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Gastrointestinal Clear Cell Sarcoma/Malignant Gastrointestinal Neuroectodermal Tumor

MedGen UID:
1757920
•Concept ID:
C5417751
•
Neoplastic Process

Definition

A gastrointestinal tract sarcoma with neuroectodermal differentiation. It is characterized by the presence of neoplastic round cells with pale eosinophilic cytoplasm. Cytoplasmic clearing is seen only in a minority of cases. Half of the cases contain osteoclastic giant cells. It is associated with gene fusion translocations involving the EWSR1 gene, usually EWSR1-ATF1 fusion or EWSR1-CREB1 fusion. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVGastrointestinal Clear Cell Sarcoma/Malignant Gastrointestinal Neuroectodermal Tumor

Professional guidelines

PubMed

Malignant gastrointestinal neuroectodermal tumor in head and neck: two challenging cases with diverse morphology and different considerations for differential diagnosis.
Kuo CT, Kao YC, Huang HY, Hsiao CH, Lee JC
Virchows Arch 2022 Jul;481(1):131-136. Epub 2022 Jan 18 doi: 10.1007/s00428-022-03274-y. PMID: 35039897
Malignant gastrointestinal neuroectodermal tumor showing overlapped immunophenotype with synovial sarcoma: CD99 and SOX10 antibodies are useful in differential diagnosis.
Suárez-Vilela D, Izquierdo FM, Tojo-Ramallo S, R Riera-Velasco J, Escobar-Stein J
Am J Surg Pathol 2012 Dec;36(12):1905-8; author reply 1908. doi: 10.1097/PAS.0b013e31826f5b28. PMID: 23154774

Recent clinical studies

Etiology

A Case Series of Metastatic Malignant Gastrointestinal Neuroectodermal Tumors and Comprehensive Genomic Profiling Analysis of 20 Cases.
Kandler T, Cortez E, Clinton L, Hemmerich A, Ahmed O, Wong R, Forns T, MacNeill AJ, Hamilton TD, Khorasani M, Feng X
Curr Oncol 2022 Feb 21;29(2):1279-1297. doi: 10.3390/curroncol29020109. PMID: 35200608Free PMC Article
Clear cell sarcoma of the gastrointestinal tract and malignant gastrointestinal neuroectodermal tumour: distinct or related entities? A review.
Green C, Spagnolo DV, Robbins PD, Fermoyle S, Wong DD
Pathology 2018 Aug;50(5):490-498. Epub 2018 Jun 30 doi: 10.1016/j.pathol.2018.05.001. PMID: 29970252
Clear Cell Sarcoma-like Tumor of the Gastrointestinal Tract: Clinical Outcome and Pathologic Features of a Molecularly Characterized Tertiary Center Case Series.
Libertini M, Thway K, Noujaim J, Puls F, Messiou C, Fisher C, Jones RL
Anticancer Res 2018 Mar;38(3):1479-1483. doi: 10.21873/anticanres.12374. PMID: 29491075
Clear cell sarcoma of the gastrointestinal tract after very low-dose therapeutic radiation therapy: a case report.
Yang JC, Chou AJ, Oeffinger KC, La Quaglia MP, Wolden SL
J Pediatr Surg 2012 Oct;47(10):1943-5. doi: 10.1016/j.jpedsurg.2012.08.014. PMID: 23084213
Discovered on gastrointestinal stromal tumours 1 (DOG1) expression in non-gastrointestinal stromal tumour (GIST) neoplasms.
Hemminger J, Iwenofu OH
Histopathology 2012 Aug;61(2):170-7. Epub 2012 May 9 doi: 10.1111/j.1365-2559.2011.04150.x. PMID: 22571292

Diagnosis

Extraenteric Malignant Gastrointestinal Neuroectodermal Tumor-A Clinicopathologic and Molecular Genetic Study of 11 Cases.
Ulici V, Hornick JL, Davis JL, Mehrotra S, Meis JM, Halling KC, Fletcher CDM, Kao E, Folpe AL
Mod Pathol 2023 Jul;36(7):100160. Epub 2023 Mar 17 doi: 10.1016/j.modpat.2023.100160. PMID: 36934861
Malignant gastrointestinal neuroectodermal tumor: A case-based review of literature.
Mishra P, Biswas D, Pattnaik SA, Patra S, Muduly DK, Balasubiramaniyan V, Adhya AK
J Cancer Res Ther 2022 Jul-Sep;18(4):885-897. doi: 10.4103/jcrt.JCRT_829_19. PMID: 36149136
Malignant Gastrointestinal Neuroectodermal Tumor: A New Kid on the Block?
Morani AC, Ramani NS, Yedururi S, Prasad SR
J Comput Assist Tomogr 2022 Sep-Oct 01;46(5):676-681. Epub 2022 Jun 21 doi: 10.1097/RCT.0000000000001350. PMID: 35759779
Malignant gastrointestinal neuroectodermal tumor-A case report.
Harshavardhini S, Saishalini CN, Pavithra V, Shah NM, Sankar S
Indian J Pathol Microbiol 2021 Apr-Jun;64(2):373-375. doi: 10.4103/IJPM.IJPM_269_20. PMID: 33851638
Clear cell sarcoma-like/malignant gastrointestinal neuroectodermal tumor of the tongue: a clinicopathologic and molecular case report.
Sbaraglia M, Zanatta L, Toffolatti L, Spallanzani A, Bertolini F, Mattioli F, Lami F, Presutti L, Dei Tos AP
Virchows Arch 2021 Jun;478(6):1203-1207. Epub 2020 Oct 2 doi: 10.1007/s00428-020-02933-2. PMID: 33005982

Therapy

Malignant gastrointestinal neuroectodermal tumor (clear cell sarcoma-like tumor of the gastrointestinal tract) of the small intestine in a 12-year-old boy.
Wolak P, Wincewicz A, Czauderna P, Spałek M, Kruczak A, Urbaniak-Wąsik S, Ryś J, Michalak E, Woltanowska M, Sulkowski S
Dev Period Med 2018;22(4):358-363. PMID: 30636233
An unusual association of malignant gastrointestinal neuroectodermal tumor (clear cell sarcoma-like) and Ewing sarcoma.
Insabato L, Guadagno E, Natella V, Somma A, Bihl M, Pizzolorusso A, Mainenti PP, Apice G, Tornillo L
Pathol Res Pract 2015 Sep;211(9):688-92. Epub 2015 Jun 17 doi: 10.1016/j.prp.2015.06.001. PMID: 26163185
Management of primary retroperitoneal sarcoma (RPS) in the adult: a consensus approach from the Trans-Atlantic RPS Working Group.
Trans-Atlantic RPS Working Group
Ann Surg Oncol 2015 Jan;22(1):256-63. Epub 2014 Oct 15 doi: 10.1245/s10434-014-3965-2. PMID: 25316486
Intraabdominal follicular dendritic cell sarcoma: a report of three cases and review of the literature.
Urun Y, Kankaya D, Koral L, Yalcin B, Karabork A, Ceyhan K, Boruban MC, Utkan G, Demirkazik A
Tumori 2013 Mar-Apr;99(2):e65-9. doi: 10.1177/030089161309900231. PMID: 23748832
Clinical evaluation of neutron beam therapy. Current results and prospects, 1983.
Cohen L, Hendrickson FR, Kurup PD, Mansell JA, Awschalom M, Rosenberg I, Ten Haken RK
Cancer 1985 Jan 1;55(1):10-7. doi: 10.1002/1097-0142(19850101)55:1<10::aid-cncr2820550103>3.0.co;2-1. PMID: 3917351

Prognosis

Extraenteric Malignant Gastrointestinal Neuroectodermal Tumor-A Clinicopathologic and Molecular Genetic Study of 11 Cases.
Ulici V, Hornick JL, Davis JL, Mehrotra S, Meis JM, Halling KC, Fletcher CDM, Kao E, Folpe AL
Mod Pathol 2023 Jul;36(7):100160. Epub 2023 Mar 17 doi: 10.1016/j.modpat.2023.100160. PMID: 36934861
Malignant Gastrointestinal Neuroectodermal Tumor: A New Kid on the Block?
Morani AC, Ramani NS, Yedururi S, Prasad SR
J Comput Assist Tomogr 2022 Sep-Oct 01;46(5):676-681. Epub 2022 Jun 21 doi: 10.1097/RCT.0000000000001350. PMID: 35759779
Malignant gastrointestinal neuroectodermal tumor-A case report.
Harshavardhini S, Saishalini CN, Pavithra V, Shah NM, Sankar S
Indian J Pathol Microbiol 2021 Apr-Jun;64(2):373-375. doi: 10.4103/IJPM.IJPM_269_20. PMID: 33851638
Clear cell sarcoma of the gastrointestinal tract and malignant gastrointestinal neuroectodermal tumour: distinct or related entities? A review.
Green C, Spagnolo DV, Robbins PD, Fermoyle S, Wong DD
Pathology 2018 Aug;50(5):490-498. Epub 2018 Jun 30 doi: 10.1016/j.pathol.2018.05.001. PMID: 29970252
Malignant gastrointestinal neuroectodermal tumor: a case report and review of the literature.
Alyousef MJ, Alratroot JA, ElSharkawy T, Shawarby MA, Al Hamad MA, Hashem TM, Alsayyah A
Diagn Pathol 2017 Mar 20;12(1):29. doi: 10.1186/s13000-017-0620-9. PMID: 28320420Free PMC Article

Clinical prediction guides

Extraenteric Malignant Gastrointestinal Neuroectodermal Tumor-A Clinicopathologic and Molecular Genetic Study of 11 Cases.
Ulici V, Hornick JL, Davis JL, Mehrotra S, Meis JM, Halling KC, Fletcher CDM, Kao E, Folpe AL
Mod Pathol 2023 Jul;36(7):100160. Epub 2023 Mar 17 doi: 10.1016/j.modpat.2023.100160. PMID: 36934861
Malignant gastrointestinal neuroectodermal tumor: a case report and review of the literature.
Alyousef MJ, Alratroot JA, ElSharkawy T, Shawarby MA, Al Hamad MA, Hashem TM, Alsayyah A
Diagn Pathol 2017 Mar 20;12(1):29. doi: 10.1186/s13000-017-0620-9. PMID: 28320420Free PMC Article
Oncocytic variant of malignant gastrointestinal neuroectodermal tumor: a potential diagnostic pitfall.
Boland JM, Folpe AL
Hum Pathol 2016 Nov;57:13-16. Epub 2016 Jun 23 doi: 10.1016/j.humpath.2016.05.026. PMID: 27346570
Gastrointestinal clear cell sarcoma-like tumour of the ascending colon.
Gahanbani Ardakani A, Boyle DJ, Elton C
Ann R Coll Surg Engl 2016 Mar;98(3):e37-9. doi: 10.1308/rcsann.2016.0073. PMID: 26890846Free PMC Article
Cutaneous clear cell sarcoma: a clinicopathologic, immunohistochemical, and molecular analysis of 12 cases emphasizing its distinction from dermal melanoma.
Hantschke M, Mentzel T, Rütten A, Palmedo G, Calonje E, Lazar AJ, Kutzner H
Am J Surg Pathol 2010 Feb;34(2):216-22. doi: 10.1097/PAS.0b013e3181c7d8b2. PMID: 20087159Free PMC Article

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