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Malignant Epithelioid Cell Type Gastrointestinal Stromal Tumor

MedGen UID:
415168
•Concept ID:
C2825773
•
Neoplastic Process

Definition

An epithelioid cell type gastrointestinal stromal tumor with an aggressive clinical course. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVMalignant Epithelioid Cell Type Gastrointestinal Stromal Tumor

Professional guidelines

PubMed

Epithelioid Leiomyosarcoma of the Uterus: Modern Outcome-based Appraisal of Diagnostic Criteria in a Large Institutional Series.
Chapel DB, Nucci MR, Quade BJ, Parra-Herran C
Am J Surg Pathol 2022 Apr 1;46(4):464-475. doi: 10.1097/PAS.0000000000001795. PMID: 34419987
Gastrointestinal stromal tumors: review on morphology, molecular pathology, prognosis, and differential diagnosis.
Miettinen M, Lasota J
Arch Pathol Lab Med 2006 Oct;130(10):1466-78. doi: 10.5858/2006-130-1466-GSTROM. PMID: 17090188
Gastrointestinal stromal tumors--definition, clinical, histological, immunohistochemical, and molecular genetic features and differential diagnosis.
Miettinen M, Lasota J
Virchows Arch 2001 Jan;438(1):1-12. doi: 10.1007/s004280000338. PMID: 11213830

Recent clinical studies

Etiology

Molecular and clinicopathological features of KIT/PDGFRA wild-type gastrointestinal stromal tumors.
Nishida T, Naito Y, Takahashi T, Saito T, Hisamori S, Manaka D, Ogawa K, Hirota S, Ichikawa H
Cancer Sci 2024 Mar;115(3):894-904. Epub 2024 Jan 5 doi: 10.1111/cas.16058. PMID: 38178783Free PMC Article
Inflammatory Myofibroblastic Tumor in Female Genital Tract.
Shukla PS, Mittal K
Arch Pathol Lab Med 2019 Jan;143(1):122-129. Epub 2018 Jul 2 doi: 10.5858/arpa.2017-0575-RA. PMID: 29965784
Gastrointestinal stromal tumor: clinicopathological characteristics and pathologic prognostic analysis.
Jumniensuk C, Charoenpitakchai M
World J Surg Oncol 2018 Dec 3;16(1):231. doi: 10.1186/s12957-018-1532-1. PMID: 30509310Free PMC Article
Gastrointestinal stromal tumours.
Miettinen M, Sarlomo-Rikala M, Lasota J
Ann Chir Gynaecol 1998;87(4):278-81. PMID: 9891765
Oral leiomyomas.
Damm DD, Neville BW
Oral Surg Oral Med Oral Pathol 1979 Apr;47(4):343-8. doi: 10.1016/0030-4220(79)90257-3. PMID: 285402

Diagnosis

Malignant perivascular epithelioid cell tumor (PEComa) of the uterus.
Cao B, Huang Y
BMC Womens Health 2022 Dec 16;22(1):523. doi: 10.1186/s12905-022-02119-9. PMID: 36522714Free PMC Article
Inflammatory Myofibroblastic Tumor in Female Genital Tract.
Shukla PS, Mittal K
Arch Pathol Lab Med 2019 Jan;143(1):122-129. Epub 2018 Jul 2 doi: 10.5858/arpa.2017-0575-RA. PMID: 29965784
Gastrointestinal stromal tumor: clinicopathological characteristics and pathologic prognostic analysis.
Jumniensuk C, Charoenpitakchai M
World J Surg Oncol 2018 Dec 3;16(1):231. doi: 10.1186/s12957-018-1532-1. PMID: 30509310Free PMC Article
Gastrointestinal stromal tumors: review on morphology, molecular pathology, prognosis, and differential diagnosis.
Miettinen M, Lasota J
Arch Pathol Lab Med 2006 Oct;130(10):1466-78. doi: 10.5858/2006-130-1466-GSTROM. PMID: 17090188
Gastrointestinal stromal tumor (GIST).
Joensuu H
Ann Oncol 2006 Sep;17 Suppl 10:x280-6. doi: 10.1093/annonc/mdl274. PMID: 17018739

Therapy

Recurrent vaginal epithelioid leiomyosarcoma; a case report from Botswana and review of the literature.
Benti TM, Mercy N, Eshetu AB, Iwuh IE, Kassa MW
Afr J Reprod Health 2021 Feb;25(1):161-168. doi: 10.29063/ajrh2021/v25i1.17. PMID: 34077121
Gastrointestinal stromal tumors: what do we know now?
Corless CL
Mod Pathol 2014 Jan;27 Suppl 1:S1-16. doi: 10.1038/modpathol.2013.173. PMID: 24384849
Gastrointestinal stromal tumors in children and young adults: a clinicopathologic, molecular, and genomic study of 15 cases and review of the literature.
Prakash S, Sarran L, Socci N, DeMatteo RP, Eisenstat J, Greco AM, Maki RG, Wexler LH, LaQuaglia MP, Besmer P, Antonescu CR
J Pediatr Hematol Oncol 2005 Apr;27(4):179-87. doi: 10.1097/01.mph.0000157790.81329.47. PMID: 15838387
Pleomorphic liposarcoma of the uterus: case report and literature review.
Levine PH, Wei XJ, Gagner JP, Flax H, Mittal K, Blank SV
Int J Gynecol Pathol 2003 Oct;22(4):407-11. doi: 10.1097/01.pgp.0000092156.33490.89. PMID: 14501826
Pathologic considerations of uterine smooth muscle tumors.
Prayson RA, Hart WR
Obstet Gynecol Clin North Am 1995 Dec;22(4):637-57. PMID: 8786875

Prognosis

Molecular and clinicopathological features of KIT/PDGFRA wild-type gastrointestinal stromal tumors.
Nishida T, Naito Y, Takahashi T, Saito T, Hisamori S, Manaka D, Ogawa K, Hirota S, Ichikawa H
Cancer Sci 2024 Mar;115(3):894-904. Epub 2024 Jan 5 doi: 10.1111/cas.16058. PMID: 38178783Free PMC Article
Smooth Muscle Tumors of the Female Genital Tract.
Devereaux KA, Schoolmeester JK
Surg Pathol Clin 2019 Jun;12(2):397-455. doi: 10.1016/j.path.2019.02.004. PMID: 31097110
Gastrointestinal stromal tumor: clinicopathological characteristics and pathologic prognostic analysis.
Jumniensuk C, Charoenpitakchai M
World J Surg Oncol 2018 Dec 3;16(1):231. doi: 10.1186/s12957-018-1532-1. PMID: 30509310Free PMC Article
Gastrointestinal stromal tumors: review on morphology, molecular pathology, prognosis, and differential diagnosis.
Miettinen M, Lasota J
Arch Pathol Lab Med 2006 Oct;130(10):1466-78. doi: 10.5858/2006-130-1466-GSTROM. PMID: 17090188
Oral leiomyomas.
Damm DD, Neville BW
Oral Surg Oral Med Oral Pathol 1979 Apr;47(4):343-8. doi: 10.1016/0030-4220(79)90257-3. PMID: 285402

Clinical prediction guides

Incidental superficial soft tissue epithelioid angioleiomyoma.
Val-Bernal JF, Fontanil N, García-Montesinos B, Martino M
J Cutan Pathol 2022 Aug;49(8):731-735. Epub 2022 Apr 19 doi: 10.1111/cup.14233. PMID: 35357042
Uterine angiomyolipoma: a clinical analysis of 8 cases and literature review.
Wang J, Yang Q, Zhang N, Wang D
Arch Gynecol Obstet 2021 Jul;304(1):171-177. Epub 2021 Feb 10 doi: 10.1007/s00404-021-05992-1. PMID: 33569612
Gastrointestinal stromal tumors: what do we know now?
Corless CL
Mod Pathol 2014 Jan;27 Suppl 1:S1-16. doi: 10.1038/modpathol.2013.173. PMID: 24384849
Gastrointestinal stromal tumors: review on morphology, molecular pathology, prognosis, and differential diagnosis.
Miettinen M, Lasota J
Arch Pathol Lab Med 2006 Oct;130(10):1466-78. doi: 10.5858/2006-130-1466-GSTROM. PMID: 17090188
Gastrointestinal stromal tumors. A review of characteristics morphologic, immunohistochemical, and molecular genetic features.
Goldblum JR
Am J Clin Pathol 2002 Jun;117 Suppl:S49-61. doi: 10.1309/7EWE-PF04-VWJR-G4WQ. PMID: 14569802

Recent systematic reviews

The Uterine Plexiform Lesions Revisited.
Karpathiou G, Papoudou-Bai A, Clemenson A, Chauleur C, Peoc'h M
Am J Clin Pathol 2020 Jul 7;154(2):178-189. doi: 10.1093/ajcp/aqaa054. PMID: 32459343
Uterine lesions with sex cord-like architectures: a systematic review.
Jia M, Sun PL, Gao H
Diagn Pathol 2019 Nov 18;14(1):129. doi: 10.1186/s13000-019-0909-y. PMID: 31739799Free PMC Article

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