8936/1 GIST, NOS



Definitions

Stomach
ICD-O-3 topography code: C16

The term gastrointestinal stromal tumour (GIST) is used for a specific group of tumours comprising the majority of gastrointestinal mesenchymal tumours, including those previously termed leiomyoma, cellular leiomyoma, leiomyoblastoma and leiomyosarcoma 1
 
Hamilton SR, Aaltonen LA (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of the Digestive System.
3rd Edition
IARC Press: Lyon 2000



. Rare cases involving tissues outside the gastrointestinal tract have also been reported (extragastrointestinal gastrointestinal stromal tumors).

Gastric GIST
Approximately 60% of GISTs arise in the stomach
2
Click to access Pubmed
Nilsson B, BŁmming P, Meis-Kindblom JM, Odťn A, Dortok A, Gustavsson B, Sablinska K, Kindblom LG (2005)
Gastrointestinal stromal tumors: the incidence, prevalence, clinical course, and prognostication in the preimatinib mesylate era--a population-based study in western Sweden.
Cancer 103: 821-9



; they are the most common primary mesenchymal tumour of the gastrointestinal tract GIST spans a clinical spectrum from benign to malignant; it has been estimated that about 25% of gastric GISTs (not counting minimal incidental tumours) are clinically malignant. The median age at manifestation is 60-65 years. GIST are KIT-expressing and KIT signaling-driven mesenchymal tumors respond favourably to treatment with the KIT tyrosine kinase inhibitor imatinib mesylate / gleevec 3
Click to access Pubmed
Druker BJ (2004)
Imatinib as a paradigm of targeted therapies.
Adv Cancer Res 91: 1-30



.



Small intestine
ICD-O-3 topography code: C17

The term gastrointestinal stromal tumour (GIST) is used for a specific group of tumours comprising the majority of gastrointestinal mesenchymal tumours, including those previously termed leiomyoma, cellular leiomyoma, leiomyoblastoma and leiomyosarcoma
4
 
Hamilton SR, Aaltonen LA (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of the Digestive System.
3rd Edition
IARC Press: Lyon 2000



. Rare cases involving tissues outside the gastrointestinal tract have also been reported (extragastrointestinal gastrointestinal stromal tumors).
GIST are KIT-expressing and KIT-signaling-driven mesenchymal tumors that repsond favourably to treatment with the KIT tyrosine kinase inhibitor imatinib mesylate / Gleevec
5
Click to access Pubmed
Druker BJ (2004)
Imatinib as a paradigm of targeted therapies.
Adv Cancer Res 91: 1-30



.
Small intestinal and duodenal GISTs comprise about 30% and 5%, respectively, of all GISTs
6
 
Bosman FT, Carneiro F, Hruban RH, Theise ND (Eds.)
WHO Classification of Tumours of the Digestive System.
4th Edition
International Agency for Research on Cancer: Lyon 2010



.



Colon and rectum
ICD-O-3 topography code: C18-C20

The term gastrointestinal stromal tumour (GIST) is used for a specific group of tumours comprising the majority of gastrointestinal mesenchymal tumours, including those previously termed leiomyoma, cellular leiomyoma, leiomyoblastoma and leiomyosarcoma
7
 
Hamilton SR, Aaltonen LA (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of the Digestive System.
3rd Edition
IARC Press: Lyon 2000



. Rare cases involving tissues outside the gastrointestinal tract have also been reported (extragastrointestinal gastrointestinal stromal tumors). GIST are KIT-expressing and KIT-signaling-driven mesenchymal tumors that repsond favourably to treatment with the KIT tyrosine kinase inhibitor imatinib mesylate / Gleevec 8
Click to access Pubmed
Druker BJ (2004)
Imatinib as a paradigm of targeted therapies.
Adv Cancer Res 91: 1-30



.

GISTs of the colon and rectum
GISTs are very rare in the colon (about 1% of all GISTs) and have a predilection for the sigmoid. However, microscopic GISTs have been detected in 0.2% of retrospectively examined sigmoid-colon resections. Rectal GISTs comprise 4% of all GISTs and occur in any segment. These tumours vary from incidentally detected small mural nodules to large, complex pelvic masses that can cause intestinal obstruction or gastrointestinal bleeding. Those with anterior extension can abut the prostate gland and clinically simulate prostate cancer
9
Click to access Pubmed
Agaimy A, WŁnsch PH, Dirnhofer S, Bihl MP, Terracciano LM, Tornillo L (2008)
Microscopic gastrointestinal stromal tumors in esophageal and intestinal surgical resection specimens: a clinicopathologic, immunohistochemical, and molecular study of 19 lesions.
Am J Surg Pathol 32: 867-73



10
Click to access Pubmed
Hassan I, You YN, Shyyan R, Dozois EJ, Smyrk TC, Okuno SH, Schleck CD, Hodge DO, Donohue JH (2008)
Surgically managed gastrointestinal stromal tumors: a comparative and prognostic analysis.
Ann Surg Oncol 15: 52-9



11
Click to access Pubmed
Miettinen M, Furlong M, Sarlomo-Rikala M, Burke A, Sobin LH, Lasota J (2001)
Gastrointestinal stromal tumors, intramural leiomyomas, and leiomyosarcomas in the rectum and anus: a clinicopathologic, immunohistochemical, and molecular genetic study of 144 cases.
Am J Surg Pathol 25: 1121-33



12
Click to access Pubmed
Miettinen M, Sarlomo-Rikala M, Sobin LH, Lasota J (2000)
Gastrointestinal stromal tumors and leiomyosarcomas in the colon: a clinicopathologic, immunohistochemical, and molecular genetic study of 44 cases.
Am J Surg Pathol 24: 1339-52



.
Most colonic GISTs are advanced tumours when detected, and have a poor prognosis. The behaviour of rectal GISTs is often aggressive, and even small tumours of < 2 cm in size with mitotic activity can recur and metastasize. Pelvic extension and liver metastasis is common, and bone metastases may develop more often than in gastric and small-intestinal GISTs
13
 
Bosman FT, Carneiro F, Hruban RH, Theise ND (Eds.)
WHO Classification of Tumours of the Digestive System.
4th Edition
International Agency for Research on Cancer: Lyon 2010



.



Esophagus
ICD-O-3 topography code: C15

The term gastrointestinal stromal tumour (GIST) is used for a specific group of tumours comprising the majority of gastrointestinal mesenchymal tumours, including those previously termed leiomyoma, cellular leiomyoma, leiomyoblastoma and leiomyosarcoma
14
 
Hamilton SR, Aaltonen LA (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of the Digestive System.
3rd Edition
IARC Press: Lyon 2000



. Rare cases involving tissues outside the gastrointestinal tract have also been reported (extragastrointestinal gastrointestinal stromal tumors).
GIST are KIT-expressing and KIT-signaling-driven mesenchymal tumors that repsond favourably to treatment with the KIT tyrosine kinase inhibitor imatinib mesylate / Gleevec
15
Click to access Pubmed
Druker BJ (2004)
Imatinib as a paradigm of targeted therapies.
Adv Cancer Res 91: 1-30



.

GISTs are very rare in the oesophagus and comprise 10?20% of the combined group of smooth-muscle and stromal tumours. Most are clinically detected as intraluminal distal oesophageal masses causing dysphagia, but externally extending oesophageal GISTs can manifest as mediastinal tumours.
Occasional examples have been incidentally detected during radiological screening or surveillance studies, and such tumours can have a good prognosis, an exception among this group of tumours, among which sarcomas predominate
16
 
Bosman FT, Carneiro F, Hruban RH, Theise ND (Eds.)
WHO Classification of Tumours of the Digestive System.
4th Edition
International Agency for Research on Cancer: Lyon 2010



.



Connective, subcutaneous and other soft tissues
ICD-O-3 topography code: C49

The term gastrointestinal stromal tumour (GIST) is used for a specific group of tumours comprising the majority of gastrointestinal mesenchymal tumours, including those previously termed leiomyoma, cellular leiomyoma, leiomyoblastoma and leiomyosarcoma
17
 
Hamilton SR, Aaltonen LA (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of the Digestive System.
3rd Edition
IARC Press: Lyon 2000



. Rare cases involving tissues outside the gastrointestinal tract have also been reported (extragastrointestinal gastrointestinal stromal tumors).
GIST are KIT-expressing and KIT-signaling-driven mesenchymal tumors that repsond favourably to treatment with the KIT tyrosine kinase inhibitor imatinib mesylate / Gleevec
18
Click to access Pubmed
Druker BJ (2004)
Imatinib as a paradigm of targeted therapies.
Adv Cancer Res 91: 1-30



.