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

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 sca...

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Bibliographic Details
Published in:BMJ case reports 2019-12, Vol.12 (12), p.e232481
Main Authors: Costa Almeida, Carlos, Caroço, Teresa Vieira, Albano, Miguel, Carvalho, Luís
Format: Article
Language:English
Subjects:
Abdomen
Aged
Anemia
Anemia - etiology
Blood tests
Bubbles
Case reports
Colon
Edema
Fatal Outcome
Gastrointestinal cancer
Gastrointestinal Stromal Tumors - diagnostic imaging
Gastrointestinal Stromal Tumors - pathology
Gastrointestinal Stromal Tumors - surgery
general surgery
Humans
Inhibitor drugs
Lymphatic system
Male
Medical imaging
Medical prognosis
Mesenteric Arteries - pathology
Mesentery
Metastasis
Neoplasm Invasiveness
Rare Disease
Retroperitoneal Neoplasms - diagnostic imaging
Retroperitoneal Neoplasms - pathology
Retroperitoneal Neoplasms - surgery
Surgery
surgical oncology
Targeted cancer therapy
Tumors
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Description
Summary: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.
ISSN:1757-790X
1757-790X
DOI:10.1136/bcr-2019-232481