gms | German Medical Science

79. Jahresversammlung der Deutschen Gesellschaft für Hals-Nasen-Ohren-Heilkunde, Kopf- und Hals-Chirurgie e. V.

Deutsche Gesellschaft für Hals-Nasen-Ohren-Heilkunde, Kopf- und Hals-Chirurgie e. V.

30.04. - 04.05.2008, Bonn

Evans tumour – the low malignant fibromyxoid sarcoma – a case report of a very rare tumour entity

Meeting Abstract

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  • corresponding author Lars Ingo Fleischer - Department for ENT and HNS, St Joseph Stift, Bremen, Germany
  • Soenke Wilms - Department for ENT and HNS, St Joseph Stift, Bremen, Germany
  • Wolfgang Bergler - Department for ENT and HNS, St Joseph Stift, Bremen, Germany

German Society of Oto-Rhino-Laryngology, Head and Neck Surgery. 79th Annual Meeting of the German Society of Oto-Rhino-Laryngology, Head and Neck Surgery. Bonn, 30.04.-04.05.2008. Düsseldorf, Köln: German Medical Science; 2008. Doc08hno54

Die elektronische Version dieses Artikels ist vollständig und ist verfügbar unter: http://www.egms.de/de/meetings/hno2008/08hno54.shtml

Veröffentlicht: 8. Juli 2008

© 2008 Fleischer et al.
Dieser Artikel ist ein Open Access-Artikel und steht unter den Creative Commons Lizenzbedingungen (http://creativecommons.org/licenses/by-nc-nd/3.0/deed.de). Er darf vervielf&aauml;ltigt, verbreitet und &oauml;ffentlich zug&aauml;nglich gemacht werden, vorausgesetzt dass Autor und Quelle genannt werden.


Gliederung

Text

We present the case of woman with an Evans tumour along with an overview of its histopathology and therapy. This neoplasm was first described 1987 and belongs to the group of low-grade fibromyxoid sarcoma. So far only 40 cases are reported in literature.

It occurs in all ages, primarily in the 3rd decade. The typical localisation is the lower extremity, the groin and head-neck region. The histopathologic diagnosis is quite difficult. It presents a pseudocapsula filled with hypocellular fibroblastic tissue and only very few mitotic figures. Typical is a cytogenetic abnormality, a balanced translocation resulting in a FUS/CREB3L2 fusion gene. In literature radical surgery is pointed out to be the main therapy. The rate of recurrence is said to be 50%.

Our case deals with a 34year old woman who had had surgery in childhood on a benign neck mass. In 2003 she complained about left shoulder pain with paraesthesia radiating into the upper extremity. The MRI showed a tumour mass deep in left level V which was completely excised. A long-standing survey ended up in the histopathologic diagnosis of an Evans Tumour. 2006 and 2007 a local recurrence was found, localised on the left side in between the trapezoid muscle and the erector spinae muscle. These tumours were resected and anytime the diagnosis confirmed the recurrence of the Evans tumour. In 2007 no evidence for distant metastasis were found. Because of the second local recurrence and the difficult resection the patient underwent postoperative radiation therapy with 66 Gray. Actually the patient is still in complete remission.

The conclusion for this kind of tumour should be that lifelong aftercare is absolutely mandatory.


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