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Gastrointestinal Stromal Tumor of Uncertain Malignant Potential

MedGen UID:
312388
•Concept ID:
C1704399
•
Neoplastic Process
Synonym: Gastrointestinal stromal tumor, uncertain malignant potential
SNOMED CT: Gastrointestinal stromal tumor, uncertain malignant potential (128755003)

Definition

A gastrointestinal stromal tumor that is characterized by a maximum diameter greater than 5 cm and equal or less than 10 cm (gastric localization), or greater than 2 cm and equal or less than 5 cm (intestinal localization) and no more than 5 mitotic figures per 50 high power fields. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVGastrointestinal Stromal Tumor of Uncertain Malignant Potential

Professional guidelines

PubMed

Gastrointestinal stromal tumors: review on morphology, molecular pathology, diagnostics, prognosis and treatment options.
Paral J, Slaninka I, Kalabova H, Hadzi-Nikolov D
Acta Gastroenterol Belg 2010 Jul-Sep;73(3):349-59. PMID: 21086937
From RECIST to PERCIST: Evolving Considerations for PET response criteria in solid tumors.
Wahl RL, Jacene H, Kasamon Y, Lodge MA
J Nucl Med 2009 May;50 Suppl 1(Suppl 1):122S-50S. doi: 10.2967/jnumed.108.057307. PMID: 19403881Free PMC Article
A guide for the diagnosis and management of gastrointestinal stromal cell tumors.
Sepe PS, Brugge WR
Nat Rev Gastroenterol Hepatol 2009 Jun;6(6):363-71. Epub 2009 Apr 14 doi: 10.1038/nrgastro.2009.43. PMID: 19365407

Recent clinical studies

Etiology

Expression of PIN1 in Gastrointestinal Stromal Tumours and its Clinical Significance.
Jiang L, Cao M, Hu J, Chen J
Anticancer Res 2016 Mar;36(3):1275-80. PMID: 26977025
Gastrointestinal stromal tumors: review on morphology, molecular pathology, diagnostics, prognosis and treatment options.
Paral J, Slaninka I, Kalabova H, Hadzi-Nikolov D
Acta Gastroenterol Belg 2010 Jul-Sep;73(3):349-59. PMID: 21086937
Colorectal gastrointestinal stromal tumor.
Amato A
Tech Coloproctol 2010 Nov;14 Suppl 1:S91-5. doi: 10.1007/s10151-010-0631-y. PMID: 20967481
A guide for the diagnosis and management of gastrointestinal stromal cell tumors.
Sepe PS, Brugge WR
Nat Rev Gastroenterol Hepatol 2009 Jun;6(6):363-71. Epub 2009 Apr 14 doi: 10.1038/nrgastro.2009.43. PMID: 19365407
Gastrointestinal stromal tumours.
Miettinen M, Sarlomo-Rikala M, Lasota J
Ann Chir Gynaecol 1998;87(4):278-81. PMID: 9891765

Diagnosis

Mesenchymal tumors of the prostate.
McKenney JK
Mod Pathol 2018 Jan;31(S1):S133-142. doi: 10.1038/modpathol.2017.155. PMID: 29297486
Duodenal gastrointestinal stromal tumors: review on clinical and surgical aspects.
Cavallaro G, Polistena A, D'Ermo G, Pedullà G, De Toma G
Int J Surg 2012;10(9):463-5. Epub 2012 Aug 30 doi: 10.1016/j.ijsu.2012.08.015. PMID: 22939976
Gastrointestinal stromal tumors: review on morphology, molecular pathology, diagnostics, prognosis and treatment options.
Paral J, Slaninka I, Kalabova H, Hadzi-Nikolov D
Acta Gastroenterol Belg 2010 Jul-Sep;73(3):349-59. PMID: 21086937
From RECIST to PERCIST: Evolving Considerations for PET response criteria in solid tumors.
Wahl RL, Jacene H, Kasamon Y, Lodge MA
J Nucl Med 2009 May;50 Suppl 1(Suppl 1):122S-50S. doi: 10.2967/jnumed.108.057307. PMID: 19403881Free PMC Article
A guide for the diagnosis and management of gastrointestinal stromal cell tumors.
Sepe PS, Brugge WR
Nat Rev Gastroenterol Hepatol 2009 Jun;6(6):363-71. Epub 2009 Apr 14 doi: 10.1038/nrgastro.2009.43. PMID: 19365407

Therapy

Duodenal gastrointestinal stromal tumors: review on clinical and surgical aspects.
Cavallaro G, Polistena A, D'Ermo G, Pedullà G, De Toma G
Int J Surg 2012;10(9):463-5. Epub 2012 Aug 30 doi: 10.1016/j.ijsu.2012.08.015. PMID: 22939976
Clinical effectiveness and cost-effectiveness of imatinib dose escalation for the treatment of unresectable and/or metastatic gastrointestinal stromal tumours that have progressed on treatment at a dose of 400 mg/day: a systematic review and economic evaluation.
Hislop J, Quayyum Z, Elders A, Fraser C, Jenkinson D, Mowatt G, Sharma P, Vale L, Petty R
Health Technol Assess 2011 Jun;15(25):1-178. doi: 10.3310/hta15250. PMID: 21689502Free PMC Article
Gastrointestinal stromal tumors: review on morphology, molecular pathology, diagnostics, prognosis and treatment options.
Paral J, Slaninka I, Kalabova H, Hadzi-Nikolov D
Acta Gastroenterol Belg 2010 Jul-Sep;73(3):349-59. PMID: 21086937
Prevalence and management of gastrointestinal stromal tumors.
Machado-Aranda D, Malamet M, Chang YJ, Jacobs MJ, Ferguson L, Silapaswan S, Goriel Y, Kolachalam R, Mittal VK
Am Surg 2009 Jan;75(1):55-60. PMID: 19213398

Prognosis

Mesenchymal Neoplasms of the Prostate and Seminal Vesicles: Spectrum of Disease with Radiologic-Pathologic Correlation.
Marcal LP, Surabhi VR, Ramani NS, Katabathina VS, Paspulati RM, Prasad SR
Radiographics 2022 Mar-Apr;42(2):417-432. Epub 2022 Jan 14 doi: 10.1148/rg.210084. PMID: 35030067
Expression of PIN1 in Gastrointestinal Stromal Tumours and its Clinical Significance.
Jiang L, Cao M, Hu J, Chen J
Anticancer Res 2016 Mar;36(3):1275-80. PMID: 26977025
Gastrointestinal stromal tumors: review on morphology, molecular pathology, diagnostics, prognosis and treatment options.
Paral J, Slaninka I, Kalabova H, Hadzi-Nikolov D
Acta Gastroenterol Belg 2010 Jul-Sep;73(3):349-59. PMID: 21086937
From RECIST to PERCIST: Evolving Considerations for PET response criteria in solid tumors.
Wahl RL, Jacene H, Kasamon Y, Lodge MA
J Nucl Med 2009 May;50 Suppl 1(Suppl 1):122S-50S. doi: 10.2967/jnumed.108.057307. PMID: 19403881Free PMC Article
Role of PTEN in gastrointestinal stromal tumor progression.
Ricci R, Maggiano N, Castri F, Rinelli A, Murazio M, Pacelli F, Potenza AE, Vecchio FM, Larocca LM
Arch Pathol Lab Med 2004 Apr;128(4):421-5. doi: 10.5858/2004-128-421-ROPIGS. PMID: 15043466

Clinical prediction guides

Ki-67 labelling index is related to the risk classification and prognosis of gastrointestinal stromal tumours: a retrospective study.
Wang JP, Liu L, Li ZA, Wang Q, Wang XY, Lin J
Gastroenterol Hepatol 2021 Feb;44(2):103-114. Epub 2020 Jul 25 doi: 10.1016/j.gastrohep.2020.05.022. PMID: 32718848
Expression of PIN1 in Gastrointestinal Stromal Tumours and its Clinical Significance.
Jiang L, Cao M, Hu J, Chen J
Anticancer Res 2016 Mar;36(3):1275-80. PMID: 26977025
Clinical effectiveness and cost-effectiveness of imatinib dose escalation for the treatment of unresectable and/or metastatic gastrointestinal stromal tumours that have progressed on treatment at a dose of 400 mg/day: a systematic review and economic evaluation.
Hislop J, Quayyum Z, Elders A, Fraser C, Jenkinson D, Mowatt G, Sharma P, Vale L, Petty R
Health Technol Assess 2011 Jun;15(25):1-178. doi: 10.3310/hta15250. PMID: 21689502Free PMC Article
A guide for the diagnosis and management of gastrointestinal stromal cell tumors.
Sepe PS, Brugge WR
Nat Rev Gastroenterol Hepatol 2009 Jun;6(6):363-71. Epub 2009 Apr 14 doi: 10.1038/nrgastro.2009.43. PMID: 19365407
High prognostic value of p16INK4 alterations in gastrointestinal stromal tumors.
Schneider-Stock R, Boltze C, Lasota J, Miettinen M, Peters B, Pross M, Roessner A, Günther T
J Clin Oncol 2003 May 1;21(9):1688-97. doi: 10.1200/JCO.2003.08.101. PMID: 12721243

Recent systematic reviews

Diagnostic value of contrast-enhanced harmonic endoscopic ultrasonography in predicting the malignancy potential of submucosal tumors: a systematic review and meta-analysis.
Yang YT, Shen N, Ao F, Chen WQ
Surg Endosc 2020 Sep;34(9):3754-3765. Epub 2020 Apr 29 doi: 10.1007/s00464-020-07585-6. PMID: 32350667
Clinical effectiveness and cost-effectiveness of imatinib dose escalation for the treatment of unresectable and/or metastatic gastrointestinal stromal tumours that have progressed on treatment at a dose of 400 mg/day: a systematic review and economic evaluation.
Hislop J, Quayyum Z, Elders A, Fraser C, Jenkinson D, Mowatt G, Sharma P, Vale L, Petty R
Health Technol Assess 2011 Jun;15(25):1-178. doi: 10.3310/hta15250. PMID: 21689502Free PMC Article

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