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Case report
Extragastrointestinal stromal tumour (EGIST) presented as a mesenteric and retroperitoneal mass
  1. Carlos Costa Almeida1,
  2. Teresa Vieira Caroço2,
  3. Miguel Albano1 and
  4. Luís Carvalho1
  1. 1 General Surgery, Hospital Geral (Covões), Centro Hospitalar e Universitario de Coimbra EPE, Coimbra, Portugal
  2. 2 General Surgery, Instituto Portugues de Oncologia de Coimbra Francisco Gentil EPE, Coimbra, Portugal
  1. Correspondence to Dr Carlos Costa Almeida; carloscostaalmeida{at}yahoo.com

Abstract

Extragastrointestinal stromal tumour (EGIST) occurs outside the gastrointestinal tract and has histopathological and molecular characteristics similar to gastrointestinal stromal tumour (GIST). This tumour is rare and aggressive. A male patient was admitted with anaemia and lower limb oedema. CT scan showed a tumour in the mesentery and retroperitoneum, suspected to be a small bowel GIST. During laparotomy an unresectable mass was found compressing the retroperitoneal structures. Pathology and immunohistochemistry (CD117) confirmed an EGIST. EGIST arises from Cajal-like cells or from pluripotent stem cells outside the gastrointestinal tract. It is aggressive and has a worse prognosis than GIST. Immunohistochemistry is crucial for diagnosis. Surgery aimed at debulking as much of a tumour mass as possible is the cornerstone of treatment. The role of imatinib is not clear. EGIST is rare and has a bad prognosis, and there is no consensus on grading and management. A low threshold of suspicion is crucial for early diagnosis.

  • general surgery
  • surgical oncology

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Footnotes

  • Contributors CCA: data collection, writing, review. TVC: writing, review. MA, LC: review.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Patient consent for publication Next of kin consent obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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