Surgical management of esophageal sarcoma: a multicenter European experience. Issue 3 (9th February 2018)

• Record Type: Journal Article
• Authors: Mege, D; Depypere, L; Piessen, G; Slaman, A E; Wijnhoven, B P L; Hölscher, A; Nilsson, M; van Berge Henegouwen, M I; van Lanschot, J J B; Schroeder, W; Thomas, P A; Nafteux, P; D'Journo, X B
• Is Part Of: Diseases of the esophagus. Volume 31:Issue 3(2018)
• Publication Date: 2018-02-09
• Subjects: carcinosarcoma -- esophagectomy -- leiomyosarcoma -- sarcoma; Esophagus -- Diseases -- Periodicals; 616.32
• DOI: 10.1093/dote/dox146
• Languages: English
• ISSNs: 1120-8694

Abstract

SUMMARY: Esophageal sarcomas are rare and evidence in literature is scarce making their management difficult. The objective is to report surgical and oncological outcomes of esophageal sarcoma in a large multicenter European cohort. This is a retrospective multicenter study including all patients who underwent en-bloc esophagectomy for esophageal sarcoma in seven European tertiary referral centers between 1987 and 2016. The main outcomes and measures are pathological results, early and long-term outcomes. Among 10, 936 esophageal resections for cancer, 21 (0.2%) patients with esophageal sarcoma were identified. The majority of tumors was located in the middle ( n = 7) and distal ( n = 9) third of the esophagus. Neoadjuvant chemoradiotherapy was performed in five patients. All the patients underwent en-bloc transthoracic esophagectomy (19 open, 2 minimally invasive). Postoperative mortality occurred in 1 patient (5%). One patient received adjuvant chemotherapy. Definitive pathological results were carcinosarcoma ( n = 7), leiomyosarcoma ( n = 5), and other types of sarcoma ( n = 9). Microscopic R1 resection was present in one patient (5%) and seven patients (33%) had positive lymph nodes. Median follow-up was 16 (3–79) months in 20 of 21 patients (95%). One-, 3-, and 5-year overall survival rates were 74%, 43%, and 35%, respectively. One-, 3- and 5-years disease-free survival rates were 58%, 40%, and 33%, respectively. Median overall survival was 6 months in N+ patients vs. 37 months for N0 patients ( p = 0.06). At the end of the follow-up period, nine patients had died from cancer recurrences (43%), three patients died from other reasons (14%), one patient was still alive with recurrence (5%) and the seven remaining patients were free of disease (33%). Recurrence was local ( n = 3), metastatic ( n = 3), or both ( n = 4). In conclusion, carcinosarcoma and leiomyosarcoma were the most common esophageal sarcoma histological subtypes. Lymph node involvement was seen in one third of cases. A transthoracic en-bloc esophagectomy with radical lymphadenectomy should be the best surgical option to achieve complete resection. Long-term survival remained poor with a high local and distant recurrence rate.