Evaluation of malignancy and prognosis of gastrointestinal stromal tumors: A review

Markku Miettinen, Wael El-Rifai, Leslie Sobin, Jerzy Lasota

Research output: Contribution to journalArticle

537 Citations (Scopus)

Abstract

This paper reviews data on the prognosis of gastrointestinal stromal tumors (GISTs). These tumors are specific KIT-expressing and KIT-signaling-driven mesenchymal tumors, many of which have KIT-activating mutations. GISTs occur in the entire gastrointestinal (GI) tract and may also arise from the omentum, mesenteries, and retroperitoneum. They range from small benign tumors to sarcomas at all sites of occurrence. A KIT tyrosine kinase inhibitor, STI-571 (imatinib [Gleevec]; Novartis, Basel, Switzerland), has recently shown promise in the treatment of metastatic GISTs. Understanding the natural history of GIST before introduction of STI-571 will help assess the impact and position of this new treatment. The frequency of benign versus malignant GISTs varies between sites. Benign GISTs outnumber malignant GISTs in the stomach, whereas malignant GISTs are more common in the intestines. Tumors that have metastasized at presentation have a very poor prognosis. Traditionally, the 3 key prognostic factors have been mitotic rate, tumor size, and site. Tumors that are small (≤2 cm) and show mitotic activity not exceeding 5 mitoses per 50 high-power fields (HPFs) have an excellent prognosis, probably independent of site, although this has not been shown specifically for all sites. In the stomach, most epithelioid GISTs are benign, provided that mitotic counts do not exceed 5/50 HPFs. However, a small proportion of tumors apparently lacking mitotic activity do metastasize. Tumors with a mitotic rate >5/50 HPFs usually have a malignant behavior. The Ki67 index may help identify tumors with malignant potential, but large site-specific series are not yet available. Genetic markers, including DNA-copy number changes, telomerase activity, and KIT mutation status, may be useful in more accurately identifying tumors with malignant potential.

Original languageEnglish (US)
Pages (from-to)478-483
Number of pages6
JournalHuman Pathology
Volume33
Issue number5
DOIs
StatePublished - Jan 1 2002
Externally publishedYes

Fingerprint

Gastrointestinal Stromal Tumors
Neoplasms
Stomach
DNA Copy Number Variations
Mutation
Omentum
Mesentery
Telomerase
Switzerland
Genetic Markers
Mitosis
Sarcoma
Protein-Tyrosine Kinases
Intestines
Gastrointestinal Tract

Keywords

  • Allelic losses
  • Gastrointestinal stromal tumor
  • KIT mutation
  • Prognosis
  • Sarcoma

ASJC Scopus subject areas

  • Pathology and Forensic Medicine

Cite this

Evaluation of malignancy and prognosis of gastrointestinal stromal tumors : A review. / Miettinen, Markku; El-Rifai, Wael; Sobin, Leslie; Lasota, Jerzy.

In: Human Pathology, Vol. 33, No. 5, 01.01.2002, p. 478-483.

Research output: Contribution to journalArticle

Miettinen, Markku ; El-Rifai, Wael ; Sobin, Leslie ; Lasota, Jerzy. / Evaluation of malignancy and prognosis of gastrointestinal stromal tumors : A review. In: Human Pathology. 2002 ; Vol. 33, No. 5. pp. 478-483.
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abstract = "This paper reviews data on the prognosis of gastrointestinal stromal tumors (GISTs). These tumors are specific KIT-expressing and KIT-signaling-driven mesenchymal tumors, many of which have KIT-activating mutations. GISTs occur in the entire gastrointestinal (GI) tract and may also arise from the omentum, mesenteries, and retroperitoneum. They range from small benign tumors to sarcomas at all sites of occurrence. A KIT tyrosine kinase inhibitor, STI-571 (imatinib [Gleevec]; Novartis, Basel, Switzerland), has recently shown promise in the treatment of metastatic GISTs. Understanding the natural history of GIST before introduction of STI-571 will help assess the impact and position of this new treatment. The frequency of benign versus malignant GISTs varies between sites. Benign GISTs outnumber malignant GISTs in the stomach, whereas malignant GISTs are more common in the intestines. Tumors that have metastasized at presentation have a very poor prognosis. Traditionally, the 3 key prognostic factors have been mitotic rate, tumor size, and site. Tumors that are small (≤2 cm) and show mitotic activity not exceeding 5 mitoses per 50 high-power fields (HPFs) have an excellent prognosis, probably independent of site, although this has not been shown specifically for all sites. In the stomach, most epithelioid GISTs are benign, provided that mitotic counts do not exceed 5/50 HPFs. However, a small proportion of tumors apparently lacking mitotic activity do metastasize. Tumors with a mitotic rate >5/50 HPFs usually have a malignant behavior. The Ki67 index may help identify tumors with malignant potential, but large site-specific series are not yet available. Genetic markers, including DNA-copy number changes, telomerase activity, and KIT mutation status, may be useful in more accurately identifying tumors with malignant potential.",
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