Trials and recommendations - Sarcoma
SSG guidelines for referral of soft tissue tumors of the extremities and trunk wall

Soft tissue sarcomas are rare malignancies with a clinical presentation which is often not that which is expected from a malignant tumor. Patients with soft tissue sarcomas as a rule, rather than exception, have no symptoms from the tumor. Pain, tenderness or loss of function is often not present and the person is feeling perfectly healthy. The only sign of a possibly malignant tumor is the mere existence of a lump accidentally noted by the patient. This is why these tumors are often misinterpreted as benign lesions and left without further notice by both patients and physicians. Most tissue sarcomas, about ¾,  are however highly malignant and around 1/3 of  the patients are killed by metastatic disease.

Due to the fact that soft tissue sarcomas are rare, knowledge about these tumors are generally limited within the health care organization. Therefore, diagnosis and treatment should be performed at special centres with multidisciplinary, specific competence in these tumors for optimal care.

The most important thing with guidelines for referral is to spread awareness of the existence of soft tissue sarcomas and that a ”clinically benign” lump may well be highly malignant. Since most soft tissue lumps are, still, benign there has to be guidelines for when to suspect a soft tissue sarcoma. Epidemiologic data for benign and malignant soft tissue tumors shows that the risk for malignancy increases with size and deep location. Apart from these characteristics of the lump there  are no specific features that can be used in descerning the probability of a malignant tumor. Within the SSG we have therefore preferred to keep the guidelines simple.

Hence, we recommend that patients presenting with a tumor or lump  with the following criteria should be referred to a sarcoma center as soon as possible and before any surgical intervention:
All tumors >5 cm
All deep tumors, irrespective of size

Scandinavian Sarcoma Group (SSG) www.ssg-org.net
SSG VII:4 Centralized Registration of Sarcoma Patients in Scandinavia. 1995
SSG XVI Centralized registration of patients with
surgically treated skeletal metastases. 2002
SSG XX

A Scandinavian Sarcoma Group
treatment protocol for adult patients with non-metastatic high-risk soft tissue sarcoma
of the extremities and trunk wall. 2007

   
Italian and Scandinavian Sarcoma Group (ISG/SSG)
ISG/SSG III Treatment protocol for nonmetastatic Ewing´s family tumors. 1999
Since 2008 standard treatment care program.
   
European Sarcoma Groups (ISG, COSS and SSG)
Euroboss I A European treatment protocol for bone sarcoma in patients older than 40 years. 2003
   
European and American Osteosarcoma Study Group (EOI, GPOH, NCRI, SSG)
EURAMOS I

A randomized trial of the European and American Osteosarcoma Study Group to optimize treatment strategies for resectable osteosarcoma based on histological response to pre-operative chemotherapy. 2005.

EURAMOS study protocol can be received by contacting SSG trial centre ssg@med.lu.se. Latest CRF version and corresponding instructions can be downloaded from www.ssg-org.net -For members only.
Details regarding this trial you can find at www.euramos.org

   
Recommendations
Analysis of Genetic Changes in Musculoskeletal Tumors. 3:rd edition. 2000
SSG XVII Version 2 Recommendations for the Diagnosis and Treatment of Intraabdominal, Retroperitoneal, and Uterine Sarcoma. 2008
SSG XIX Recommendations for treatment of metastatic soft-tissueS sarcomas in adult patients. 2004
 

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Last updated: 2010 12 02
Mona Martinsson Svensson
E-post: ocsyd@med.lu.se