sarcomatous" with pleomorphic bizarre cells, including giant forms. According to these authors, the metastatic rate of the so-called "sar­ comatous" elements was amazingly low. In Lane's cases the survival rate seems to be directly related to the stage of the carcinoma, rather than to any morpho-

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logic features of the sarcoma. The 4 survivors had a minute proportion of car­ cinoma to pseudosarcoma (all except one being entirely intramucosal) but did well in spite of an inadequate resection for a true sarcoma. Both Lane 5 and Stout11 believe that a number of the carcinosarcomas*' 13 reported in the litera­ ture which showed no metastases of stromal elements may have been pseudosarcomas. SUMMARY

One case of pseudosarcoma of the esophagus, including a review of the literature, is reported. A short discussion of the known biologic characteristics of these tumors is presented, but no attempt is made to argue the nature of these lesions. The important point is that polypoid lesions of the esophagus should alert the surgeon to the possibility of a pseudosarcoma. Therefore, if a lesion appears sarcoma-like (anaplastic with bizarre tumor giant cells and suggests either liposarcoma or rhabdomyosarcoma), a careful search must be made for an associated squamous cell carcinoma in the base of the tumor. REFERENCES

1. Elton, S. E., and Joannides, M.: Careinosarcoma of t h e Esophagus, Dis. Chest 4 1 : 111, 1962. 2. Gerald, B . E . : Careinosarcoma of t h e Esophagus, Am. J . Gastroenterol. 3 : 417, 1961. 3. Hay-Roe, V., Hill, R. L., a n d Civin, W. H . : An Unelassifiable Tumor of the Esophagus, J . THORACIC & CARDIOVAS. SURG. 40: 107, 1960.

4. King, H . A., a n d Koerner, T. A.: Careinosarcoma of t h e Esophagus, J . THORACIC & CARDIOVAS. SURG. 42: 389, 1957.

5. Lane, N . : Pseudosarcoma Associated W i t h Squamous Cell Carcinoma of t h e Mouth, Fauces and Larynx,'Cancer 10: 19, 1957. 6. Moore, T. C , Battersby, J . S., Vellios, F . , a n d Loehr, W. M.: Careinosarcoma of t h e Esophagus, J . THORACIC & CARDIOVAS. SURG. 4 5 : 281, 1963.

7. Pearlman, S. J . : So-Called Careinosarcoma of t h e Esophagus, Ann. Otol. Rhinol. & Laryngol. 49: 805, 1940. 8. Saphir, O., a n d Vass, A.: Careinosarcoma, Am. J . Cancer 33: 331-361, 1938. 9. Shirwin, R. P . , Strong, M. S., a n d Vaughn, C. W . : Polypoid a n d Junctional Squamous Cell Carcinoma of t h e Tongue and L a r y n x With Spindle Cell Sarcoma (Pseudosarcome), Cancer 16: 51, 1963. 10. Stout, A. P., a n d L a t t e s , R.: Atlas of Tumor Pathology, 1957, Section 5, Faseiele 20, pp. 83-103. 11. Stout, A. P . , Humphreys, G. H., and Rottenberg, L. A.: A Case of Careinosarcoma of t h e Esophagus, Am. J . Roentgenol. 6 1 : 461, 1949. 12. Talbert, J . L., Cantrell, J . R., a n d Blalock, A.: Clinical and Pathologic Characteristics of Careinosarcoma of t h e Esophagus, J . THORACIC & CARDIOVAS. SURG. 4 5 : 1, 1963. 13. Thompson, J . R.: Careinosarcoma of t h e Esophagus, J . THORACIC SURG. 2 5 : 261, 1953.