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Rare disease
CASE REPORT
A Rare Case of Interdigitating Dendritic Cell Sarcoma of the Rectum: Review of Histopathology and Management Strategy
  1. Sameer A Hirji1,
  2. James C Senturk1,
  3. Jason Hornick2,
  4. Toyooki Sonoda3,
  5. Ronald Bleday1
  1. 1Department of Surgery, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts, USA
  2. 2Department of Pathology, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts, USA
  3. 3Department of Surgery, New York Presbyterian/Weill Cornell Medical Center, New York, USA
  1. Correspondence to Sameer A Hirji, shirji{at}partners.org

Summary

Interdigitating dendritic cell sarcoma (IDCS) is a rare neoplasm arising from a subclass of dendritic cells, known for their role in mediating various immunological functions, including T-cell mediated immunity. Although existing literature on IDCS is limited to scattered reports, extranodal manifestation in the gastrointestinal tract, and in particular, the rectum is extremely rare. To our knowledge, we report only the second case of IDCS arising in the rectum in a young 20-year-old man, successfully managed surgically and with a good oncological outcome. Existing literature on the incidence, pathophysiology and treatment strategies is also examined.

  • carcinogenesis
  • gastrointestinal surgery
  • general surgery
  • colon cancer

https://doi.org/10.1136/bcr-2017-221754

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    Footnotes

    • Contributors All authors have contributed significantly and equally to the manuscript. SH, JS and JH were involved in the drafting and obtaining images for manuscript. RB, TS, SH were involved in extensive editing, review of literature and finalizing the draft.

    • Competing interests None declared.

    • Patient consent Obtained.

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