Clinical Practice Guideline for Adequate Diagnosis and Effective Treatment of Gastrointestinal Stromal Tumor in Korea

Yoon-Koo Kang

doi:10.5124/jkma.2007.50.9.830

J Korean Med Assoc > Volume 50(9); 2007 > Article

Kang: Clinical Practice Guideline for Adequate Diagnosis and Effective Treatment of Gastrointestinal Stromal Tumor in Korea

Continuing Education Column

J Korean Med Assoc 2007; 50(9): 830-841.

Published online: January 20, 2007

DOI: http://dx.doi.org/10.5124/jkma.2007.50.9.830

Clinical Practice Guideline for Adequate Diagnosis and Effective Treatment of Gastrointestinal Stromal Tumor in Korea

Yoon-Koo Kang, MD

Devision of Oncology / Department of Internal Medicine, Ulsan University College of Medicine E-mail: ykkang@amc.seoul.kr

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Despite the rarity in incidence and prevalence, gastrointestinal stromal tumor (GIST) has emerged as a distinct pathogenetic entity, and the clinical management of GIST has been evolving very rapidly due to the recent recognition of its oncogenic signal transduction pathway and the introduction of new molecular?targeted therapy. Successful management of GIST in localized and advanced stages requires a multidisciplinary approach firmly based on accurate histopathologic diagnosis. However, standardized guidelines for the management of Korean GIST patients do not exist. This study was performed to provide a guideline for standardized diagnosis and treatment for GIST in Korea. Expert panel members of the Korean GIST Study Group (KGSG) thoroughly reviewed the relevant literature including European Society of Medical Oncology and National Comprehensive Cancer Network guidelines and shared their experience and opinions to make a consensus on twenty?five topics related with pathologic diagnosis, surgical management, and medical treatment of GIST. The consensus described in this article was presented as the basis for a guideline of diagnosis and treatment for patients with GIST that would be used to facilitate the optimal clinical practice in Korea.

Figure 1.

Photograph of the representative findings of gastrointestinal stromal tumors (GISTs). A) Epithelioid type GIST. B) Spindle cell type GIST. C) Mixed epithelioid and spindle cell type GIST. D) Hyaline changes observed in GIST. E) Myxoid changes observed in GIST. F) Ischemic tumor necrosis observed in GIST.

Figure 2.

Immunohistochemical staining of c?kit in gastrointestinal stromal tumor. c?kit is diffusely positive in the cytoplasm and membranes (A&B). In this picture, internal negative control is vascular endothelial cells. Sometimes, c?kit is more intensely stained along the membrane (C). Less frequently, perinuclear golgi?like staining can be observed (D).

Figure 3.

Algorithm to diagnose gastrointestinal stromal tumors based on immunohistochemistry.

Table 1.

Proposed approach for defining risk of aggressive behavior in GISTs

	Size	Mitotic Count
Very low risk	< 2 cm	≤ 5 / 50 HPF
Low risk	2~5 cm	≤ 5 / 50 HPF
Intermediate risk	< 5 cm	6~10 / 50 HPF
Intermediate risk	5~10 cm	≤ 5 / 50 HPF
High risk	> 5 cm	> 5 / 50 HPF
	>10 cm	Any mitotic rate
	Any size	>10 /50 HPF

Abbreviation: HPF, high?power field.

Table 2.

Risk stratification of primary GISTs^∗

Tumor features		Risk of tumor progression
Mitotic index	Size (cm)	Stomach	Small bowel
≥ 5 / 50 HPF	≤ 2	Very low	Very low
	> 2 and ≤ 5	Very low	Low
	> 5 and ≤ 10	Low	Moderate
	> 10	Moderate	High
≥ 5 / 50 HPF	≤ 2	Very low	Moderate
	> 2 and ≤ 5	Moderate	High
	>5 and ≤10	High	High
	>10	High	High

∗ GISTs arising at other anatomical sites should probably be stratified in a similar fashion as small bowel tumors

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