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The Unresolved Case of Sacral Chordoma: From Misdiagnosis to Challenging Surgery and Medical Therapy Resistance

대한대장항문학회지 2014년 30권 3호 p.122 ~ 131
 ( Garofalo Fabio ) - Intercantonal Hospital Broye Department of Surgery

 ( Christoforidis Dimitrios ) - University Hospital of Lausanne Department of Visceral Surgery
 ( Demartines Nicolas ) - University Hospital of Lausanne Department of Visceral Surgery
 ( Matter Maurice ) - University Hospital of Lausanne Department of Visceral Surgery
 ( Summa Pietro G. di ) - University Hospital of Lausanne Department of Plastic and Reconstructive Surgery
 ( Raffoul Wassim ) - University Hospital of Lausanne Department of Plastic and Reconstructive Surgery
 ( Gay Beatrice ) - University Hospital of Lausanne Department of Oncology
 ( Cherix Stephane ) - University Hospital of Lausanne Department of Orthopedics

Abstract


Purpose: A sacral chordoma is a rare, slow-growing, primary bone tumor, arising from embryonic notochordal remnants. Radical surgery is the only hope for cure. The aim of our present study is to analyse our experience with the challenging treatment of this rare tumor, to review current treatment modalities and to assess the outcome based on R status.

Methods: Eight patients were treated in our institution between 2001 and 2011. All patients were discussed by a multidisciplinary tumor board, and an en bloc surgical resection by posterior perineal access only or by combined anterior/posterior accesses was planned based on tumor extension.

Results: Seven patients underwent radical surgery, and one was treated by using local cryotherapy alone due to low performance status. Three misdiagnosed patients had primary surgery at another hospital with R1 margins. Reresection margins in our institution were R1 in two and R0 in one, and all three recurred. Four patients were primarily operated on at our institution and had en bloc surgery with R0 resection margins. One had local recurrence after 18 months. The overall morbidity rate was 86% (6/7 patients) and was mostly related to the perineal wound. Overall, 3 out of 7 resected patients were disease-free at a median follow-up of 2.9 years (range, 1.6-8.0 years).

Conclusion: Our experience confirms the importance of early correct diagnosis and of an R0 resection for a sacral chordoma invading pelvic structures. It is a rare disease that requires a challenging multidisciplinary treatment, which should ideally be performed in a tertiary referral center.

키워드

Sacrum;Chordoma;perineum
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