Display Settings:

Format

Send to:

Choose Destination

Gastrointestinal stroma tumor(GIST)

MedGen UID:
451979
Concept ID:
CN117614
Finding
Synonyms: Gastrointestinal stromal tumor; GASTROINTESTINAL STROMAL TUMORS; GIST
 
HPO: HP:0100723

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews
  • CROGGastrointestinal stroma tumor

Conditions with this feature

Gastrointestinal Stromal Tumors
MedGen UID:
116049
Concept ID:
C0238198
Neoplastic Process
Gastrointestinal stromal tumors are mesenchymal tumors found in the gastrointestinal tract that originate from the interstitial cells of Cajal, the pacemaker cells that regulate peristalsis in the digestive tract. Approximately 70% of GISTs develop in the stomach, 20% in the small intestine, and less than 10% in the esophagus, colon, and rectum. GISTs are typically more cellular than other gastrointestinal sarcomas. They occur predominantly in patients who are 40 to 70 years old but in rare cases may occur in younger persons (Miettinen et al., 1999, 1999). GISTs can also be seen in neurofibromatosis-1 (NF1; 162200) due to mutations in the NF1 gene, and are thus distinct from the GISTs described here. Sandberg and Bridge (2002) reviewed the cytogenetics and molecular genetics of gastrointestinal stromal tumors. Coffey et al. (2007) reviewed the clinical features, pathogenesis, and molecular treatments of Menetrier disease (137280) and GIST, both of which are hyperproliferative disorders of the stomach caused by dysregulated receptor tyrosine kinases.
Paragangliomas 4
MedGen UID:
349380
Concept ID:
C1861848
Disease or Syndrome
Hereditary paraganglioma-pheochromocytoma (PGL/PCC) syndromes are characterized by paragangliomas (tumors that arise from neuroendocrine tissues symmetrically distributed along the paravertebral axis from the base of the skull to the pelvis) and by pheochromocytomas (paragangliomas that are confined to the adrenal medulla). Sympathetic paragangliomas hypersecrete catecholamines; parasympathetic paragangliomas are most often nonsecretory. Extra-adrenal parasympathetic paragangliomas are located predominantly in the skull base, neck, and upper medistinum; approximately 95% of such tumors are nonsecretory. In contrast, sympathetic extra-adrenal paragangliomas are generally confined to the lower mediastinum, abdomen, and pelvis, and are typically secretory. Pheochromocytomas, which arise from the adrenal medulla, typically hypersecrete catecholamines. Symptoms of PGL/PCC result either from mass effects or catecholamine hypersecretion (e.g., sustained or paroxysmal elevations in blood pressure, headache, episodic profuse sweating, forceful palpitations, pallor, and apprehension or anxiety). The risk for malignant transformation is greater for extra-adrenal sympathetic paragangliomas than for pheochromocytomas or skull base and neck paragangliomas.

Recent clinical studies

Etiology

Seidel C, Fenner M, Länger F, Bantel H, Ganser A, Grünwald V
BMC Cancer 2012 May 21;12:186. doi: 10.1186/1471-2407-12-186. [Epub ahead of print] PMID: 22612794Free PMC Article
Freidl T, Lackner H, Huber J, Sovinz P, Moser A, Schroettner B, Schwinger W, Urban C
Klin Padiatr 2008 Nov-Dec;220(6):384-7. Epub 2008 Oct 23 doi: 10.1055/s-0028-1083807. PMID: 18949676
Bogoevski D, Mann O, Schurr P, Izbicki JR, Strate T
JSLS 2007 Jul-Sep;11(3):394-7. PMID: 17931527Free PMC Article
Meyer L, Meyer F, Dralle H, Ernst M, Lippert H, Gastinger I; East German Study Group for Quality Control in Operative Medicine and Regional Development in Surgery
Langenbecks Arch Surg 2005 Nov;390(6):510-6. Epub 2005 Aug 6 doi: 10.1007/s00423-005-0575-2. [Epub ahead of print] PMID: 16086189
Sakurai S, Oguni S, Hironaka M, Fukayama M, Morinaga S, Saito K
Jpn J Cancer Res 2001 May;92(5):494-8. PMID: 11376557

Diagnosis

Seidel C, Fenner M, Länger F, Bantel H, Ganser A, Grünwald V
BMC Cancer 2012 May 21;12:186. doi: 10.1186/1471-2407-12-186. [Epub ahead of print] PMID: 22612794Free PMC Article
Cho H, Watanabe T, Aoyama T, Hayashi T, Yamada T, Ogata T, Yoshikawa T, Tsuburaya A, Sekiguchi H, Nakamura Y, Sakuma Y, Kameda Y, Miyagi Y
Int J Clin Oncol 2012 Jun;17(3):294-8. Epub 2011 Aug 18 doi: 10.1007/s10147-011-0296-1. [Epub ahead of print] PMID: 21850386
Millonig G, Giesel FL, Reimann FM, Topalidis T, Seitz HK, Mueller S
Z Gastroenterol 2010 Apr;48(4):479-81. Epub 2009 Dec 2 doi: 10.1055/s-0028-1109604. [Epub ahead of print] PMID: 20352594
Freidl T, Lackner H, Huber J, Sovinz P, Moser A, Schroettner B, Schwinger W, Urban C
Klin Padiatr 2008 Nov-Dec;220(6):384-7. Epub 2008 Oct 23 doi: 10.1055/s-0028-1083807. PMID: 18949676
Egberts JH, Scharrer ML, Hinz S, Schafmayer C, Klomp HJ, Faendrich F, Tepel J
Hepatogastroenterology 2007 Jan-Feb;54(73):129-34. PMID: 17419246

Therapy

Seidel C, Fenner M, Länger F, Bantel H, Ganser A, Grünwald V
BMC Cancer 2012 May 21;12:186. doi: 10.1186/1471-2407-12-186. [Epub ahead of print] PMID: 22612794Free PMC Article
Bogoevski D, Mann O, Schurr P, Izbicki JR, Strate T
JSLS 2007 Jul-Sep;11(3):394-7. PMID: 17931527Free PMC Article
Dedes KJ, Schiesser M, Schäfer M, Clavien PA
J Gastrointest Surg 2006 Jan;10(1):123-7. doi: 10.1016/j.gassur.2005.04.010. PMID: 16368501
Meyer L, Meyer F, Dralle H, Ernst M, Lippert H, Gastinger I; East German Study Group for Quality Control in Operative Medicine and Regional Development in Surgery
Langenbecks Arch Surg 2005 Nov;390(6):510-6. Epub 2005 Aug 6 doi: 10.1007/s00423-005-0575-2. [Epub ahead of print] PMID: 16086189

Prognosis

Freidl T, Lackner H, Huber J, Sovinz P, Moser A, Schroettner B, Schwinger W, Urban C
Klin Padiatr 2008 Nov-Dec;220(6):384-7. Epub 2008 Oct 23 doi: 10.1055/s-0028-1083807. PMID: 18949676
Bogoevski D, Mann O, Schurr P, Izbicki JR, Strate T
JSLS 2007 Jul-Sep;11(3):394-7. PMID: 17931527Free PMC Article
Egberts JH, Scharrer ML, Hinz S, Schafmayer C, Klomp HJ, Faendrich F, Tepel J
Hepatogastroenterology 2007 Jan-Feb;54(73):129-34. PMID: 17419246
Sakurai S, Oguni S, Hironaka M, Fukayama M, Morinaga S, Saito K
Jpn J Cancer Res 2001 May;92(5):494-8. PMID: 11376557

Clinical prediction guides

Cho H, Watanabe T, Aoyama T, Hayashi T, Yamada T, Ogata T, Yoshikawa T, Tsuburaya A, Sekiguchi H, Nakamura Y, Sakuma Y, Kameda Y, Miyagi Y
Int J Clin Oncol 2012 Jun;17(3):294-8. Epub 2011 Aug 18 doi: 10.1007/s10147-011-0296-1. [Epub ahead of print] PMID: 21850386
Cohen JD, Babiarz JE, Abrams RM, Guo L, Kameoka S, Chiao E, Taunton J, Kolaja KL
Toxicol Appl Pharmacol 2011 Nov 15;257(1):74-83. Epub 2011 Aug 27 doi: 10.1016/j.taap.2011.08.020. [Epub ahead of print] PMID: 21906609
Meyer L, Meyer F, Dralle H, Ernst M, Lippert H, Gastinger I; East German Study Group for Quality Control in Operative Medicine and Regional Development in Surgery
Langenbecks Arch Surg 2005 Nov;390(6):510-6. Epub 2005 Aug 6 doi: 10.1007/s00423-005-0575-2. [Epub ahead of print] PMID: 16086189
Sakurai S, Oguni S, Hironaka M, Fukayama M, Morinaga S, Saito K
Jpn J Cancer Res 2001 May;92(5):494-8. PMID: 11376557

Supplemental Content

Table of contents

    Consumer resources

    Outreach and support

    Clinical Trials

    Recent activity

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...