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Gastroblastoma

MedGen UID:: 1731587
•Concept ID:: C5419768
•: Neoplastic Process

Definition

A very rare biphasic tumor arising in the gastric muscularis propria (usually of the antrum), generally in boys and young men. It is associated with MALAT1-GLI1 gene fusion. (WHO 2019) [from NCI]

Term Hierarchy

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVGastroblastoma

Pathological process
- Disease
  - Neoplasm
    - Malignant neoplastic disease
      - Malignant gastrointestinal tract tumors
        Gastric cancer
        Gastroblastoma

Recent clinical studies

Etiology

Gastroblastoma without GLI1 and EWSR1 gene breaks.

Gong C, Xu J, Qiao S, Zhang X, Yi M
World J Surg Oncol 2023 Sep 1;21(1):274. doi: 10.1186/s12957-023-03159-7. PMID: 37658451 Free PMC Article

Primary Cutaneous Epithelioid Mesenchymal Tumor With a Novel ATP2B4::GLI1 Gene Fusion.

Rezaee N, Cohen B, Eno C, Shon W
Am J Dermatopathol 2023 Sep 1;45(9):642-645. Epub 2023 Jul 28 doi: 10.1097/DAD.0000000000002497. PMID: 37506273

Expanding the spectrum of GLI1-altered mesenchymal tumors-A high-grade uterine sarcoma harboring a novel PAMR1::GLI1 fusion and literature review of GLI1-altered mesenchymal neoplasms of the gynecologic tract.

Punjabi LS, Goh CHR, Sittampalam K
Genes Chromosomes Cancer 2023 Feb;62(2):107-114. Epub 2022 Oct 26 doi: 10.1002/gcc.23099. PMID: 36222065

A Distinct Malignant Epithelioid Neoplasm With GLI1 Gene Rearrangements, Frequent S100 Protein Expression, and Metastatic Potential: Expanding the Spectrum of Pathologic Entities With ACTB/MALAT1/PTCH1-GLI1 Fusions.

Antonescu CR, Agaram NP, Sung YS, Zhang L, Swanson D, Dickson BC
Am J Surg Pathol 2018 Apr;42(4):553-560. doi: 10.1097/PAS.0000000000001010. PMID: 29309307 Free PMC Article

Gastroblastoma harbors a recurrent somatic MALAT1-GLI1 fusion gene.

Graham RP, Nair AA, Davila JI, Jin L, Jen J, Sukov WR, Wu TT, Appelman HD, Torres-Mora J, Perry KD, Zhang L, Kloft-Nelson SM, Knudson RA, Greipp PT, Folpe AL
Mod Pathol 2017 Oct;30(10):1443-1452. Epub 2017 Jul 21 doi: 10.1038/modpathol.2017.68. PMID: 28731043

See all (5)

Diagnosis

Gastroblastoma without GLI1 and EWSR1 gene breaks.

Gong C, Xu J, Qiao S, Zhang X, Yi M
World J Surg Oncol 2023 Sep 1;21(1):274. doi: 10.1186/s12957-023-03159-7. PMID: 37658451 Free PMC Article

Gastroblastoma of the Pylorus: A Case Report and Review of the Literature.

McCammon N, Dunn A, Graham R, McHugh J, Lamps L, Bresler SC, Cole T, Rottmann D
Int J Surg Pathol 2023 Dec;31(8):1516-1521. Epub 2023 Feb 23 doi: 10.1177/10668969231157310. PMID: 36823781

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

Gene fusions in gastrointestinal tract cancers.

Rahi H, Olave MC, Fritchie KJ, Greipp PT, Halling KC, Kipp BR, Graham RP
Genes Chromosomes Cancer 2022 May;61(5):285-297. doi: 10.1002/gcc.23035. PMID: 35239225

Antonescu CR, Agaram NP, Sung YS, Zhang L, Swanson D, Dickson BC
Am J Surg Pathol 2018 Apr;42(4):553-560. doi: 10.1097/PAS.0000000000001010. PMID: 29309307 Free PMC Article

See all (13)

Therapy

Gastroblastoma in a 28-year-old man with nodal metastasis: proof of the malignant potential.

Wey EA, Britton AJ, Sferra JJ, Kasunic T, Pepe LR, Appelman HD
Arch Pathol Lab Med 2012 Aug;136(8):961-4. doi: 10.5858/arpa.2011-0372-CR. PMID: 22849746

See all (1)

Prognosis

Gastroblastoma without GLI1 and EWSR1 gene breaks.

Gong C, Xu J, Qiao S, Zhang X, Yi M
World J Surg Oncol 2023 Sep 1;21(1):274. doi: 10.1186/s12957-023-03159-7. PMID: 37658451 Free PMC Article

Gastroblastoma of the Pylorus: A Case Report and Review of the Literature.

McCammon N, Dunn A, Graham R, McHugh J, Lamps L, Bresler SC, Cole T, Rottmann D
Int J Surg Pathol 2023 Dec;31(8):1516-1521. Epub 2023 Feb 23 doi: 10.1177/10668969231157310. PMID: 36823781

Gene fusions in gastrointestinal tract cancers.

Rahi H, Olave MC, Fritchie KJ, Greipp PT, Halling KC, Kipp BR, Graham RP
Genes Chromosomes Cancer 2022 May;61(5):285-297. doi: 10.1002/gcc.23035. PMID: 35239225

Recent developments in gastroesophageal mesenchymal tumours.

Papke DJ Jr, Hornick JL
Histopathology 2021 Jan;78(1):171-186. doi: 10.1111/his.14164. PMID: 33382494

Antonescu CR, Agaram NP, Sung YS, Zhang L, Swanson D, Dickson BC
Am J Surg Pathol 2018 Apr;42(4):553-560. doi: 10.1097/PAS.0000000000001010. PMID: 29309307 Free PMC Article

See all (9)

Clinical prediction guides

Gastroblastoma without GLI1 and EWSR1 gene breaks.

Gong C, Xu J, Qiao S, Zhang X, Yi M
World J Surg Oncol 2023 Sep 1;21(1):274. doi: 10.1186/s12957-023-03159-7. PMID: 37658451 Free PMC Article

Gastroblastoma mimics the embryonic mesenchyme of the foregut: a case report.

Sugimoto R, Uesugi N, Yamada N, Osakabe M, Baba S, Yanagawa N, Akiyama Y, Habano W, Sasaki A, Oda Y, Sugai T
Diagn Pathol 2023 Feb 17;18(1):24. doi: 10.1186/s13000-023-01310-2. PMID: 36803776 Free PMC Article

PDGFRA Immunohistochemistry Predicts PDGFRA Mutations in Gastrointestinal Stromal Tumors.

Papke DJ Jr, Forgó E, Charville GW, Hornick JL
Am J Surg Pathol 2022 Jan 1;46(1):3-10. doi: 10.1097/PAS.0000000000001720. PMID: 33859072

Gastroblastoma harbors a recurrent somatic MALAT1-GLI1 fusion gene.

See all (4)