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Int J Clin Exp Pathol 2010;3(5):461-471

Review Article
Gastrointestinal stromal tumors (GIST) from risk stratification systems to the new TNM
proposal: more questions than answers? a review emphasizing the need for a
standardized GIST reporting

Abbas Agaimy

Institute of Pathology, Friedrich-Alexander-University, Erlangen, Germany

Received April 21, 2010, accepted May 3, 2010, available online May 5, 2010

Abstract: Following the successful introduction of the receptor tyrosine kinase inhibitors (TKI) as the mainstay for the treatment of advanced
and metastatic gastrointestinal stromal tumor (GIST), GIST has received a special attention in the recent literature. This resulted in major
achievements on the surgical pathology diagnosis and improved our understanding of the molecular biology of the disease. Availability of the
effective TKI therapy has emphasized the need for a more reliable and reproducible system for assessment of the malignant potential in GIST
to allow for an optimal individualized patient treatment. All of the risk stratification systems proposed so far have emphasized the value of tumor
size, mitotic count and anatomic site for risk estimation, at the same time appreciating the difficulty of classifying individual tumors as either
benign or malignant. The newly proposed UICC TNM classification for GISTs represents the most recent hallmark on this topic; yet its
usefulness remains to be tested in future clinical studies. This review briefly summarizes and discusses the most pertinent risk systems
proposed for assessment of the malignant potential of GIST stressing their advantages and limitations and including some critical remarks on
the newly proposed UICC TNM system for classifying GIST. Most importantly, an emphasis is made on the urgent need for a standardized
approach for histopathological evaluation and reporting of GIST specimens to allow for a reproducible tumor size, mitotic count and tumor
growth pattern, and hence for a better risk classification. (IJCEP1004005).

Key words: GIST, risk classification, KIT, TNM, pediatric GIST, EGIST

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Address all correspondence to:
Abbas Agaimy, MD
Institut für Pathologie
Universitätsklinikum
Krankenhausstrasse 12
91054 Erlangen
Germany
Tel. +49-9131-8522288
Fax +49-9131-8524745
E-mail:
abbas.agaimy@uk-erlangen.de