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Ann Thorac Surg 1998;66:894-897
© 1998 The Society of Thoracic Surgeons

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Original articles: General Thoracic

Leiomyosarcoma of the esophagus: results of surgical treatment

^a Section of General Thoracic Surgery, Mayo Clinic and Mayo Foundation, Rochester, Minnesota, USA

Address reprint requests to Dr Trastek, Mayo Clinic, 200 First St, SW, Rochester, MN 55905

Presented at the Thirty-fourth Annual Meeting of The Society of Thoracic Surgeons, New Orleans, LA, Jan 26–28, 1998.

	Abstract

Top Abstract Introduction Material and methods Results Comment References

 
Background. This study examined the results of surgical treatment of leiomyosarcoma of the esophagus.

Methods. Between January 1920 and December 1996, 17 patients (9 men and 8 women) with leiomyosarcoma of the esophagus were treated surgically at the Mayo Clinic. Median age was 58 years and ranged from 26 to 76 years. Symptoms included dysphagia in 11 patients (64.7%) and odynophagia in 6 (35.3%). The tumor was located in the middle third of the esophagus in 10 patients (58.8%) and in the cervical esophagus in 7 (41.2%). Procedures performed included esophagogastrectomy in 9 patients (Ivor Lewis in 5, left thoracoabdominal in 3, and transhiatal in 1), enucleation in 3, transgastric excision in 1, and exploration without resection in 4.

Results. The procedure was considered curative in 11 patients (64.7%). There was one operative death (mortality, 5.9%). Complications occurred in 3 patients (17.6%) and included anastomotic leak in 2 and bleeding requiring reoperation in 1. Growth pattern was infiltrating in 7, polypoid in 5, and intramural in 5. Histologically, the tumor was grade 1 in 6 patients, grade 2 in 2, grade 3 in 7, and grade 4 in 2. The tumor was postsurgically classified as stage I in 2 patients, stage IIA in 7, stage IIB in 1, stage IIIA in 5, stage IV in 1, and unknown in 1. Six patients (35.3%) received adjuvant treatment. Follow-up was complete in 16 patients (94.1%) and ranged from 1 to 182 months (median, 48 months). Five- and 10-year actuarial survivals were 47.0% and 31.0%, respectively. Seven patients (41.2%) are currently alive (median survival, 72 months); all underwent curative resection. Factors affecting survival included completeness of resection, growth pattern, postsurgical stage, tumor grade, and tumor location (p < 0.05).

Conclusions. We conclude that leiomyosarcoma of the esophagus is rare. Complete resection provides long-term survival.

	Introduction

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Leiomyosarcoma of the esophagus was first diagnosed by Howard in 1902 [1], but it was not until 1945 that Harrington [2] performed the first successful resection. Little, however, is known of long-term survival after resection. The purpose of this review is to analyze our experience with resection of leiomyosarcoma of the esophagus.

	Material and methods

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Patient selection
Between January 1920 and December 1996, 6,359 patients with esophageal carcinoma were evaluated at our institution. Nineteen (0.3%) of these patients had histologically proven esophageal leiomyosarcoma. Two of the 19 patients were diagnosed endoscopically and had no further evaluation or treatment. Both were excluded from further analysis. The remaining 17 patients all underwent surgical intervention. Esophageal resection was considered curative if the patient was cancer-free after the operation. The records of these patients were analyzed for age, sex, signs and symptoms at presentation, type of surgical procedure, postoperative therapy, postsurgical stage, tumor grade, and long-term survival. All tumors were staged by the TNM classification system of the American Joint Committee for Cancer Staging and End-Results Reporting [3]. Histologic grade was according to Broder’s classification. Operative mortality included patients who died within 30 days after operation or during the same hospitalization. Follow-up data were obtained from the patient’s most recent clinic visit. Survival was estimated by the Kaplan-Meier method using the date of operation as the starting point [4]. The influence of variables on survival was analyzed using the log-rank test for discrete variables [5]. A p value less than 0.05 was considered significant.

Clinical findings
There were 9 men and 8 women. Median age was 58 years (range, 26 to 76 years). Preoperative signs and symptoms were present in 15 patients (88.2%) and included dysphagia in 11 (64.7%), odynophagia in 6 (35.3%), back pain in 2, weight loss in 2, hoarseness and cough in 2, and anemia in 1. The median duration of symptoms was 3 months (range, 0 to 12 months). No patients received neoadjuvant therapy. Three patients had prior surgery related to their leiomyosarcoma. Two patients had metastatic leiomyosarcoma removed before identification of the primary lesion; one had a liver nodule removed 4 years before, whereas another had a pulmonary nodule resected 5 months before. The third patient had an esophageal leiomyoma enucleated 6 years before esophagectomy.

A curative resection was done in 11 patients (64.7%), which included 9 esophagogastrectomies and 2 local excisions (transgastric excision and enucleation). The esophagogastrectomy was performed through an Ivor Lewis approach in 5 patients, left thoracoabdominal in 3, and a transhiatal in 1. An incomplete resection was done in 6 patients (35.3%), which included 4 explorations and 2 enucleations. The 4 patients were explored without resection because of invasion of contiguous mediastinal or cervical structures. A pyloromyotomy was done in 3 patients (17.6%) and a pyloroplasty in 2 (11.8%). In an additional patient, a tracheostomy was done to protect the airway because of unresectable cervical disease.

The tumor was located in the middle third of the esophagus in 10 patients (58.8%) and in the cervical region in 7 (41.2%). Grossly, the growth pattern was infiltrating in 7 patients (41.2%), intramural in 5 (29.4%), and polypoid in 5 (29.4%). Two patients were postsurgical stage I (11.7%), 7 were stage IIA (41.2%), 1 was stage IIB (5.9%), 5 were stage III (29.4%), and 1 was stage IV (5.9%). Postsurgical stage was unknown in 1 patient (5.9%). Histologically, the tumor was grade 1 in 6 patients (35.4%), grade 2 in 2 (11.7%), grade 3 in 7 (41.2%), and grade 4 in 2 (11.7%). Six patients underwent additional treatment postoperatively, which included chemotherapy in 3, chemoradiation in 2, and radiation alone in 1.

Postoperative complications occurred in 3 patients (17.6%); 2 had an anastomotic leak and 1 required reoperation for postoperative bleeding. There was one operative death (mortality, 5.9%). Cause of death was sepsis after an anastomotic leak. Median hospitalization was 13 days and ranged from 2 to 45 days.

	Results

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Follow-up was available in 16 patients (94.1%) and ranged from 1 to 182 months (median, 48 months). Seven patients, all with curative resection, were alive and free of disease at a median survival of 72 months (range, 2 to 182 months) (Table 1). Two other patients were alive with recurrent sarcoma. The remaining 7 patients all died of recurrent tumor. The recurrence was disseminated in 6 patients and regional in 3.

View larger version (7K): [in this window] [in a new window]   Fig 1. Overall probability of survival with leiomyosarcoma of the esophagus after surgical treatment. Zero time on the abscissa represents day of operation.

	Comment

Top Abstract Introduction Material and methods Results Comment References

 
Leiomyosarcoma of the esophagus is the most common sarcoma of the esophagus [6]. It accounts for 0.5% of all esophageal and for 5% of gastrointestinal sarcomas [7]. Leiomyosarcomas are observed more commonly in men than in women and are equally distributed throughout the esophagus [6]. We found no sex predilection in our series nor were any definite predisposing factors identified [8].

The histologic distinction between well-differentiated leiomyosarcoma and leiomyoma can be difficult [6, 9, 10]. Clinical behavior and gross appearance are the only reliable indications of the malignant nature of these lesions [6]. The slow growth rate of leiomyosarcomas combined with the late onset of obstructive symptoms may falsely reinforce the clinical suspicion of benignity. Any pedunculated esophageal lesion, however, should be considered as potentially malignant [6].

Clinical findings are usually nonspecific [10, 11]. Like others [8, 10], we found that dysphagia is the most common symptom. An intramural mass is present in most cases on barium swallow, which may also reveal the presence of a sinus tract between the esophageal lumen and a cavitated tumor [10]. The sensitivity of endoscopic biopsies depends on the protrusion of the mass into the lumen [11, 12]. Intraoperative pathologic analysis of the specimen may be inconclusive, forcing the surgeon to decide whether resection is necessary based only on the macroscopic appearance of the tumor [6, 12].

Because curative resection offered a significant survival benefit to the patients in our series, we believe that esophagectomy should be the standard management for most patients with esophageal leiomyosarcoma [8, 13, 14]. Nonetheless, local excision did achieve long-term survival in some of our patients. One of our patients was alive and free of disease 108 months after local excision of a well-differentiated polypoid leiomyosarcoma. Others have reported similar findings [9, 15].

Polypoid and intramural growths have shown improved long-term survival compared with infiltrating lesions [11]. Likewise, intrathoracic leiomyosarcomas have fared better than cervical tumors. Finally, patients with well-differentiated leiomyosarcoma have a significantly improved long-term survival after resection. Contrary to other reports [16], adjuvant therapy did not have an impact on long-term survival in our patients.

In a recent metaanalysis study, Koga and associates [11] reported a 1-, 3-, and 5-year survival of 60.3%, 42.8%, and 32.1%, respectively. Patient selection, however, may partially explain our favorable results as 47% of our patients had well-differentiated leiomyosarcomas, 53.0% had early stage (I and IIA), and 59.0% had either an intramural or polypoid growth pattern.

Our study demonstrated that the most common pattern of failure is either local recurrence or distant metastasis to the lung or to the liver. Others have reported similar findings [13]. The finding of metastatic disease at presentation, however, should not contraindicate surgical excision of the primary tumor because significant palliation and long-term survival still can be achieved [17, 18]. Resection of liver metastases can also be successfully accomplished years after removal of the primary esophageal tumor [17].

In conclusion, esophageal leiomyosarcoma is a rare malignancy. Surgical resection can be done with low morbidity and mortality. Once the diagnosis is established, complete resection should be performed whenever possible to achieve maximal benefit.

	References

Top Abstract Introduction Material and methods Results Comment References

 

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This Article Abstract Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Gaetano Rocco Victor F. Trastek Claude Deschamps Mark S. Allen Daniel L. Miller Peter C. Pairolero Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Rocco, G. Articles by Pairolero, P. C. Search for Related Content PubMed PubMed Citation Articles by Rocco, G. Articles by Pairolero, P. C.