Skip to main content

Advertisement

SpringerLink
Log in

Malignant Gastrointestinal Neuroectodermal Tumour—Case Report with Review of Literature

  • Case Report
  • Published:
Journal of Gastrointestinal Cancer Aims and scope Submit manuscript

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3

Data Availability

The authors confirm that the data supporting the findings of this study are available within the article (and/or) its supplementary material.

References

  1. Zambrano E, Reyes-Mugica M, Franchi A, Rosai J. An osteoclast-rich tumor of the gastrointestinal tract with features resembling clear cell sarcoma of soft parts: reports of 6 cases of a GIST simulator. Int J Surg Pathol. 2003;11(2):75–81. https://doi.org/10.1177/106689690301100202.

    Article  PubMed  Google Scholar 

  2. Stockman DL, Miettinen M, Suster S, Spagnolo D, Dominguez-Malagon H, Hornick JL, et al. Malignant gastrointestinal neuroectodermal tumor: clinicopathologic, immunohistochemical, ultrastructural and molecular analysis of 16 cases with a reappraisal of clear cell sarcoma-like tumors of the gastrointestinal tract. Am J Surg Pathol. 2012;36(6):857–68. https://doi.org/10.1097/PAS.0b013e31824644ac.

    Article  PubMed  PubMed Central  Google Scholar 

  3. Green C, Spagnolo D, Robbins P, Fermoyle S, Wong D. Clear cell sarcoma of the gastrointestinal tract and malignant gastrointestinal neuroectodermal tumour: distinct or related entities? A review. Pathology. 2018;50(5):490–8. https://doi.org/10.1016/j.pathol.2018.05.001.

    Article  PubMed  Google Scholar 

  4. Libertini M, Thway K, Noujamim J, Puls F, Messiou C, Fisher C, et al. Clear cell sarcoma-like tumor of the gastrointestinal tract: clinical outcome and pathologic features of a molecularly characterized tertiary center case series. Anticancer Res. 2018;38(3):1479–83. https://doi.org/10.21873/anticanres.12374.

    Article  PubMed  Google Scholar 

  5. Sonai MK, Rastogi S, Madhusudhan KS, Gupta SD, Das P. Clear cell sarcoma like tumor of gastrointestinal tract: experience of three cases and review of literature. Indian J Pathol Microbiol. 2020;63:90–5. https://doi.org/10.4103/ijpm_195_19.

    Article  PubMed  Google Scholar 

  6. Chang B, Yu L, Guo L, Sheng W, Wang L, Lao I, et al. Malignant gastrointestinal neuroectodermal tumor clinicopathologic, immunohistochemical, and molecular analysis of 19 cases. Am J Surg Pathol. 2020;44(4):456–66. https://doi.org/10.1097/PAS.0000000000001396.

    Article  PubMed  Google Scholar 

  7. Wang J, Thway K. Clear cell sarcoma–like tumor of the gastrointestinal tract: an evolving entity. Arch Pathol Lab Med. 2015;139(3):407–12. https://doi.org/10.5858/arpa.2013-0547-RS.

    Article  PubMed  Google Scholar 

  8. Insabato L, Guadagno E, Natella V, Somma A, Mainenti PP. An unusual association of malignant gastrointestinal neuroectodermal tumor (clear cell sarcoma-like) and Ewing sarcoma. Pathol Res Pract. 2015;211(9):688–92. https://doi.org/10.1016/j.prp.2015.06.001.

    Article  CAS  PubMed  Google Scholar 

  9. Alyousef MJ, Alratoot JA, et al. Malignant gastrointestinal neuroectodermal tumor: a case report and review of the literature. Diagn Pathol. 2017;12:29. https://doi.org/10.1186/s13000-017-0620-9.

    Article  PubMed  PubMed Central  Google Scholar 

  10. Antonescu CR, Nafa K, Segal NH, Dal Cin P, Ladanyi M. EWS-CREB1: a recurrent variant fusion in clear cell sarcoma—association with gastrointestinal location and absence of melanocytic differentiation. Clin Cancer Res. 2006;12(18):5356–62. https://doi.org/10.1158/1078-0432.CCR-05-2811.

    Article  CAS  PubMed  Google Scholar 

  11. Thway K, Judson I, Fisher C. Clear cell sarcoma-like tumor of the gastrointestinal tract, presenting as a second malignancy after childhood hepatoblastoma. Case Rep Med. 2014:Article ID 984369. https://doi.org/10.1155/2014/984369.

  12. Subbiah V, Holmes O, Gowen K, Spritz D, Amini B, Wang W, et al. Activity of c-met/ALK inhibitor crizotinib and multi-kinase VEGF inhibitor pazopanib in metastatic gastrointestinal neurorectodermal tumour (GNET) harbouring EWSR1-CREB1 fusion. Oncology. 2016;91(6):348–353.10.1159/000449204.

    Article  CAS  Google Scholar 

  13. Ekfors TO, Kujari H, Isomäki M. Clear cell sarcoma of tendons and aponeuroses (malignant melanoma of soft parts) in the duodenum: the first visceral case. Histopathology. 1993;22(3):255–60. https://doi.org/10.1111/j.1365-2559.1993.tb00115.x.

    Article  CAS  PubMed  Google Scholar 

  14. Singh D, Atieh MK, Russell MA, Kittaneh M. Malignant gastrointestinal neuroectodermal tumor (GNET) with prolonged disease-free survival after platinum-based chemotherapy. Case Rep Oncol Med. 2020:Article ID 8880202. https://doi.org/10.1155/2020/8880202.

  15. Donner LR, Trompler RA, Dobin S. Clear cell sarcoma of the ileum: the crucial role of cytogenetics for the diagnosis. Am J SurgPathol. 1998;22(1):121–4. https://doi.org/10.1097/00000478-199801000-00016.

    Article  CAS  Google Scholar 

  16. Pauwels P, Debiec-Rychter M, Sciot R, Vlasveld T, den Butter B, Hagemeijer A, et al. Clear cell sarcoma of the stomach. Histopathology. 2002;41(6):526–30. https://doi.org/10.1046/j.1365-2559.2002.01509.x.

    Article  CAS  PubMed  Google Scholar 

  17. Venkatraman G, Quinn AM, et al. Clear cell sarcoma of the small bowel: a potential pitfall. APMIS. 2005;113:716–9. https://doi.org/10.1111/j.1600-0463.2005.apm_243.x.

    Article  Google Scholar 

  18. Friedrich N, Testi M, Moiraghi L, et al. Clear cell sarcoma-like tumor with osteoclast-like giant cells in the small bowel: further evidence for a new tumor entity. Int J Surg Pathol. 2005;13(4):313–8. https://doi.org/10.1177/106689690501300402.

    Article  Google Scholar 

  19. Taminelli L, Zaman K, Gengler C, et al. Primary clear cell sarcoma of the ileum: an uncommon and misleading site. Virchows Arch. 2005;447(4):772–7. https://doi.org/10.1007/s00428-005-0019-y.

    Article  PubMed  Google Scholar 

  20. Huang W, Zhang X, Li D, et al. Osteoclast-rich tumor of the gastrointestinal tract with features resembling those of clear cell sarcoma of soft parts. Virchows Arch. 2006;448:200–3. https://doi.org/10.1007/s00428-0050051-y.

    Article  PubMed  Google Scholar 

  21. Granville L, Hicks J, Popek E, Dishop M, Tatevian N, Lopez-Terrada D. Visceral clear cell sarcoma of soft tissue with confirmation by EWS-ATF1 fusion detection. Ultrastruct Pathol. 2006;30(1):111–8. https://doi.org/10.1080/01913120500406400.

    Article  PubMed  Google Scholar 

  22. Comin CE, Novelli L, Tornaboni D, Messerini L. Clear cell sarcoma of the ileum: report of a case and review of literature. Virchows Arch. 2007;451:839–45. https://doi.org/10.1007/s00428-007-0454-z.

    Article  PubMed  Google Scholar 

  23. Lyle PL, Amato CM, Fitzpatrick JE, Robinson WA. Gastrointestinal melanoma or clear cell sarcoma? Molecular evaluation of 7 cases previously diagnosed as malignant melanoma. Am J SurgPathol. 2008;32:858–66. https://doi.org/10.1097/PAS.0b013e31815b8288.

    Article  Google Scholar 

  24. Lagmay JP, Ranalli M, Arcila M, Baker P. Clear cell sarcoma of the stomach. Pediatr Blood Cancer. 2009;53:214–6. https://doi.org/10.1002/pbc.22014.

    Article  PubMed  Google Scholar 

  25. Terazawa K, Otsuka H, Morita N, Yamashita K, Nishitani H. Clear-cell sarcoma of the small intestine detected by FDGPET/CT during comprehensive examination of an inflammatory reaction. J Med Investig. 2009;56:70–5. https://doi.org/10.2152/jmi.56.70.

    Article  Google Scholar 

  26. Joo M, Chang SH, Kim H, Gardner JM, Ro JY. Primary gastrointestinal clear cell sarcoma: report of 2 cases, one case associated with IgG4-related sclerosing disease, and review of literature. Ann Diagn Pathol. 2009;13(1):30–5. https://doi.org/10.1016/j.anndiagpath.2008.10.003.

    Article  PubMed  Google Scholar 

  27. D’Amico FE, Ruffolo C, Romeo S, Massani M, Dei Tos AP, Bassi N. Clear cell sarcoma of the ileum: report of a case and review of the literature. Int J Surg Pathol. 2012;20:401–6. https://doi.org/10.1177/1066896911428073.

    Article  PubMed  Google Scholar 

  28. Shenjere P, Salman WD, Singh M, Mangham DC, Williams A, Eyden BP, et al. Intra-abdominal clear-cell sarcoma: a report of 3 cases, including 1 case with unusual morphological features, and review of the literature. Int J Surg Pathol. 2012;20:378–85. https://doi.org/10.1177/1066896911425485.

    Article  PubMed  Google Scholar 

  29. Yang JC, Chou AJ, Oeffinger KC, La Quaglia MP, Wolden SL. Clear cell sarcoma of the gastrointestinal tract after very low dose therapeutic radiation therapy: a case report. J Pediatr Surg. 2012;47:1943–5. https://doi.org/10.1016/j.jpedsurg.2012.08.014.

    Article  PubMed  Google Scholar 

  30. Balkaransingh P, Saad S, Govil S, Thind P. Clear cell sarcoma of the gastrointestinal tract presenting as a second malignant neoplasm following neuroblastoma in infancy. Pediatr Blood Cancer. 2012;58(3):481–2. https://doi.org/10.1002/pbc.23330.

    Article  PubMed  Google Scholar 

  31. Lasithiotakis K, Protonotarios A, Lazarou V, Tzardi M, Chalkiadakis G. Clear cell sarcoma of the jejunum: a case report. World J Surg Oncol. 2013;11:17. https://doi.org/10.1186/1477-7819-11-17.

    Article  PubMed  PubMed Central  Google Scholar 

  32. Zhao Z, Zhang D, Li W, Zhang L, Li Z, Zhou J. Primary malignant neuroectodermal tumor of the ileum with predominantly uncommon pseudopapillary architecture. Int J Clin Exp Pathol. 2014;7(12):8967–71.

    PubMed  PubMed Central  Google Scholar 

  33. Kong J, Li N, Wu S, Guo X, CongyouGu FZ. Malignant gastrointestinal neuroectodermal tumor: a case report and review of the literature. Oncol Lett. 2014;8(6):2687–90. https://doi.org/10.3892/ol.2014.2524.

    Article  PubMed  PubMed Central  Google Scholar 

  34. Raskin GA, Pozharisski KM, Iyevleva AG, Rikov IV, Orlova RV, Imyanitov EN. Unusual clinical presentation of gastrointestinal clear cell sarcoma. Gastrointest Tumors. 2015;2:83–8. https://doi.org/10.1159/000430881.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  35. Yegen G, Güllüoğlu M, Mete Ö, Önder S, Kapran Y. Clear cell sarcoma-like tumor of the gastrointestinal tract: a case report and review of the literature. Int J Surg Pathol. 2015;23(1):61–7. https://doi.org/10.1177/1066896914547046.

    Article  PubMed  Google Scholar 

  36. Keditsu KK, Patkar S, Shrikhande S, Goel M. Gastrointestinal neuroectodermal tumor: a diagnostic dilemma. Indian J Surg. 2016;79(2):166–8. https://doi.org/10.1007/s12262-016-1499-5.

    Article  PubMed  PubMed Central  Google Scholar 

  37. Boland JM, Folpe AL. Oncocytic variant of malignant gastrointestinal neuroectodermal tumor: a potential diagnostic pitfall. Hum Pathol. 2016;57:13–6. https://doi.org/10.1016/j.humpath.05.026.

    Article  CAS  PubMed  Google Scholar 

  38. Ardakani A, Boyle D, Elton C. Gastrointestinal clear cell sarcoma-like tumour of the ascending colon. Ann R Coll Surg Engl. 2016;98(3):e37–9. https://doi.org/10.1308/rcsann.2016.0073.

    Article  Google Scholar 

  39. Prado G, Gorcey SA, Szallasi A, Maluf H. Indolent gastrointestinal neuroectodermal tumor (GNET) of the colon: a new entity? J Cancer Sci Res. 2017;3:S2. https://doi.org/10.4172/2576-1447.1000S2-007.

    Article  Google Scholar 

  40. Kansal S, Rao S. Malignant gastrointestinal neuroectodermal tumor: a unique rare neoplasm. Indian J Surg Oncol. 2017;8(4):630–3. https://doi.org/10.1007/s13193-017-0654-1.

    Article  PubMed  PubMed Central  Google Scholar 

  41. Kato T, Ichihara S, Gotoda H, Muraoka S, Kubo T, Sugita S, et al. Imprint cytology of clear cell sarcoma-like tumor of the gastrointestinal tract in the small intestine: a case report. Diagn Cytopathol. 2017;45(12):1137–41. https://doi.org/10.1002/dc.23786.

    Article  PubMed  Google Scholar 

  42. Song S, Shin J, JooRyu H, Kim D, Park S. Successful surgical treatment of a recurrent esophageal malignant gastrointestinal neuroectodermal tumor. Korean J Thorac Cardiovasc Surg. 2018;51(2):142–5. https://doi.org/10.5090/kjtcs.2018.51.2.142.

    Article  PubMed  PubMed Central  Google Scholar 

  43. Segawa K, Sugita S, Aoyama T, Kubo T, Asanuma H, Sugawara T, et al. Detection of specific gene rearrangements by fluorescence in situ hybridization in 16 cases of clear cell sarcoma of soft tissue and 6 cases of clear cell sarcoma-like gastrointestinal tumor. Diagn Pathol. 2018;13(1):73. https://doi.org/10.1186/s13000-018-0752-6.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  44. Mukherjee U, Khurana A, Patil N. An unusual intra-abdominal tumor of the gastrointestinal tract. Indian J Pathol Microbiol. 2018;61(3):457–8. https://doi.org/10.4103/IJPM.IJPM_658_17.

    Article  PubMed  Google Scholar 

  45. Breton S, Dubois M, GeayJ F, Gillebert Q, Tordjman M, Guinebretière JM, et al. Clear cell sarcoma or gastrointestinal neuroectodermal tumor (GNET) of the tongue? Case report and review of the literature of extremely rare tumor localization. Ann Pathol. 2018;39(2):167–71. https://doi.org/10.1016/j.annpat.2018.10.004.

    Article  PubMed  Google Scholar 

  46. Buiar PG, de Lima Gossling GC, Schwartsmann G. A young man with constitutional symptoms and a small bowel vegetating lesion. Braz J Oncol. 2018;14(49):1–4. https://doi.org/10.5935/2526-8732.20180001.

    Article  Google Scholar 

  47. Huang G, Chen Q, Zhong L, Chen H, Zhang H, Liu X, et al. Primary malignant gastrointestinal neuroectodermal tumor occurring in the ileum with intra-abdominal granulomatous nodules: a case report and review of the literature. Oncol Lett. 2019;17(4):3899–909. https://doi.org/10.3892/ol.2019.10060.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  48. Ito A, Kurashige M, Murakami H, Koide T, Ando R, Kiriyama AK, et al. A case of clear cell sarcoma-like tumor of the gastrointestinal tract (CCSLGT) with two EWSR1-ATF1 fusions. Hum Pathol Case Rep. 2019;17:200300. https://doi.org/10.1016/j.hpcr.2019.200300.

    Article  Google Scholar 

  49. Askan G, Kombak F, Seven I, Basturk O. Clear cell sarcoma-like tumor of the gastrointestinal tract. J Gastrointest Cancer. 2019;50(3):651–6. https://doi.org/10.1007/s12029-018-0069-4.

    Article  PubMed  PubMed Central  Google Scholar 

  50. Bossenmeyer C, Duchalais E, Moreau A, Bossard C. Unexpected DOG1 expression in gastrointestinal neuroectodermal tumor of the rectum: a diagnostic pitfall. Hum Pathol Case Rep. 2019;17:200317. https://doi.org/10.1016/j.ehpc.2019.200317.

    Article  Google Scholar 

  51. Zhan M, Yu J, Luo R, Hou Y. Malignant gastrointestinal neuroectodermal tumor, presenting as a second malignancy after gastric adenocarcinoma: a case report and literature review. J Gastrointest Oncol. 2019;10(6):1144–50. https://doi.org/10.21037/jgo.2019.08.04.

    Article  PubMed  PubMed Central  Google Scholar 

  52. Oye M, Kandah F, Mikulic S, Patel B, Villabon D, Gopinath A. Malignant gastrointestinal neuroectodermal tumor presenting with recurrent ascites. Curr Probl Cancer Case Rep. 2020;1:100017. https://doi.org/10.1016/j.cpccr.2020.100017.

    Article  Google Scholar 

  53. Kumar N, Bhoriwal S, Yadav R, Deo SVS. Clear cell sarcoma of sigmoid colon: a rare malignancy with common clinical manifestations. J Gastrointest Cancer. 2020;51(3):1070–3. https://doi.org/10.1007/s12029-020-00413-z.

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Pathology, Amrita Institute of Medical Sciences, Kochi, India

    Aditi Damle, Niha Rebecca Mathews & Roopa Rachel Paulose

  2. Department of Radiation Oncology, Amrita Institute of Medical Sciences, Kochi, India

    Yarlagadda Sreenija

  3. Department of GI surgery, Amrita Institute of Medical Sciences, Kochi, India

    Krishnanunni Nair

  4. Department of Medical Oncology, Amrita Institute of Medical Sciences, Kochi, India

    Arun Philp & Keechilat Pavithran

Authors
  1. Aditi Damle
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Yarlagadda Sreenija
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Niha Rebecca Mathews
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Krishnanunni Nair
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Arun Philp
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Keechilat Pavithran
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Roopa Rachel Paulose
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Roopa Rachel Paulose.

Ethics declarations

Conflict of Interest

The authors declare that they have no conflict of interest.

Ethics Approval

NA

Consent to Participate

Yes

Consent for Publication

Yes

Additional information

Suggestions

Further studies on archived cases and forming global clinical cohorts with inter-institutional colloborations will help to improve the understanding of the pathogenesis of the tumour and aid in establising guidelines/standards for therapeutic care.

Publisher’s Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Damle, A., Sreenija, Y., Mathews, N.R. et al. Malignant Gastrointestinal Neuroectodermal Tumour—Case Report with Review of Literature. J Gastrointest Canc 52, 1125–1130 (2021). https://doi.org/10.1007/s12029-020-00575-w

Download citation

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s12029-020-00575-w

Search

Navigation