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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
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
Nasal cavity vascular leiomyoma: case report and literature review.
Campelo VE, Neves MC, Nakanishi M, Voegels RL
Braz J Otorhinolaryngol 2008 Jan-Feb;74(1):147-50. doi: 10.1016/s1808-8694(15)30766-7. PMID: 18392517Free 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

Benign-looking primary fibrosarcoma of the uterus.
Braun M, Kuncman W, Szumera-Ciećkiewicz A, Koseła-Paterczyk H, Musiał J, Jesionek-Kupnicka D, Ryś J, Kordek R
Pol J Pathol 2019;70(2):148-152. doi: 10.5114/pjp.2019.87108. PMID: 31556568
Imaging features of rare mesenychmal liver tumours: beyond haemangiomas.
Thampy R, Elsayes KM, Menias CO, Pickhardt PJ, Kang HC, Deshmukh SP, Ahmed K, Korivi BR
Br J Radiol 2017 Nov;90(1079):20170373. Epub 2017 Aug 2 doi: 10.1259/bjr.20170373. PMID: 28766950Free PMC Article
Nasal cavity vascular leiomyoma: case report and literature review.
Campelo VE, Neves MC, Nakanishi M, Voegels RL
Braz J Otorhinolaryngol 2008 Jan-Feb;74(1):147-50. doi: 10.1016/s1808-8694(15)30766-7. PMID: 18392517Free 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
Pseudo-pseudocysts of the pancreas.
Gillatt DA, Cooper MJ, Williamson RC
Pancreas 1986;1(5):460-3. doi: 10.1097/00006676-198609000-00012. PMID: 3550786

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
Giant Epithelioid Angiomyolipoma: An Imaging-related Differential Diagnosis Among Fat-containing Renal Masses.
Galatola R, Stanzione A, Sirignano C, Mainolfi C, Guadagno E, Carlomagno N, Insabato L, Santangelo M, Maurea S
Clin Genitourin Cancer 2020 Feb;18(1):e5-e9. Epub 2019 Nov 6 doi: 10.1016/j.clgc.2019.10.028. PMID: 31786119
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
Risk of occult malignancy in morcellated hysterectomy: a case series.
Hagemann IS, Hagemann AR, LiVolsi VA, Montone KT, Chu CS
Int J Gynecol Pathol 2011 Sep;30(5):476-83. doi: 10.1097/PGP.0b013e3182107ecf. PMID: 21804400
Leiomyomatosis peritonealis disseminata.
Buckshee K, Verma A, Karak AK
Int J Gynaecol Obstet 1998 May;61(2):191-2. doi: 10.1016/s0020-7292(98)00046-0. PMID: 9639228

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
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: 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

Combined Positive Score for Programmed Death Ligand-1 Expression and Inflammatory Microenvironment in Gastrointestinal Stromal Tumors.
Herlea V, Roșulescu A, Calotă VC, Croitoru V, Stoica Mustafa E, Vasilescu C, Alexandrescu S, Dumitrașcu T, Popescu I, Dima SO, Sajin M
Medicina (Kaunas) 2022 Jan 24;58(2) doi: 10.3390/medicina58020174. PMID: 35208498Free PMC Article
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
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
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

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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