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Diane Mege, Lieven Depypere, Guillaume Piessen, Annelijn E Slaman, Bas P L Wijnhoven, Arnulf Hölscher, Magnus Nilsson, Mark I Van Berge Henegouwen, J Jan B Van Lanschot, Wolfgang Schroeder, Pascal Thomas, Philippe Nafteux, Xavier Benoit D'Journo, RA05.01: SURGICAL MANAGEMENT OF ESOPHAGEAL SARCOMA: A MULTICENTER EUROPEAN EXPERIENCE, Diseases of the Esophagus, Volume 31, Issue Supplement_1, September 2018, Pages 26–27, https://doi.org/10.1093/dote/doy089.RA05.01
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Abstract
Esophageal sarcomas (ES) are rare and evidence in literature is scarce making their management difficult. The objective is to report surgical and oncological outcomes of ES in a large multicenter European cohort.
This is a retrospective multicenter study including all patients who underwent en-bloc esophagectomy for ES in 7 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 ES 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 5 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). Median tumor length was 5 cm [1–10]. Microscopic R1 resection was present in 1 patient (5%) and 7 patients (33%) were N + . Median follow-up was 16 (3–79) months in 20 of 21 patients (95%). One-, 3- and 5-year overall survival (OS) rates were 74%, 43% and 35%, respectively. One-, 3- and 5-years disease-free survival (DFS) rates were 58%, 40% and 33% respectively. Median overall survival (OS) was 33 months for patients with a tumor ≤ 5 cm and 13 months for patients with a tumor > 5 cm (P = 0.54). Median OS was 6 months in N + patients vs. 37 months for N0 patients (P = 0.06). At the end of the follow-up period, 9 patients had died from cancer recurrences (43%), 3 patients from other reasons (14%), 1 patient was still alive with recurrence (5%) and the 7 remaining patients were free of disease (33%). Recurrence was local (n = 3), metastatic (n = 3) or both (n = 4).
Carcinosarcoma and leiomyosarcoma were the most common ES histological sub-types. Tumor size and N + disease seemed prognosis factors. Transthoracic en-bloc esophagectomy with radical lymphadenectomy should be recommended to achieve complete resection. Long-term survival remained poor with a high local and distant recurrence rate.
All authors have declared no conflicts of interest.
