Extragastrointestinal stromal tumour (EGIST) presented as a mesenteric and retroperitoneal mass.

Autor: Costa Almeida C; General Surgery, Hospital Geral (Covões), Centro Hospitalar e Universitario de Coimbra EPE, Coimbra, Portugal carloscostaalmeida@yahoo.com., Caroço TV; General Surgery, Instituto Portugues de Oncologia de Coimbra Francisco Gentil EPE, Coimbra, Portugal., Albano M; General Surgery, Hospital Geral (Covões), Centro Hospitalar e Universitario de Coimbra EPE, Coimbra, Portugal., Carvalho L; General Surgery, Hospital Geral (Covões), Centro Hospitalar e Universitario de Coimbra EPE, Coimbra, Portugal.
Jazyk: angličtina
Zdroj: BMJ case reports [BMJ Case Rep] 2019 Dec 02; Vol. 12 (12). Date of Electronic Publication: 2019 Dec 02.
DOI: 10.1136/bcr-2019-232481
Abstrakt: 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.
Competing Interests: Competing interests: None declared.
(© BMJ Publishing Group Limited 2019. No commercial re-use. See rights and permissions. Published by BMJ.)
Databáze: MEDLINE
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