The unresolved case of sacral chordoma: from misdiagnosis to challenging surgery and medical therapy resistance.

Details

Serval ID
serval:BIB_4797ADD9E026
Type
Article: article from journal or magazin.
Collection
Publications
Title
The unresolved case of sacral chordoma: from misdiagnosis to challenging surgery and medical therapy resistance.
Journal
Annals of Coloproctology
Author(s)
Garofalo F., Christoforidis D., di Summa P.G., Gay B., Cherix S., Raffoul W., Demartines N., Matter M.
ISSN
2287-9714 (Print)
ISSN-L
2287-9714
Publication state
Published
Issued date
2014
Volume
30
Number
3
Pages
122-131
Language
english
Notes
Publication types: Journal Article
Publication Status: ppublish
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.
Pubmed
Open Access
Yes
Create date
10/10/2016 8:57
Last modification date
02/06/2020 6:26
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