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Polypoid Sarcomas of the Esophagus
Polypoid Sarcomas of the Esophagus A Rare but Potentially Curable Neoplasm Tom R. DeMeester, M.D., a n d David B. Skinner, M.D. ABSTRACT Five patients with polypoid esophageal sarcoma are reported. All had dysphagia similar to that occurring in epithelial carcinoma of the esophagus. The clinical diagnosis of sarcoma was suspected when barium swallow showed a large polypoid lesion. Biopsy was often inconclusive concerning the nature of the lesion except to identify it as a neoplastic process. In spite of their large size, the tumors remained superficial within the esophageal wall. Nodal or distant organ metastasis was absent in 4 of the 5 patients. The histology of these tumors suggests that so-calledcarcinosarcoma is an epithelial carcinoma of the esophagus with varying amounts of spindle cell features and should be considered separately from sarcoma arising from mesenchymal tissue. Unlike carcinoma, esophageal sarcoma has a favorable prognosis following radical resection, and recent advancesin esophageal surgery have decreasedthe mortality and morbidity of esophagectomy.
S
arcomas - and carcinosarcomas, which are included with them - are rare neoplasms accounting for approximately 0.1 to 1.5% of all esophageal tumors [ 151. The rarity of these lesions is illustrated by the small number of previous reports, consistingof single or small groups of patients. Despite their infrequent occurrence they exhibit several clinically important behavior patterns, which are illustrated by the 5 patients reported in this study.
Clinical Material PATIENT 1
A 57-year-old white man was referred to the University of Chicago Hospitals with a nine-month history of dysphagia, three pound weight loss, and four weeks of tarry stools. Barium swallow showed a 9 cm polypoid intraluminal lesion in the upper third of the esophagus (Fig. 1A). A biopsy obtained by esophagoscopy was reported as several pieces of granulation tissue and one fragment suggestive but not diagnostic of carcinoma. Esophagectomy with esophagogastrostomy was performed through a left posterolateral thoracotomy incision. The esophagus was divided 3 cm below the apex of the chest at a point approximately 2 cm above the tumor. The surgical specimen contained an 8 cm polypoid tumor of the esophagus with necrosis and hemorrhagic changes on its surface. The tumor was noted not to extend into the muscularis propria, and there were adequate peripheral margins of uninvolved tissue in all planes. The polyp was composed of small nests of From the Department of Surgery, University of Chicago Pritzker School of Medicine, Chicago, Ill. Presented at the Eleventh Annual Meeting of The Society of Thoracic Surgeons, Montreal, Que., Canada, Jan. 20-22, 1975. Address reprint requests to Dr. DeMeester, Department of Surgery, Section of Thoracic and Vascular Surgery, University of Chicago Pritzker School of Medicine, 950 E. 59th St., Chicago, Ill. 60637.
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FIG. 1 . (Patient I .) ( A ) Barium swallow demonstrating a polypoid lesion in the upper third of the esophagus. ( B ) Photomicrograph of the polyp shows distinct nests of squamous eplthelium surrounded by interlacing bundles of spindle-shaped cells. (HWE; origznal magnification X 150.)
squamous epithelium surrounded by interlacing bundles of spindle-shaped cells with numerous mitoses and occasional atypical giant cells (Fig. 1B). Regional lymph nodes were free of tumor. The final histological diagnosis was carcinosarcoma of the esophagus composed of squamous cell carcinoma and undifferentiated spindle cell sarcoma. On the tenth postoperative day the patient died suddenly following an episode of severe aspiration. Postmortem examination showed that the small bronchi throughout the lung contained stomach contents. There was no evidence of lymphatic or hematogenous tumor metastasis. PATIENT 2
An 83-year-old white man was referred to the University of Chicago Hospitals with a six-week history of dysphagia. A left supraclavicular lymph node was palpable. Barium swallow showed a 9 cm polypoid intraluminal lesion in the middle third of the esophagus (Fig. 2A). Biopsy by esophagoscopy was reported as demonstrating carcinoma. Biopsy of the supraclavicular lymph node showed it to be completely replaced with large hyperchromatic pleomorphic cells, representing an undifferentiated neoplasm suggestive of carcinoma. Esophagectomy with esophagogastrostomy was performed through a left posterolateral thoracotomy. T h e esophagus was divided at the arch of the aorta 2 cm above the palpable tumor. T h e surgical specimen (Fig. 2B) contained a 7 cm polypoid tumor of the esophagus. T h e tumor was noted not to extend into the muscularis propria, and 406
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Polypoid Sarcomas of the Esophagus
FIG. 2. (Patzent 2 . ) ( A ) Spotjzlmjrom barzum swallow showing a polypoid lesion in the middle third of the esophagus. (Retouched for clarification.) ( B ) Surgical specimen containing a 7 cm polypoid lesion of the esophagus. (C) Photomicrograph of the polyp shows nests of lurge pleomorphic cells closely associated with and surrounded by loose bundles of spindle-shaped cells. (HE3E; original magnzpcation ~ 1 . 5 0 . )
there were adequate peripheral margins of uninvolved tissue in all planes. T h e polyp was composed of large pleomorphic cells with hyperchromatic nuclei showingmany mitotic figures. In certain regions the tumor was traversed with a spindle cell stroma (Fig. 2C). Regional lymph nodes were replaced by metastatic epithelioid cells. T h e final histological diagnosis was atypical polypoid carcinoma of the esophagus with carcinosarcomatous features. In the immediate postoperative period the patient suddenly became cyanotic, with changes in his cardiac rhythm. T h e day after operation he developed ventricular fibrillation from which h e died. On postmortem examination he had a coiled 21 x 2 cm thromboembolus within the right heart, extending through the phlmonary valve into the first 5 cm of the pulmonary artery. T h e tracheobronchial nodes contained metastatic epithelioid tumor. PATIENT 3
A 70-year-old white man was referred to the University of Chicago Hospitals with a one-month history of dysphagia. Barium swallow showed an 8 cm polypoid intraluminal lesion in the lower third of the esophagus. Biopsy by esophagoscopy showed a neoplasm consisting of spindle cells with pleomorphic nuclei and numerous mitotic figures. Nowhere within the specimen was there any evidence of esophageal epithelium. T h e histological diagnosis was sarcoma of the esophagus. Esophagectomy with esophagogastrostomy was performed through a left posterolateral thoracotomy. T h e esophagus was divided just below the aortic arch at a point 2 cm above the palpable tumor. T h e surgical specimen (Fig. 3A) included a 5 cm polypoid tumor of the VOL. 20, NO. 4, OCTOBER, 1975
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FIG. 3 . (Patient 3.) ( A ) Surpcal specimen shows a 5 cm polypoid tumor protrudingf r o m the end o j tht resected e.~ophngus. ( B ) Photomicrograph of the polyp reveals nests of squamous epthelium surrounded by interlacing bundles of spindle-shaped cells. An area of transition f r o m ordinary epdermoid carcinoma to the spindle cell variant is demonstrated i n the upper left-hand corner (arrow). ( H U E ; oripnal magnzfication x 150.)
esophagus. There was no apparent direct tumor invasion of the esophageal wall beneath the base of the tumor. Adequate peripheral margins of uninvolved tissue were present in all planes. The polyp was composed of nests of squamous epithelium surrounded by interlacing bundles of spindle-shaped cells with numerous mitoses and occasional atypical giant cells. In some regions the epithelial cells appeared to fan out and form the spindle-shaped cells of the mesenchymal stroma (Fig. 3B). Regional lymph nodes were free of tumor. T h e final histological diagnosis was carcinosarcoma of the esophagus composed o f squamous cell carcinoma with a sarcomatous stroma. T h e patient was discharged on the twenty-second postoperative day. He remained healthy and free from tumor for thirteen years, eventually dying from bronchial pneumonia. PATIENT 4
A 60-year-old white woman was transferred to the University of Chicago Hospitals with total esophageal obstruction that had persisted for three weeks 408
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FIG. 4 . (Patient 4 . ) ( A ) Barium swallow shows a polypoid mass in the upper thoracic and cervical esophagus. (B) Photomicrograph of the polyp demonstrates large spindte-shaped cells. No carcinoma zuas seeti withiu the sectioxs. (HME; original magnzfication X 150.)
following cervical esophagomyotomy. She had complained of cervical dysphagia and an eighteen-pound weight loss over four months prior to the myotomy. Barium swallow showed a 7 cm polypoid intraluminal mass in the cervical esophagus (Fig. 4A). At esophagoscopy large quantities of necrotic tissue were removed before a biopsy of viable tissue was obtained. T h e biopsy showed spindle cells, neoplastic giant cells, and occasional foci of polyhedral cells suggestive of carcinoma. T h e histological diagnosis was carcinosarcoma. After three weeks of intravenous hyperalimentation and treatment for aspiration pneumonia, a total esophagectomy and en bloc laryngectomy were performed. An isoperistaltic segment of left and transverse colon based on the left colic artery was used to reestablish continuity between the pharynx and stomach. T h e surgical specimen consisted of the entire esophagus with an attached larynx. T h e upper esophagus contained a polypoid tumor measuring 6.5 cm in length that was firmly attached to the anterior and posterior walls of the esophagus, forming two lumens. There was no evidence of tumor penetration through the esophageal wall, and adequate peripheral margins of uninvolved tissue were apparent in all planes. There was no tumor invasion into the tissues between the esophagus and larynx or into the larynx itself. T h e polyp was composed of large spindle-shaped cells with numerous mitoses (Fig. 4B).T h e tumor was well demarcated from surrounding compressed skeletal muscle. No carcinoma was seen within the sections. Myofibrils were demonstrated on electron VOL. 20, NO. 4, OCTOBER, 1975
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DE MEESTER AND SKINNER microscopy, but there were no cross-striations characteristic of skeletal muscle. Regional lymph nodes were free of tumor. The final histological diagnosis was leiomyosarcoma of the cervical esophagus. After a prolonged recovery she was discharged. When evaluated seventeen months after operation she was free from tumor, had gained fifteen pounds, and was beginning attempts at mastering the art of colonic speech. PATIENT 5
A 62-year-oldblack woman was referred to the University of Chicago Hospitals for radiotherapy for a presumed esophageal carcinoma. She gave a history of dysphagia for nine months, sixty-pound weight loss, and one month of respiratory stridor. Two previous esophageal biopsies had revealed only necrotic tissue. Barium swallow showed a 10 cm polypoid mass within the middle third of the esophagus (Fig.5A). Bronchoscopy showed compression of both main bronchi. At esophagoscopy a large quantity of necrotic tissue was removed before a biopsy of bleeding tissue was obtained. The frozen-section diagnosis on this tissue was poorly differentiated squamous cell carcinoma, but on closer inspection of the tissue by electron microscopy it was noted to contain myofibrils, and the diagnosis of leiomyoblastoma of the esophagus was made. An esophagectomy was performed through a right posterolateral thoracotomy. The stomach was mobilized through the esophageal hiatus, and after performing a pyloroplasty, an end-toside esophagogastrostomy was constructed. The surgical specimen (Fig. 5B) contained a 9 cm polypoid tumor within the esophagus. The tumor at its pedicle extended through the muscularis propria into the adventitia, but on microscopical observation there were adequate peripheral margins of uninvolved tissue in all planes. Regional lymph nodes were free of tumor. The polyp was composed largely of necrotic tissue except for its pedicle portion, where there were cells containing eccentric nuclei with cytoplasmic vacuolization (Fig. 5C). There was only scattered evidence of mitosis. The final histological diagnosis was polypoid leiomyoblastoma of the esophagus. On the sixteenth postoperative day the patient was discharged and has remained well in follow-up for fifteen months.
Comment
These 5 patients illustrate a number of factors common to previous reports on the clinical behavior of esophageal sarcoma. An awareness of these factors is helpful in the clinical management of this rare tumor. CLINICAL PRESENTATION
The symptom of dysphagia is due to esophageal obstruction and does not really differ from the dysphagia associated with the more common epithelial carcinoma. Tumors located within the cervical or high thoracic esophagus can cause symptoms of pulmonary aspiration. This occurred in 1 of our patients, in whom the mistaken diagnosis of cricopharyngeal achalasia led to an unnecessary esophageal myotomy and aggravated her symptom of aspiration. Large tumors
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FIG. 5 . (Pntierit 5.) ( A ) Bnriurti .szuiillozo reuerils (1 /iirgepolypviil tnrrss within the muldle third of the e.sophn,gus. ( R ) Sur,giccil specirnen shows ii 9 ctti polypoid turnor within the esophngus with necrosis a d hetniirrhngic chntigu.t on it.s ,c.uifiicu.(C) Phiitr)microgrcipii qf thu peilicle portion of the polyp rlemonstrates cells cotitnining eccentrk nuclei with cvtoplasrnic zmcuoliuition. (HUE; original magnification X15O.j
originating at the level of the tracheal bifurcation can produce symptoms of airway obstruction by direct compression of the tracheobronchial tree. This was present in 1 of our patients who, along with her dysphagia, was complaining of respiratory stridor. The duration of dysphagia and range of ages were similar to that in carcinoma of the esophagus. ROENTGENOGRAPHIC STUDIES
Barium swallow in all our patients showed a large poiypoid intraluminal esophageal mass with partial obstruction and dilatation of the esophagus in the region of and proximal to the tumor. The smooth polypoid nature of the lesion, although not itself diagnostic, is distinctive enough to differentiate sarcoma from the more common ulcerating, stenosing carcinoma. ESOPHAGOSCOPY
Esophagoscopy in our patients showed an intraluminal necrotic mass. When biopsy of the mass is attempted it is important to remove necrotic tissue until viable tissue is obtained, as manifested by bleeding from the tumor surface. When this is not done the biopsy shows only tissue necrosis, as was demonstrated in 1 of our patients. When tumor is obtained by biopsy, it has been our experience and that of others [l 11 that it can be identified as either carcinoma, sarcoma, or carcinosarcoma, depending on the histology of the portion biopsied. Therefore one cannot rely on the biopsy to identify the presence of sarcoma and must depend on the polypoid nature of the lesion to indicate that it may be something other than
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DE MEESTER AND SKINNER carcinoma. Better definition of the biopsy specimen can be obtained with electron microscopy. In 1 of our patients the frozen section was reported as showing poorly differentiated squamous cell carcinoma, but this was more precisely defined as leiomyoblastoma when electron microscopy was used. GROWTH PATTERN
Polypoid sarcoma of the esophagus, in contrast to infiltrating carcinoma, remains superficial to the muscularis propria. T h e patient with leiomyoblastoma was the only one with extension outside the muscularis propria of the esophagus. An adequate peripheral margin of uninvolved tissue was present in all planes of the surgical specimen in every patient. Metastasis to the lymph nodes was seen in only 1 of the 5 patients. This growth characteristic of polypoid sarcoma was apparent in 14 patients diagnosed postmortem, in whom local extension or tumor metastasis would have prevented potentially curative resection in only 5 instances [ 11,161. Thus, the presence of a large polypoid tumor, even in the upper thoracic or cervical esophagus, should not deter the surgeon from resecting the lesion. HISTOLOGY
Sarcomatous lesions of the esophagus can be divided into epidermoid carcinomas with spindle cell features, such as carcinosarcoma, and true sarcomas that arise from mesenchymal tissue, such as leiomyosarcoma, fibrosarcoma, and rhabdomyosarcoma. Based on current histological criteria as a requisite for diagnosis, fibrosarcoma and rhabdomyosarcoma of the esophagus are extremely rare lesions of doubtful existence [ 151. Three of our patients had polypoid tumors consisting of in situ and infiltrating epidermoid carcinoma surrounded by sarcomatous areas of spindle cells. Spindle cell transformation has been recognized for many years as a minor feature in a considerable number of epidermoid carcinomas, and the sarcomatous element may be an anaplastic component of the epithelial tumor [ 131. Indeed, zones of transition from ordinary epidermoid carcinoma to the spindle cell variant were observed in 1 of our patients (see Fig. 3B) and have been reported by others [ 171. For this reason carcinosarcoma has been described as epidermoid carcinoma with various spindle cell features [ 121. In one variety the epithelial and mesenchymal elements are very distinct; its behavior is that of a slow-growing, noninvasive sarcoma, and when it metastasizes the secondary foci appear sarcomatous. T h e second variety consists of an infiltrating tumor that appears to behave like squamous cell carcinoma but differs histologically because of spindle-shaped, sarcomalike cells associated with epithelial elements; when these tumors metastasize the secondary foci appear carcinomatous [9]. Different mixtures of the two elements result in the varied histological picture and behavior of the tumor. When carcinomatous tissue is prominent, a course similar to pure carcinoma may be expected. Two of our 3 patients were consistent with the first variety (see Figs. lB, 3B) and had no evidence of distant metastasis in their lymph nodes. T h e other patient appeared to have the second variety and showed epithelial nodal metastasis even though the primary lesion was polypoid and superficial in its growth (see Fig. 2C). Certainly, polypoid epidermoid carcinoma with spindle cell features behaves 412
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Polypozd Sarcomas of the Esophagus differently than the pure epidermoid carcinoma. Local extension has occurred at the time of diagnosis in 75% of patients with carcinoma, and regional lymph node involvement is present in practically all cases [l]. In contrast, the absence of local extension and lymph node metastasis is the rule in polypoid carcinoma with marked spindle cell features. T h e leiomyosarcoma removed from our patient showed interlacing spindleshaped pleomorphic cells with numerous mitotic figures (see Fig. 4B). T h e tumor was well demarcated from surrounding compressed skeletal muscle, and lymph node metastases were absent. T h e clinical behavior of these tumors parallels their microscopical appearance in that the more aggressive tumors show numerous mitotic figures and cellular anaplasia. Tumors possessing these histological features have a higher incidence of lymph node and distant organ metastasis [ 171. Leiomyoblastoma differs from leiom yosarcoma by containing cells with eccentrically placed nuclei and large vascular spaces in the cytoplasm, giving the cells an epithelioid appearance resembling that of a signet-ring cell of adenocarcinoma (see Fig. 5C). T h e cytoplasmic vacuoles, however, d o not react to periodic acid-Schiff, mucin-carmine, and fat stains [7]. As with leiomyosarcoma, the malignancy of these tumors is based on the presence of nuclear pleomorphism and mitotic activity. When lymph node metastasis occurs, the secondary foci generally have the morphology of the primary tumor. T h e stomach is the site of origin for most leiomyoblastomas, but extragastric primaries do occur, and to our knowledge our patient is the second with leiomyoblastoma of the esophagus to be reported [7]. TREATMENT
Surgical resection of polypoid sarcoma of the esophagus is the treatment of choice, since these tumors remain superficial and local invasion or distant metastases occur late in the course of the disease. Resection also provides an excellent means of palliating the patient’s symptoms. Furthermore, radiation therapy has met with limited success, and resection appears responsible for the majority of the recorded five-year survivals [ 10, 171. T h e surgical exposure for resection of the esophagus and subsequent reconstruction of gastrointestinal continuity depends on the location of the tumor. Lesions located in the lower third of the thoracic esophagus are approached through a left posterolateral thoracotomy and reconstructed with an esophagogastrostomy at the level of the aortic arch. Tumors in the middle third or upper portion of the lower third of the thoracic esophagus are approached through a right posterolateral thoracotomy, as described by Belsey and Hiebert [3], or through a right anterior thoracotomy and midline abdominal incision, as originally described by Lewis [8] and recently popularized by Fisher and associates [51. Gastrointestinal continuity is reestablished with an esophagogastrostomy done just below the thoracic inlet. In both situations a single-layer end-to-side esophagogastrostomy is performed using fine wire sutures. T h e stomach is anchored well to the paravertebral fascia high in the chest, and after construction of the anastomosis it is rolled medially and sutured to the mediastinal pleura. This keeps the whole anastomosis within the mediastinum and gives better protection VOL. 20, NO. 4, OCTOBER, 1975
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DE MEESTER AND SKINNER against hazardous reflux of gastric contents into the esophagus. Combined esophagectomy and laryngectomy are performed for lesions in the cervical or upper third of the thoracic esophagus in which resection would leave a minimal amount of esophagus below the cricopharyngeus muscle. Reconstruction consists of a permanent tracheostomy and reestablishment of continuity between the pharynx and stomach using an isoperistaltic segment of left and transverse colon based on the left colic artery, as described by Belsey [2]. T o prevent aspiration it is necessary that the patient retain sufficient esophagus to allow peristalsis below the cricopharyngeus sphincter. If insufficient esophagus is left and gastrointestinal reconstruction is performed without a laryngectomy, the patient is hampered with repeated episodes of aspiration. This was responsible for the death of our first patient, who had an episode of massive aspiration on the tenth postoperative day. RESULTS OF RESECTION
One is impressed with two facts when reviewing previous reports on esophageal sarcoma. First, the reported operative mortality for this group of patients is surprisingly high, estimated at 45% [ 161. Indeed, 2 of our 5 patients died, both early in the series. We believe that improved postoperative care of thoracotomy patients and use of the newer techniques outlined in this report should cause a major reduction in operative mortality, as reflected by the outcome with our last 3 patients. Second, previous reports were generated out of a desire to document the presence of this rare tumor on a histological basis, and as a result only minor attention was directed toward survival data. We were able to find 8 patients with carcinosarcoma who survived resection for cure and who either died from recurrent disease during their follow-up period or lived longer than five years [l 1, 13, 14, 171. Patients reported alive and well during the five-year follow-up period were excluded. Four of the 8 patients survived for five years or longer. One of these patients was our own who lived thirteen years, 2 survived ten years, and 1 was reported to have lived five years. Even though this number is small, it suggests that resection produces better results in epithelial carcinoma with spindle cell features than in squamous cell carcinoma of the esophagus. Similarly, with leiomyosarcoma of the esophagus the same scattered reports exist with little information on survival. Using the same criteria in our review as for carcinosarcoma, we were able to find 7 patients who had surgical resection for cure and who either died from their disease within the follow-up period or survived five years or longer [4,6,17].Of the 7 patients, 2 died from their disease, one three months and the other four years seven months after resection. The other 5 patients were reported to have survived twelve, seven, seven, six, and five years, respectively. Turnbull and associates [17] reported on 3 patients who received radiotherapy for leiomyosarcoma of the esophagus. Two died two and seventeen months after radiation, and the third survived eleven years before dying of recurrent widespread disease. It is difficult to evaluate the benefits of resection for leiomyoblastoma of the esophagus since the only other reported patient died from the disease nine years
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Polypozd Sarcomas of th Esophagus after radiation therapy [71. In view of the fact that 38% of patients whose primary tumor was in the stomach and 55% with an extragastric primary tumor died from the disease at an average of three years, leiomyoblastoma ought to be considered potentially malignant and apt to behave like a leiomyosarcoma. In general, the presence of nuclear hyperchromatism, increased mitotic figures (more than 1 per high-power field), tumor size larger than 10 cm, and clinical symptoms of longer than six months’ duration are signs of a poor prognosis [7]. We conclude, therefore, that polypoid sarcomas of the esophagus, including those epithelial carcinomas with spindle cell features, are amenable to successful resection with an acceptable prognosis and operative mortality. Gastrointestinal continuity should be restored using techniques that minimize the complications of this operation: regurgitation and pulmonary aspiration.
References 1. Anderson, W. A. D. Pathology (2d ed). St. Louis: Mosby, 1953. 2. Belsey, R. Reconstruction of the esophagus with left colon.J Thorac Cardiovusc Surg 49:33, 1965. 3. Belsey, R., and Hiebert, C. A. An exclusive right thoracic approach for cancer of the middle third of the esophagus. Ann Thorac Surg 18:1, 1974. 4. Borrie, J. Sarcoma of esophagus: Surgical treatment. J Thorac Surg 37:4 13, 1959. 5. Fisher, D. R., Brawley, R. K., and Kieffer, R. F. Esophagogastrostomy in the treatment of carcinoma of the distal two-thirds of the esophagus. Ann Thoruc Surg 14:658, 1972. 6. Johnston, J. B., Clagett, 0. T., and McDonald, J. R. Smooth-muscle tumors of the esophagus. Thorax 8:251, 1953. 7. Lavin, P., Hajdu, S. I., and Foote, F. W., Jr. Gastric and extragastric leiomyoblastomas. Cancer 29~305,1972. 8. Lewis, I. T h e surgical treatment of carcinoma of the esophagus, with special reference to a new operation for growths of the middle third. Br J Surg 2: 18, 1946. 9. Lin, M. H., Luna-Munoz, M. I., Kraft, J. R., and Marks, L. M. Carcinosarcoma ofthe esophagus. Am J Gastroenterol 55:249, 1971. 10. McCort, J. J. Esophageal carcinosarcoma and pseudosarcoma. Radiology 102:519, 1972. 11. Moore, T. C., Battersby, J. S., Vellios, F., and Loehr, W. M. Carcinosarcoma of the esophagus.J Thorac Cardiovasc Surg 45:281, 1963. 12. Pearlman, S. J. So-called carcino-sarcoma of the esophagus. A n n Otol49:805, 1940. 13. Smith, R., and Gowing, W. F. C. Carcinoma of the esophagus with histological appearances simulating a carcinosarcoma. B r J Surg 40:487, 1953. 14. Stout, A. P., Humphreys, G. H., and Rottenberg, L. A. A case of carcinosarcoma of the esophagus. Am J Roentgen01 Radium Ther Nucl Med 61~461,1949. 15. Stout, A. P., and Lattes, R. L. Tumors of the Esophagus. In Atlas ofTumor Pathology, Sect. V, Fasc. 20. Washington, D.C.: Armed Forces Institute of Pathology, 1957. P 95. 16. Talbert, J. L., and Cantrell,J. R. Clinical and pathologic characteristics of carcinosarcoma of the esophagus.J Thorac Cardiovasc Surg 45:1, 1963. 17. Turnbull, A. D., Rosen, P., Goodner,J. T., and Beattie, J. Primary malignant tumors of the esophagus other than typical epidermoid carcinoma. Ann Thorac Surg 15:463, 1973.
Discussion DR.PHILIPE. BERNATZ (Rochester, Minn.): The authors have recommended radical resection for esophageal sarcoma to improve the prognosis. I suggest there really is not a conservative operation worth anything in the management of esophageal malignancies.
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DE MEESTER AND SKINNER There may be findings in the preoperative assessment that suggest ii sarcoma, such as the polypoid appearance, and a compromise procedure may be planned iu view of limited nodal involvement: but ordinarily the degree of resectioii is based upon the level of the lesion rather than the character of the malignancy. T h e preoperative assessnieiit of polypoid lesions can be challenging, especially in the cervical and upper thoracic areas where benign lesions are more prone to occur and where the problems of esophageal resection can be so formidable. To illustrate, we removed a polypoid lesion of the cervical esophagus through a cervical esophagotomy. T h e clue permitting local removal was the calcification evident in the lesion. It was a chondroma that had appeared ominous by endoscopy because of its ulcerated surface. Polypoid lesions in the upper esophagus are more inclined to be benign and are usually fibrolipomas. Endoscopic biopsy can be helpful but is not a consistently valuable route to an easy treatment plan, and it may be misleading. For example, the surface epithelium in a fibrolipoma is often proliferative and in a small specimen can look like a well-differentiated squamous cell carcinoma. Its underlying inflammatory tissue can be equally deluding with respect to sarcoma, as in a carcinosarcoma that Dr. DeMeester showed us. The unusual appearance of esophageal sarcoma, the difficulties with histological classification, and the rarity of the lesion alone have led to delays and inadequate treatment. Dr. DeMeester and his associates deserve thanks for bringing to our attention these rare but challenging lesions and for emphasizing appropriate resection. (San Francisco, Calif.): I would like to add another case to DR. ARTHURN. THOMAS Dr. DeMeester’s report on these relatively rare esophageal tumors. An 80-year-old exriveter had five months’ progressive dysphagia and a fifty-pound weight loss. An esophagogram showed two separate polypoid leiom yosarcomas involving the distal esophagus. Resection was never seriously considered because the patient was debilitated and had cirrhosis, bronchopneumonia, amyotrophic lateral sclerosis, and an abnormal electrocardiogram. After two weeks of preoperative preparation we decided to treat his esophageal obstruction by placement of a Celestin tube. Postoperatively this less aggressive approach appeared justified because he developed cardiopulmonary failure that necessitated, among other things, continuous mechanical ventilation for five days in the intensive care unit. After that he improved and rapidly progressed to a regular diet. He gained weight and was asymptomatic until he suddenly died from cardiac disease three and a half months later. Postmortem examination showed the patient to have leiomyosarcoma still confined to the esophagus, but also to have a nearby early squamous cell carcinoma of the esophagus. Esophageal leiomyosarcoma in an 80-year-old is unusual; the oldest we found among 39 reported patients was 78. Benign esophageal leiomyoma may be multiple in 10% of patients, but multiple sarcoma is rare, Combined leiomyosarcoma and epidermoid carcinoma have been reported in 4 other patients. Celestin tube palliation of this more indolent esophageal cancer seems useful, but we could find no patient in whom this had previously been used. When possible, leiomyosarcoma should be widely resected, but even local resection can give prolonged benefit. Celestin tube placement should be considered for patients who are elderly and debilitated and who will not tolerate thoracotomy, or for individuals in whom a more extensive procedure is unwarranted, since the result of no treatment at all or gastrostomy alone has led to early death from aspiration pneumonia in most cases.
DR. RAYMOND C. READ(Little Rock, Ark.): I would like to add 1 patient with leiomyoblastoma of the esophagus because, as Dr. DeMeester pointed out, only 1 other case has been described, and there are some confusing features to this lesion. Esophageal leiomyoblastoma is probably not recognized as much as it should be since it was described in the stomach for the first time only in 1960. Our patient was 37 years old. Esophagoscopy showed an intramural mass without any ulceration in the cervical esophagus. The pathological diagnosis from the Armed Forces Institute of Pathology after the lesion was removed by enucleation was anaplastic carcinoma. He received radiation treatment and was well eight years later.
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Polypoid Sarcomas of the Esophagus T h e lesion was bulky and polypoid, as illustrated by Dr. DeMeester. At operation it was found to be intramural and did not extend through the muscular coat or through the mucosa. It was enucleated because it was thought to be a leiomyoma. This lesion did not extend directly into the epithelium, although part of the epithelium was removed. It appeared to be an epithelial lesion, which is why it had originally been considered an anaplastic carcinoma. Like squamous cell carcinoma of the A. PICCONE, JR. (Brooklyn, N.Y.): DR. VINCENT esophagus, the rarer neoplasms also have the appearance of an obstructive lesion, the true nature of which is identified in later studies or at operation. We have seen two rare lesions, a primary malignant melanoma and a primary plasmocytoma, among 75 patients undergoing resection and esophagogastrostomy over the last ten years. Survival after resection of malignant melanoma is very low. Our patient had the first reported primary plasmocytoma in the esophagus, which was identified by the absence of Bence Jones protein in the urine, electrophoresis negative for myeloma-band protein, and normal bone marrow studies. Incidentally, all 75 reconstructions were by esophagogastrostomy after resection, without pyloroplasty, and there was no evidence of gastric retention in the patients.
DR.DEMEESTER:In reference to Dr. Bernatz’ comments, it is difficult at times to know when these lesions are benign. A rare exception is when calcium is present in the lesion or a biopsy shows lipoma or chondroma. In this situation, local resection would be adequate. In all other circumstances we recommend that a wide esophageal resection be performed. Concerning Dr. Thomas’ comments, I would emphasize that even though these tumors can be large, they still may be operable. In a series of 14 esophageal sarcomas found postmortem, all but 5 could have been resected in the absence of distant metastasis or local invasion, so large tumor size should not be a deterrent to resection. We appreciate Dr. Read’s report of an additional esophageal leiomyoblastoma. This brings the total reported cases to 3. I would like to emphasize that electron microscopy is helpful in differentiating a leiomyoblastoma from an undifferentiated carcinoma by demonstrating mesenchymal elements - that is, myofibrils - within the cell. Dr. Piccone, we appreciate your comments regarding the plasma cell cytoma. We did not include in our series those patients who had sarcomas of lymphohematopoietic cell origin, such as the lymphomas, Hodgkin’s disease, or reticulum cell sarcoma.
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S
arcomas - and carcinosarcomas, which are included with them - are rare neoplasms accounting for approximately 0.1 to 1.5% of all esophageal tumors [ 151. The rarity of these lesions is illustrated by the small number of previous reports, consistingof single or small groups of patients. Despite their infrequent occurrence they exhibit several clinically important behavior patterns, which are illustrated by the 5 patients reported in this study.
Clinical Material PATIENT 1
A 57-year-old white man was referred to the University of Chicago Hospitals with a nine-month history of dysphagia, three pound weight loss, and four weeks of tarry stools. Barium swallow showed a 9 cm polypoid intraluminal lesion in the upper third of the esophagus (Fig. 1A). A biopsy obtained by esophagoscopy was reported as several pieces of granulation tissue and one fragment suggestive but not diagnostic of carcinoma. Esophagectomy with esophagogastrostomy was performed through a left posterolateral thoracotomy incision. The esophagus was divided 3 cm below the apex of the chest at a point approximately 2 cm above the tumor. The surgical specimen contained an 8 cm polypoid tumor of the esophagus with necrosis and hemorrhagic changes on its surface. The tumor was noted not to extend into the muscularis propria, and there were adequate peripheral margins of uninvolved tissue in all planes. The polyp was composed of small nests of From the Department of Surgery, University of Chicago Pritzker School of Medicine, Chicago, Ill. Presented at the Eleventh Annual Meeting of The Society of Thoracic Surgeons, Montreal, Que., Canada, Jan. 20-22, 1975. Address reprint requests to Dr. DeMeester, Department of Surgery, Section of Thoracic and Vascular Surgery, University of Chicago Pritzker School of Medicine, 950 E. 59th St., Chicago, Ill. 60637.
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FIG. 1 . (Patient I .) ( A ) Barium swallow demonstrating a polypoid lesion in the upper third of the esophagus. ( B ) Photomicrograph of the polyp shows distinct nests of squamous eplthelium surrounded by interlacing bundles of spindle-shaped cells. (HWE; origznal magnification X 150.)
squamous epithelium surrounded by interlacing bundles of spindle-shaped cells with numerous mitoses and occasional atypical giant cells (Fig. 1B). Regional lymph nodes were free of tumor. The final histological diagnosis was carcinosarcoma of the esophagus composed of squamous cell carcinoma and undifferentiated spindle cell sarcoma. On the tenth postoperative day the patient died suddenly following an episode of severe aspiration. Postmortem examination showed that the small bronchi throughout the lung contained stomach contents. There was no evidence of lymphatic or hematogenous tumor metastasis. PATIENT 2
An 83-year-old white man was referred to the University of Chicago Hospitals with a six-week history of dysphagia. A left supraclavicular lymph node was palpable. Barium swallow showed a 9 cm polypoid intraluminal lesion in the middle third of the esophagus (Fig. 2A). Biopsy by esophagoscopy was reported as demonstrating carcinoma. Biopsy of the supraclavicular lymph node showed it to be completely replaced with large hyperchromatic pleomorphic cells, representing an undifferentiated neoplasm suggestive of carcinoma. Esophagectomy with esophagogastrostomy was performed through a left posterolateral thoracotomy. T h e esophagus was divided at the arch of the aorta 2 cm above the palpable tumor. T h e surgical specimen (Fig. 2B) contained a 7 cm polypoid tumor of the esophagus. T h e tumor was noted not to extend into the muscularis propria, and 406
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Polypoid Sarcomas of the Esophagus
FIG. 2. (Patzent 2 . ) ( A ) Spotjzlmjrom barzum swallow showing a polypoid lesion in the middle third of the esophagus. (Retouched for clarification.) ( B ) Surgical specimen containing a 7 cm polypoid lesion of the esophagus. (C) Photomicrograph of the polyp shows nests of lurge pleomorphic cells closely associated with and surrounded by loose bundles of spindle-shaped cells. (HE3E; original magnzpcation ~ 1 . 5 0 . )
there were adequate peripheral margins of uninvolved tissue in all planes. T h e polyp was composed of large pleomorphic cells with hyperchromatic nuclei showingmany mitotic figures. In certain regions the tumor was traversed with a spindle cell stroma (Fig. 2C). Regional lymph nodes were replaced by metastatic epithelioid cells. T h e final histological diagnosis was atypical polypoid carcinoma of the esophagus with carcinosarcomatous features. In the immediate postoperative period the patient suddenly became cyanotic, with changes in his cardiac rhythm. T h e day after operation he developed ventricular fibrillation from which h e died. On postmortem examination he had a coiled 21 x 2 cm thromboembolus within the right heart, extending through the phlmonary valve into the first 5 cm of the pulmonary artery. T h e tracheobronchial nodes contained metastatic epithelioid tumor. PATIENT 3
A 70-year-old white man was referred to the University of Chicago Hospitals with a one-month history of dysphagia. Barium swallow showed an 8 cm polypoid intraluminal lesion in the lower third of the esophagus. Biopsy by esophagoscopy showed a neoplasm consisting of spindle cells with pleomorphic nuclei and numerous mitotic figures. Nowhere within the specimen was there any evidence of esophageal epithelium. T h e histological diagnosis was sarcoma of the esophagus. Esophagectomy with esophagogastrostomy was performed through a left posterolateral thoracotomy. T h e esophagus was divided just below the aortic arch at a point 2 cm above the palpable tumor. T h e surgical specimen (Fig. 3A) included a 5 cm polypoid tumor of the VOL. 20, NO. 4, OCTOBER, 1975
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FIG. 3 . (Patient 3.) ( A ) Surpcal specimen shows a 5 cm polypoid tumor protrudingf r o m the end o j tht resected e.~ophngus. ( B ) Photomicrograph of the polyp reveals nests of squamous epthelium surrounded by interlacing bundles of spindle-shaped cells. An area of transition f r o m ordinary epdermoid carcinoma to the spindle cell variant is demonstrated i n the upper left-hand corner (arrow). ( H U E ; oripnal magnzfication x 150.)
esophagus. There was no apparent direct tumor invasion of the esophageal wall beneath the base of the tumor. Adequate peripheral margins of uninvolved tissue were present in all planes. The polyp was composed of nests of squamous epithelium surrounded by interlacing bundles of spindle-shaped cells with numerous mitoses and occasional atypical giant cells. In some regions the epithelial cells appeared to fan out and form the spindle-shaped cells of the mesenchymal stroma (Fig. 3B). Regional lymph nodes were free of tumor. T h e final histological diagnosis was carcinosarcoma of the esophagus composed o f squamous cell carcinoma with a sarcomatous stroma. T h e patient was discharged on the twenty-second postoperative day. He remained healthy and free from tumor for thirteen years, eventually dying from bronchial pneumonia. PATIENT 4
A 60-year-old white woman was transferred to the University of Chicago Hospitals with total esophageal obstruction that had persisted for three weeks 408
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Polypoid Sarcomas of the Esophagus
FIG. 4 . (Patient 4 . ) ( A ) Barium swallow shows a polypoid mass in the upper thoracic and cervical esophagus. (B) Photomicrograph of the polyp demonstrates large spindte-shaped cells. No carcinoma zuas seeti withiu the sectioxs. (HME; original magnzfication X 150.)
following cervical esophagomyotomy. She had complained of cervical dysphagia and an eighteen-pound weight loss over four months prior to the myotomy. Barium swallow showed a 7 cm polypoid intraluminal mass in the cervical esophagus (Fig. 4A). At esophagoscopy large quantities of necrotic tissue were removed before a biopsy of viable tissue was obtained. T h e biopsy showed spindle cells, neoplastic giant cells, and occasional foci of polyhedral cells suggestive of carcinoma. T h e histological diagnosis was carcinosarcoma. After three weeks of intravenous hyperalimentation and treatment for aspiration pneumonia, a total esophagectomy and en bloc laryngectomy were performed. An isoperistaltic segment of left and transverse colon based on the left colic artery was used to reestablish continuity between the pharynx and stomach. T h e surgical specimen consisted of the entire esophagus with an attached larynx. T h e upper esophagus contained a polypoid tumor measuring 6.5 cm in length that was firmly attached to the anterior and posterior walls of the esophagus, forming two lumens. There was no evidence of tumor penetration through the esophageal wall, and adequate peripheral margins of uninvolved tissue were apparent in all planes. There was no tumor invasion into the tissues between the esophagus and larynx or into the larynx itself. T h e polyp was composed of large spindle-shaped cells with numerous mitoses (Fig. 4B).T h e tumor was well demarcated from surrounding compressed skeletal muscle. No carcinoma was seen within the sections. Myofibrils were demonstrated on electron VOL. 20, NO. 4, OCTOBER, 1975
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DE MEESTER AND SKINNER microscopy, but there were no cross-striations characteristic of skeletal muscle. Regional lymph nodes were free of tumor. The final histological diagnosis was leiomyosarcoma of the cervical esophagus. After a prolonged recovery she was discharged. When evaluated seventeen months after operation she was free from tumor, had gained fifteen pounds, and was beginning attempts at mastering the art of colonic speech. PATIENT 5
A 62-year-oldblack woman was referred to the University of Chicago Hospitals for radiotherapy for a presumed esophageal carcinoma. She gave a history of dysphagia for nine months, sixty-pound weight loss, and one month of respiratory stridor. Two previous esophageal biopsies had revealed only necrotic tissue. Barium swallow showed a 10 cm polypoid mass within the middle third of the esophagus (Fig.5A). Bronchoscopy showed compression of both main bronchi. At esophagoscopy a large quantity of necrotic tissue was removed before a biopsy of bleeding tissue was obtained. The frozen-section diagnosis on this tissue was poorly differentiated squamous cell carcinoma, but on closer inspection of the tissue by electron microscopy it was noted to contain myofibrils, and the diagnosis of leiomyoblastoma of the esophagus was made. An esophagectomy was performed through a right posterolateral thoracotomy. The stomach was mobilized through the esophageal hiatus, and after performing a pyloroplasty, an end-toside esophagogastrostomy was constructed. The surgical specimen (Fig. 5B) contained a 9 cm polypoid tumor within the esophagus. The tumor at its pedicle extended through the muscularis propria into the adventitia, but on microscopical observation there were adequate peripheral margins of uninvolved tissue in all planes. Regional lymph nodes were free of tumor. The polyp was composed largely of necrotic tissue except for its pedicle portion, where there were cells containing eccentric nuclei with cytoplasmic vacuolization (Fig. 5C). There was only scattered evidence of mitosis. The final histological diagnosis was polypoid leiomyoblastoma of the esophagus. On the sixteenth postoperative day the patient was discharged and has remained well in follow-up for fifteen months.
Comment
These 5 patients illustrate a number of factors common to previous reports on the clinical behavior of esophageal sarcoma. An awareness of these factors is helpful in the clinical management of this rare tumor. CLINICAL PRESENTATION
The symptom of dysphagia is due to esophageal obstruction and does not really differ from the dysphagia associated with the more common epithelial carcinoma. Tumors located within the cervical or high thoracic esophagus can cause symptoms of pulmonary aspiration. This occurred in 1 of our patients, in whom the mistaken diagnosis of cricopharyngeal achalasia led to an unnecessary esophageal myotomy and aggravated her symptom of aspiration. Large tumors
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Polypoid Sarcomas of the Esophagus
FIG. 5 . (Pntierit 5.) ( A ) Bnriurti .szuiillozo reuerils (1 /iirgepolypviil tnrrss within the muldle third of the e.sophn,gus. ( R ) Sur,giccil specirnen shows ii 9 ctti polypoid turnor within the esophngus with necrosis a d hetniirrhngic chntigu.t on it.s ,c.uifiicu.(C) Phiitr)microgrcipii qf thu peilicle portion of the polyp rlemonstrates cells cotitnining eccentrk nuclei with cvtoplasrnic zmcuoliuition. (HUE; original magnification X15O.j
originating at the level of the tracheal bifurcation can produce symptoms of airway obstruction by direct compression of the tracheobronchial tree. This was present in 1 of our patients who, along with her dysphagia, was complaining of respiratory stridor. The duration of dysphagia and range of ages were similar to that in carcinoma of the esophagus. ROENTGENOGRAPHIC STUDIES
Barium swallow in all our patients showed a large poiypoid intraluminal esophageal mass with partial obstruction and dilatation of the esophagus in the region of and proximal to the tumor. The smooth polypoid nature of the lesion, although not itself diagnostic, is distinctive enough to differentiate sarcoma from the more common ulcerating, stenosing carcinoma. ESOPHAGOSCOPY
Esophagoscopy in our patients showed an intraluminal necrotic mass. When biopsy of the mass is attempted it is important to remove necrotic tissue until viable tissue is obtained, as manifested by bleeding from the tumor surface. When this is not done the biopsy shows only tissue necrosis, as was demonstrated in 1 of our patients. When tumor is obtained by biopsy, it has been our experience and that of others [l 11 that it can be identified as either carcinoma, sarcoma, or carcinosarcoma, depending on the histology of the portion biopsied. Therefore one cannot rely on the biopsy to identify the presence of sarcoma and must depend on the polypoid nature of the lesion to indicate that it may be something other than
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DE MEESTER AND SKINNER carcinoma. Better definition of the biopsy specimen can be obtained with electron microscopy. In 1 of our patients the frozen section was reported as showing poorly differentiated squamous cell carcinoma, but this was more precisely defined as leiomyoblastoma when electron microscopy was used. GROWTH PATTERN
Polypoid sarcoma of the esophagus, in contrast to infiltrating carcinoma, remains superficial to the muscularis propria. T h e patient with leiomyoblastoma was the only one with extension outside the muscularis propria of the esophagus. An adequate peripheral margin of uninvolved tissue was present in all planes of the surgical specimen in every patient. Metastasis to the lymph nodes was seen in only 1 of the 5 patients. This growth characteristic of polypoid sarcoma was apparent in 14 patients diagnosed postmortem, in whom local extension or tumor metastasis would have prevented potentially curative resection in only 5 instances [ 11,161. Thus, the presence of a large polypoid tumor, even in the upper thoracic or cervical esophagus, should not deter the surgeon from resecting the lesion. HISTOLOGY
Sarcomatous lesions of the esophagus can be divided into epidermoid carcinomas with spindle cell features, such as carcinosarcoma, and true sarcomas that arise from mesenchymal tissue, such as leiomyosarcoma, fibrosarcoma, and rhabdomyosarcoma. Based on current histological criteria as a requisite for diagnosis, fibrosarcoma and rhabdomyosarcoma of the esophagus are extremely rare lesions of doubtful existence [ 151. Three of our patients had polypoid tumors consisting of in situ and infiltrating epidermoid carcinoma surrounded by sarcomatous areas of spindle cells. Spindle cell transformation has been recognized for many years as a minor feature in a considerable number of epidermoid carcinomas, and the sarcomatous element may be an anaplastic component of the epithelial tumor [ 131. Indeed, zones of transition from ordinary epidermoid carcinoma to the spindle cell variant were observed in 1 of our patients (see Fig. 3B) and have been reported by others [ 171. For this reason carcinosarcoma has been described as epidermoid carcinoma with various spindle cell features [ 121. In one variety the epithelial and mesenchymal elements are very distinct; its behavior is that of a slow-growing, noninvasive sarcoma, and when it metastasizes the secondary foci appear sarcomatous. T h e second variety consists of an infiltrating tumor that appears to behave like squamous cell carcinoma but differs histologically because of spindle-shaped, sarcomalike cells associated with epithelial elements; when these tumors metastasize the secondary foci appear carcinomatous [9]. Different mixtures of the two elements result in the varied histological picture and behavior of the tumor. When carcinomatous tissue is prominent, a course similar to pure carcinoma may be expected. Two of our 3 patients were consistent with the first variety (see Figs. lB, 3B) and had no evidence of distant metastasis in their lymph nodes. T h e other patient appeared to have the second variety and showed epithelial nodal metastasis even though the primary lesion was polypoid and superficial in its growth (see Fig. 2C). Certainly, polypoid epidermoid carcinoma with spindle cell features behaves 412
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Polypozd Sarcomas of the Esophagus differently than the pure epidermoid carcinoma. Local extension has occurred at the time of diagnosis in 75% of patients with carcinoma, and regional lymph node involvement is present in practically all cases [l]. In contrast, the absence of local extension and lymph node metastasis is the rule in polypoid carcinoma with marked spindle cell features. T h e leiomyosarcoma removed from our patient showed interlacing spindleshaped pleomorphic cells with numerous mitotic figures (see Fig. 4B). T h e tumor was well demarcated from surrounding compressed skeletal muscle, and lymph node metastases were absent. T h e clinical behavior of these tumors parallels their microscopical appearance in that the more aggressive tumors show numerous mitotic figures and cellular anaplasia. Tumors possessing these histological features have a higher incidence of lymph node and distant organ metastasis [ 171. Leiomyoblastoma differs from leiom yosarcoma by containing cells with eccentrically placed nuclei and large vascular spaces in the cytoplasm, giving the cells an epithelioid appearance resembling that of a signet-ring cell of adenocarcinoma (see Fig. 5C). T h e cytoplasmic vacuoles, however, d o not react to periodic acid-Schiff, mucin-carmine, and fat stains [7]. As with leiomyosarcoma, the malignancy of these tumors is based on the presence of nuclear pleomorphism and mitotic activity. When lymph node metastasis occurs, the secondary foci generally have the morphology of the primary tumor. T h e stomach is the site of origin for most leiomyoblastomas, but extragastric primaries do occur, and to our knowledge our patient is the second with leiomyoblastoma of the esophagus to be reported [7]. TREATMENT
Surgical resection of polypoid sarcoma of the esophagus is the treatment of choice, since these tumors remain superficial and local invasion or distant metastases occur late in the course of the disease. Resection also provides an excellent means of palliating the patient’s symptoms. Furthermore, radiation therapy has met with limited success, and resection appears responsible for the majority of the recorded five-year survivals [ 10, 171. T h e surgical exposure for resection of the esophagus and subsequent reconstruction of gastrointestinal continuity depends on the location of the tumor. Lesions located in the lower third of the thoracic esophagus are approached through a left posterolateral thoracotomy and reconstructed with an esophagogastrostomy at the level of the aortic arch. Tumors in the middle third or upper portion of the lower third of the thoracic esophagus are approached through a right posterolateral thoracotomy, as described by Belsey and Hiebert [3], or through a right anterior thoracotomy and midline abdominal incision, as originally described by Lewis [8] and recently popularized by Fisher and associates [51. Gastrointestinal continuity is reestablished with an esophagogastrostomy done just below the thoracic inlet. In both situations a single-layer end-to-side esophagogastrostomy is performed using fine wire sutures. T h e stomach is anchored well to the paravertebral fascia high in the chest, and after construction of the anastomosis it is rolled medially and sutured to the mediastinal pleura. This keeps the whole anastomosis within the mediastinum and gives better protection VOL. 20, NO. 4, OCTOBER, 1975
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DE MEESTER AND SKINNER against hazardous reflux of gastric contents into the esophagus. Combined esophagectomy and laryngectomy are performed for lesions in the cervical or upper third of the thoracic esophagus in which resection would leave a minimal amount of esophagus below the cricopharyngeus muscle. Reconstruction consists of a permanent tracheostomy and reestablishment of continuity between the pharynx and stomach using an isoperistaltic segment of left and transverse colon based on the left colic artery, as described by Belsey [2]. T o prevent aspiration it is necessary that the patient retain sufficient esophagus to allow peristalsis below the cricopharyngeus sphincter. If insufficient esophagus is left and gastrointestinal reconstruction is performed without a laryngectomy, the patient is hampered with repeated episodes of aspiration. This was responsible for the death of our first patient, who had an episode of massive aspiration on the tenth postoperative day. RESULTS OF RESECTION
One is impressed with two facts when reviewing previous reports on esophageal sarcoma. First, the reported operative mortality for this group of patients is surprisingly high, estimated at 45% [ 161. Indeed, 2 of our 5 patients died, both early in the series. We believe that improved postoperative care of thoracotomy patients and use of the newer techniques outlined in this report should cause a major reduction in operative mortality, as reflected by the outcome with our last 3 patients. Second, previous reports were generated out of a desire to document the presence of this rare tumor on a histological basis, and as a result only minor attention was directed toward survival data. We were able to find 8 patients with carcinosarcoma who survived resection for cure and who either died from recurrent disease during their follow-up period or lived longer than five years [l 1, 13, 14, 171. Patients reported alive and well during the five-year follow-up period were excluded. Four of the 8 patients survived for five years or longer. One of these patients was our own who lived thirteen years, 2 survived ten years, and 1 was reported to have lived five years. Even though this number is small, it suggests that resection produces better results in epithelial carcinoma with spindle cell features than in squamous cell carcinoma of the esophagus. Similarly, with leiomyosarcoma of the esophagus the same scattered reports exist with little information on survival. Using the same criteria in our review as for carcinosarcoma, we were able to find 7 patients who had surgical resection for cure and who either died from their disease within the follow-up period or survived five years or longer [4,6,17].Of the 7 patients, 2 died from their disease, one three months and the other four years seven months after resection. The other 5 patients were reported to have survived twelve, seven, seven, six, and five years, respectively. Turnbull and associates [17] reported on 3 patients who received radiotherapy for leiomyosarcoma of the esophagus. Two died two and seventeen months after radiation, and the third survived eleven years before dying of recurrent widespread disease. It is difficult to evaluate the benefits of resection for leiomyoblastoma of the esophagus since the only other reported patient died from the disease nine years
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Polypozd Sarcomas of th Esophagus after radiation therapy [71. In view of the fact that 38% of patients whose primary tumor was in the stomach and 55% with an extragastric primary tumor died from the disease at an average of three years, leiomyoblastoma ought to be considered potentially malignant and apt to behave like a leiomyosarcoma. In general, the presence of nuclear hyperchromatism, increased mitotic figures (more than 1 per high-power field), tumor size larger than 10 cm, and clinical symptoms of longer than six months’ duration are signs of a poor prognosis [7]. We conclude, therefore, that polypoid sarcomas of the esophagus, including those epithelial carcinomas with spindle cell features, are amenable to successful resection with an acceptable prognosis and operative mortality. Gastrointestinal continuity should be restored using techniques that minimize the complications of this operation: regurgitation and pulmonary aspiration.
References 1. Anderson, W. A. D. Pathology (2d ed). St. Louis: Mosby, 1953. 2. Belsey, R. Reconstruction of the esophagus with left colon.J Thorac Cardiovusc Surg 49:33, 1965. 3. Belsey, R., and Hiebert, C. A. An exclusive right thoracic approach for cancer of the middle third of the esophagus. Ann Thorac Surg 18:1, 1974. 4. Borrie, J. Sarcoma of esophagus: Surgical treatment. J Thorac Surg 37:4 13, 1959. 5. Fisher, D. R., Brawley, R. K., and Kieffer, R. F. Esophagogastrostomy in the treatment of carcinoma of the distal two-thirds of the esophagus. Ann Thoruc Surg 14:658, 1972. 6. Johnston, J. B., Clagett, 0. T., and McDonald, J. R. Smooth-muscle tumors of the esophagus. Thorax 8:251, 1953. 7. Lavin, P., Hajdu, S. I., and Foote, F. W., Jr. Gastric and extragastric leiomyoblastomas. Cancer 29~305,1972. 8. Lewis, I. T h e surgical treatment of carcinoma of the esophagus, with special reference to a new operation for growths of the middle third. Br J Surg 2: 18, 1946. 9. Lin, M. H., Luna-Munoz, M. I., Kraft, J. R., and Marks, L. M. Carcinosarcoma ofthe esophagus. Am J Gastroenterol 55:249, 1971. 10. McCort, J. J. Esophageal carcinosarcoma and pseudosarcoma. Radiology 102:519, 1972. 11. Moore, T. C., Battersby, J. S., Vellios, F., and Loehr, W. M. Carcinosarcoma of the esophagus.J Thorac Cardiovasc Surg 45:281, 1963. 12. Pearlman, S. J. So-called carcino-sarcoma of the esophagus. A n n Otol49:805, 1940. 13. Smith, R., and Gowing, W. F. C. Carcinoma of the esophagus with histological appearances simulating a carcinosarcoma. B r J Surg 40:487, 1953. 14. Stout, A. P., Humphreys, G. H., and Rottenberg, L. A. A case of carcinosarcoma of the esophagus. Am J Roentgen01 Radium Ther Nucl Med 61~461,1949. 15. Stout, A. P., and Lattes, R. L. Tumors of the Esophagus. In Atlas ofTumor Pathology, Sect. V, Fasc. 20. Washington, D.C.: Armed Forces Institute of Pathology, 1957. P 95. 16. Talbert, J. L., and Cantrell,J. R. Clinical and pathologic characteristics of carcinosarcoma of the esophagus.J Thorac Cardiovasc Surg 45:1, 1963. 17. Turnbull, A. D., Rosen, P., Goodner,J. T., and Beattie, J. Primary malignant tumors of the esophagus other than typical epidermoid carcinoma. Ann Thorac Surg 15:463, 1973.
Discussion DR.PHILIPE. BERNATZ (Rochester, Minn.): The authors have recommended radical resection for esophageal sarcoma to improve the prognosis. I suggest there really is not a conservative operation worth anything in the management of esophageal malignancies.
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DE MEESTER AND SKINNER There may be findings in the preoperative assessment that suggest ii sarcoma, such as the polypoid appearance, and a compromise procedure may be planned iu view of limited nodal involvement: but ordinarily the degree of resectioii is based upon the level of the lesion rather than the character of the malignancy. T h e preoperative assessnieiit of polypoid lesions can be challenging, especially in the cervical and upper thoracic areas where benign lesions are more prone to occur and where the problems of esophageal resection can be so formidable. To illustrate, we removed a polypoid lesion of the cervical esophagus through a cervical esophagotomy. T h e clue permitting local removal was the calcification evident in the lesion. It was a chondroma that had appeared ominous by endoscopy because of its ulcerated surface. Polypoid lesions in the upper esophagus are more inclined to be benign and are usually fibrolipomas. Endoscopic biopsy can be helpful but is not a consistently valuable route to an easy treatment plan, and it may be misleading. For example, the surface epithelium in a fibrolipoma is often proliferative and in a small specimen can look like a well-differentiated squamous cell carcinoma. Its underlying inflammatory tissue can be equally deluding with respect to sarcoma, as in a carcinosarcoma that Dr. DeMeester showed us. The unusual appearance of esophageal sarcoma, the difficulties with histological classification, and the rarity of the lesion alone have led to delays and inadequate treatment. Dr. DeMeester and his associates deserve thanks for bringing to our attention these rare but challenging lesions and for emphasizing appropriate resection. (San Francisco, Calif.): I would like to add another case to DR. ARTHURN. THOMAS Dr. DeMeester’s report on these relatively rare esophageal tumors. An 80-year-old exriveter had five months’ progressive dysphagia and a fifty-pound weight loss. An esophagogram showed two separate polypoid leiom yosarcomas involving the distal esophagus. Resection was never seriously considered because the patient was debilitated and had cirrhosis, bronchopneumonia, amyotrophic lateral sclerosis, and an abnormal electrocardiogram. After two weeks of preoperative preparation we decided to treat his esophageal obstruction by placement of a Celestin tube. Postoperatively this less aggressive approach appeared justified because he developed cardiopulmonary failure that necessitated, among other things, continuous mechanical ventilation for five days in the intensive care unit. After that he improved and rapidly progressed to a regular diet. He gained weight and was asymptomatic until he suddenly died from cardiac disease three and a half months later. Postmortem examination showed the patient to have leiomyosarcoma still confined to the esophagus, but also to have a nearby early squamous cell carcinoma of the esophagus. Esophageal leiomyosarcoma in an 80-year-old is unusual; the oldest we found among 39 reported patients was 78. Benign esophageal leiomyoma may be multiple in 10% of patients, but multiple sarcoma is rare, Combined leiomyosarcoma and epidermoid carcinoma have been reported in 4 other patients. Celestin tube palliation of this more indolent esophageal cancer seems useful, but we could find no patient in whom this had previously been used. When possible, leiomyosarcoma should be widely resected, but even local resection can give prolonged benefit. Celestin tube placement should be considered for patients who are elderly and debilitated and who will not tolerate thoracotomy, or for individuals in whom a more extensive procedure is unwarranted, since the result of no treatment at all or gastrostomy alone has led to early death from aspiration pneumonia in most cases.
DR. RAYMOND C. READ(Little Rock, Ark.): I would like to add 1 patient with leiomyoblastoma of the esophagus because, as Dr. DeMeester pointed out, only 1 other case has been described, and there are some confusing features to this lesion. Esophageal leiomyoblastoma is probably not recognized as much as it should be since it was described in the stomach for the first time only in 1960. Our patient was 37 years old. Esophagoscopy showed an intramural mass without any ulceration in the cervical esophagus. The pathological diagnosis from the Armed Forces Institute of Pathology after the lesion was removed by enucleation was anaplastic carcinoma. He received radiation treatment and was well eight years later.
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Polypoid Sarcomas of the Esophagus T h e lesion was bulky and polypoid, as illustrated by Dr. DeMeester. At operation it was found to be intramural and did not extend through the muscular coat or through the mucosa. It was enucleated because it was thought to be a leiomyoma. This lesion did not extend directly into the epithelium, although part of the epithelium was removed. It appeared to be an epithelial lesion, which is why it had originally been considered an anaplastic carcinoma. Like squamous cell carcinoma of the A. PICCONE, JR. (Brooklyn, N.Y.): DR. VINCENT esophagus, the rarer neoplasms also have the appearance of an obstructive lesion, the true nature of which is identified in later studies or at operation. We have seen two rare lesions, a primary malignant melanoma and a primary plasmocytoma, among 75 patients undergoing resection and esophagogastrostomy over the last ten years. Survival after resection of malignant melanoma is very low. Our patient had the first reported primary plasmocytoma in the esophagus, which was identified by the absence of Bence Jones protein in the urine, electrophoresis negative for myeloma-band protein, and normal bone marrow studies. Incidentally, all 75 reconstructions were by esophagogastrostomy after resection, without pyloroplasty, and there was no evidence of gastric retention in the patients.
DR.DEMEESTER:In reference to Dr. Bernatz’ comments, it is difficult at times to know when these lesions are benign. A rare exception is when calcium is present in the lesion or a biopsy shows lipoma or chondroma. In this situation, local resection would be adequate. In all other circumstances we recommend that a wide esophageal resection be performed. Concerning Dr. Thomas’ comments, I would emphasize that even though these tumors can be large, they still may be operable. In a series of 14 esophageal sarcomas found postmortem, all but 5 could have been resected in the absence of distant metastasis or local invasion, so large tumor size should not be a deterrent to resection. We appreciate Dr. Read’s report of an additional esophageal leiomyoblastoma. This brings the total reported cases to 3. I would like to emphasize that electron microscopy is helpful in differentiating a leiomyoblastoma from an undifferentiated carcinoma by demonstrating mesenchymal elements - that is, myofibrils - within the cell. Dr. Piccone, we appreciate your comments regarding the plasma cell cytoma. We did not include in our series those patients who had sarcomas of lymphohematopoietic cell origin, such as the lymphomas, Hodgkin’s disease, or reticulum cell sarcoma.
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