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Dedifferentiated Gastrointestinal Stromal Tumor

MedGen UID:
1792396
•Concept ID:
C5555955
•
Neoplastic Process

Definition

A gastrointestinal stromal tumor that through the process of dedifferentiation, results in an uncommon phenotype. Dedifferentiation in the context of GIST, may manifest as loss of CD117 expression and regression from specialized tissue to a more anaplastic/pleomorphic appearance, high nuclear atypia, high mitotic activity, and necrosis. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVDedifferentiated Gastrointestinal Stromal Tumor

Professional guidelines

PubMed

Undifferentiated and dedifferentiated soft tissue neoplasms: Immunohistochemical surrogates for differential diagnosis.
Thway K, Fisher C
Semin Diagn Pathol 2021 Nov;38(6):170-186. Epub 2021 Sep 29 doi: 10.1053/j.semdp.2021.09.005. PMID: 34602314
The many faces of solitary fibrous tumor; diversity of histological features, differential diagnosis and role of molecular studies and surrogate markers in avoiding misdiagnosis and predicting the behavior.
Tariq MU, Din NU, Abdul-Ghafar J, Park YK
Diagn Pathol 2021 Apr 20;16(1):32. doi: 10.1186/s13000-021-01095-2. PMID: 33879215Free PMC Article
Sunitinib in the Treatment of Thyroid Cancer.
Ferrari SM, Centanni M, Virili C, Miccoli M, Ferrari P, Ruffilli I, Ragusa F, Antonelli A, Fallahi P
Curr Med Chem 2019;26(6):963-972. doi: 10.2174/0929867324666171006165942. PMID: 28990511

Recent clinical studies

Etiology

Natural history and treatment efficacy in an ambispective case series of NTRK-rearranged mesenchymal tumors.
Dufresne A, Pissaloux D, Ngo C, Penel N, Le Cesne A, Macagno N, Vanacker H, Hénon C, Jean-Denis M, Rughoo K, Tirode F, Blay JY, Brahmi M
ESMO Open 2023 Apr;8(2):101202. Epub 2023 Apr 11 doi: 10.1016/j.esmoop.2023.101202. PMID: 37054503Free PMC Article
Predictive and prognostic impact of the different features of tumor budding in stage II colorectal cancer.
Wang YT, Wang H, Li HW, Han B, Yang ZP
Neoplasma 2022 Sep;69(5):1237-1245. Epub 2022 Aug 24 doi: 10.4149/neo_2022_220525N557. PMID: 36004649
Undifferentiated and dedifferentiated soft tissue neoplasms: Immunohistochemical surrogates for differential diagnosis.
Thway K, Fisher C
Semin Diagn Pathol 2021 Nov;38(6):170-186. Epub 2021 Sep 29 doi: 10.1053/j.semdp.2021.09.005. PMID: 34602314
Soft-Tissue Sarcomas of the Abdomen and Pelvis: Radiologic-Pathologic Features, Part 1-Common Sarcomas: From the Radiologic Pathology Archives.
Levy AD, Manning MA, Al-Refaie WB, Miettinen MM
Radiographics 2017 Mar-Apr;37(2):462-483. doi: 10.1148/rg.2017160157. PMID: 28287938Free PMC Article
Cytohistological and immunohistochemical characteristics of spindle-shaped mesenchymal neoplasms occurring in the gastrointestinal tract.
Ieni A, Barresi V, Reggiani Bonetti L, Branca G, Caruso RA, Tuccari G
Scand J Gastroenterol 2017 Mar;52(3):291-299. Epub 2016 Nov 6 doi: 10.1080/00365521.2016.1251607. PMID: 27817254

Diagnosis

Assessment of The Utility of The Sarcoma DNA Methylation Classifier In Surgical Pathology.
Miettinen M, Abdullaev Z, Turakulov R, Quezado M, Luiña Contreras A, Curcio CA, Rys J, Chlopek M, Lasota J, Aldape KD
Am J Surg Pathol 2024 Jan 1;48(1):112-122. Epub 2023 Nov 3 doi: 10.1097/PAS.0000000000002138. PMID: 37921028Free PMC Article
Sarcoma care in the era of precision medicine.
Wallander K, Öfverholm I, Boye K, Tsagkozis P, Papakonstantinou A, Lin Y, Haglund de Flon F
J Intern Med 2023 Dec;294(6):690-707. Epub 2023 Sep 7 doi: 10.1111/joim.13717. PMID: 37643281
GLI1 Immunohistochemistry Distinguishes Mesenchymal Neoplasms With GLI1 Alterations From Morphologic Mimics.
Parrack PH, Mariño-Enríquez A, Fletcher CDM, Hornick JL, Papke DJ Jr
Am J Surg Pathol 2023 Apr 1;47(4):453-460. Epub 2023 Jan 23 doi: 10.1097/PAS.0000000000002018. PMID: 36693363
The many faces of solitary fibrous tumor; diversity of histological features, differential diagnosis and role of molecular studies and surrogate markers in avoiding misdiagnosis and predicting the behavior.
Tariq MU, Din NU, Abdul-Ghafar J, Park YK
Diagn Pathol 2021 Apr 20;16(1):32. doi: 10.1186/s13000-021-01095-2. PMID: 33879215Free PMC Article
Dedifferentiated gastrointestinal stromal tumor: Recent advances.
Karakas C, Christensen P, Baek D, Jung M, Ro JY
Ann Diagn Pathol 2019 Apr;39:118-124. Epub 2018 Dec 18 doi: 10.1016/j.anndiagpath.2018.12.005. PMID: 30661742

Therapy

Rare case of a duodenal de novo dedifferentiated gastrointestinal stromal tumour.
Shah PA, Babu R, Uthaiah SB, Patil S
BMJ Case Rep 2021 May 7;14(5) doi: 10.1136/bcr-2020-241442. PMID: 33962926Free PMC Article
Dedifferentiated gastrointestinal stromal tumor: Recent advances.
Karakas C, Christensen P, Baek D, Jung M, Ro JY
Ann Diagn Pathol 2019 Apr;39:118-124. Epub 2018 Dec 18 doi: 10.1016/j.anndiagpath.2018.12.005. PMID: 30661742
Sunitinib in the Treatment of Thyroid Cancer.
Ferrari SM, Centanni M, Virili C, Miccoli M, Ferrari P, Ruffilli I, Ragusa F, Antonelli A, Fallahi P
Curr Med Chem 2019;26(6):963-972. doi: 10.2174/0929867324666171006165942. PMID: 28990511
PAX8 Expression in Solitary Fibrous Tumor: A Potential Diagnostic Pitfall.
Ullman D, Gordetsky J, Siegal GP, Prieto-Granada CN, Wei S, Stevens TM
Appl Immunohistochem Mol Morphol 2019 Mar;27(3):195-202. doi: 10.1097/PAI.0000000000000561. PMID: 28749793
Gastrointestinal stromal tumors: morphological, immunohistochemical and molecular changes associated with kinase inhibitor therapy.
Díaz Delgado M, Hernández Amate A, Pereira Gallardo S, Jaramillo S, Virizuela Echaburu JA, González-Cámpora RJ
Pathol Oncol Res 2011 Sep;17(3):455-61. Epub 2011 Jan 29 doi: 10.1007/s12253-011-9362-2. PMID: 21279555

Prognosis

Sarcoma care in the era of precision medicine.
Wallander K, Öfverholm I, Boye K, Tsagkozis P, Papakonstantinou A, Lin Y, Haglund de Flon F
J Intern Med 2023 Dec;294(6):690-707. Epub 2023 Sep 7 doi: 10.1111/joim.13717. PMID: 37643281
Undifferentiated and dedifferentiated soft tissue neoplasms: Immunohistochemical surrogates for differential diagnosis.
Thway K, Fisher C
Semin Diagn Pathol 2021 Nov;38(6):170-186. Epub 2021 Sep 29 doi: 10.1053/j.semdp.2021.09.005. PMID: 34602314
The many faces of solitary fibrous tumor; diversity of histological features, differential diagnosis and role of molecular studies and surrogate markers in avoiding misdiagnosis and predicting the behavior.
Tariq MU, Din NU, Abdul-Ghafar J, Park YK
Diagn Pathol 2021 Apr 20;16(1):32. doi: 10.1186/s13000-021-01095-2. PMID: 33879215Free PMC Article
Molecular analyses in the diagnosis and prediction of prognosis in non-GIST soft tissue sarcomas: A systematic review and meta-analysis.
Kandel RA, Yao X, Dickson BC, Ghert M, Popovic S, Purgina BM, Verma S, Werier J
Cancer Treat Rev 2018 May;66:74-81. Epub 2018 Apr 22 doi: 10.1016/j.ctrv.2018.04.005. PMID: 29709714
Molecular Pathogenesis and Diagnostic, Prognostic and Predictive Molecular Markers in Sarcoma.
Mariño-Enríquez A, Bovée JV
Surg Pathol Clin 2016 Sep;9(3):457-73. doi: 10.1016/j.path.2016.04.009. PMID: 27523972Free PMC Article

Clinical prediction guides

Assessment of The Utility of The Sarcoma DNA Methylation Classifier In Surgical Pathology.
Miettinen M, Abdullaev Z, Turakulov R, Quezado M, Luiña Contreras A, Curcio CA, Rys J, Chlopek M, Lasota J, Aldape KD
Am J Surg Pathol 2024 Jan 1;48(1):112-122. Epub 2023 Nov 3 doi: 10.1097/PAS.0000000000002138. PMID: 37921028Free PMC Article
Sarcoma care in the era of precision medicine.
Wallander K, Öfverholm I, Boye K, Tsagkozis P, Papakonstantinou A, Lin Y, Haglund de Flon F
J Intern Med 2023 Dec;294(6):690-707. Epub 2023 Sep 7 doi: 10.1111/joim.13717. PMID: 37643281
The many faces of solitary fibrous tumor; diversity of histological features, differential diagnosis and role of molecular studies and surrogate markers in avoiding misdiagnosis and predicting the behavior.
Tariq MU, Din NU, Abdul-Ghafar J, Park YK
Diagn Pathol 2021 Apr 20;16(1):32. doi: 10.1186/s13000-021-01095-2. PMID: 33879215Free PMC Article
Molecular Pathogenesis and Diagnostic, Prognostic and Predictive Molecular Markers in Sarcoma.
Mariño-Enríquez A, Bovée JV
Surg Pathol Clin 2016 Sep;9(3):457-73. doi: 10.1016/j.path.2016.04.009. PMID: 27523972Free PMC Article
Gastrointestinal stromal tumors: morphological, immunohistochemical and molecular changes associated with kinase inhibitor therapy.
Díaz Delgado M, Hernández Amate A, Pereira Gallardo S, Jaramillo S, Virizuela Echaburu JA, González-Cámpora RJ
Pathol Oncol Res 2011 Sep;17(3):455-61. Epub 2011 Jan 29 doi: 10.1007/s12253-011-9362-2. PMID: 21279555

Recent systematic reviews

An evidence-based guideline on the application of molecular testing in the diagnosis, prediction of prognosis, and selection of therapy in non-GIST soft tissue sarcomas.
Yao X, Ghert M, Dickson BC, Popovic S, Purgina BM, Verma S, Werier J, Kandel RA
Cancer Treat Rev 2020 Apr;85:101987. Epub 2020 Feb 14 doi: 10.1016/j.ctrv.2020.101987. PMID: 32092619
Primary hepatic gastrointestinal stromal tumor with right adrenal gland invasion: A case report and systematic literature review.
Xu L, Zhang M, Xu M
Medicine (Baltimore) 2019 May;98(20):e15482. doi: 10.1097/MD.0000000000015482. PMID: 31096446Free PMC Article
Molecular analyses in the diagnosis and prediction of prognosis in non-GIST soft tissue sarcomas: A systematic review and meta-analysis.
Kandel RA, Yao X, Dickson BC, Ghert M, Popovic S, Purgina BM, Verma S, Werier J
Cancer Treat Rev 2018 May;66:74-81. Epub 2018 Apr 22 doi: 10.1016/j.ctrv.2018.04.005. PMID: 29709714

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