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Case report
Rare case of a duodenal de novo dedifferentiated gastrointestinal stromal tumour
  1. Pritik A Shah1,
  2. Raghavendra Babu1,
  3. Sowmya B Uthaiah2,3 and
  4. Shekar Patil4
  1. 1Department of Hepato Pancreato Biliary (HPB) Surgery and Liver Transplant, HealthCare Global Enterprises Ltd, Bangalore, Karnataka, India
  2. 2Department of Histopathology, HealthCare Global Enterprises Ltd, Bangalore, Karnataka, India
  3. 3Department of Histopathology, Strand Life Sciences, Bangalore, Karnataka, India
  4. 4Department of Medical Oncology, HealthCare Global Enterprises Ltd, Bangalore, Karnataka, India
  1. Correspondence to Dr Raghavendra Babu; raghavendrababuj{at}gmail.com

Abstract

Gastrointestinal stromal tumours (GISTs) are the most common mesenchymal neoplasms of the gastrointestinal tract. Dedifferentiation in these tumours occurs rarely, and when it does occurs most commonly after prolonged treatment with imatinib. We report the case of a 64-year-old man who presented with a mass of 8×7×3 cm dimensions involving the duodenum and head of the pancreas. On histopathology, areas of anaplastic tumour cells were negative for DOG-1, c-kit, CD-34, desmin and panCK along with a molecular level study showing wild-type KIT and PDGFRA (platelet-derived growth factor receptor alpha) gene. Based on focal GIST-like areas and CD117 positivity and absence of prior therapy, the diagnosis of a de novo dedifferentiated GIST was made. These tumours need to be reported as they pose a diagnostic challenge and their predicted response rated to targeted molecular therapies are unclear as compared with their c-kit positive counterparts.

  • pathology
  • gastrointestinal surgery
  • tyrosine Kinase Inhibitor

https://doi.org/10.1136/bcr-2020-241442

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    Footnotes

    • Contributors PAS, RB and SBU were the contributors to the conception of the case report. PAS and RB, from the HPB Surgery Department drafted the case report. SBU, a histopathologist, interpreted and reviewed the aspects of pathology. SP, a medical oncologist, and RB, a HPB surgeon, reviewed and revised it for important intellectual content. Final approval of the report was obtained from all contributing authors.

    • 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.

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