Lymphoma & related disorders

Mature T/NK cell disorders

Intestinal

Intestinal T cell lymphoma, NOS



Last author update: 6 September 2022
Last staff update: 6 September 2022

Copyright: 2022, PathologyOutlines.com, Inc.

PubMed Search: Intestinal T cell lymphoma

Busra Bacik Goksu, M.D.
Carlos A. Murga-Zamalloa, M.D.
Page views in 2021: 321
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Cite this page: Goksu BB, Murga-Zamalloa CA. Intestinal T cell lymphoma, NOS. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/lymphomaintestinalT.html. Accessed December 9th, 2022.
Definition / general
  • Primary intestinal T cell lymphomas (ITCL) are rare malignancies that account for < 10% of intestinal lymphomas
  • Diagnosis of the not otherwise specified category (ITCL, NOS) is established when there is a failure of classification with the current schemes
Essential features
Terminology
  • ITCL, NOS
  • ITCL, unclassified
ICD coding
  • ICD-10: C86.2 - enteropathy type (intestinal) T cell lymphoma
  • ICD-11: 2A90.7 - enteropathy associated T cell lymphoma
Epidemiology
Sites
  • The few cases reported demonstrate involvement in the small intestine
Etiology
Diagrams / tables

Images hosted on other servers:
Differential diagnosis

Differential diagnosis of intestinal T lymphomas

Clinical features
Diagnosis
  • Computed tomography (CT) scans can be performed to show intestinal wall thickening; however, a biopsy is required for diagnosis
Radiology description
  • Intestinal wall thickening in CT scans
Prognostic factors
Case reports
Treatment
  • Similar to the systemic peripheral T cell lymphoma, NOS counterpart and includes induction with CHOP chemotherapy (cyclophosphamide, doxorubicin hydrochloride [hydroxydaunorubicin], vincristine sulfate [Oncovin] and prednisone)
Gross description
  • Involved areas are characterized by thickened rugal folds
Microscopic (histologic) description
  • Dense and extensive infiltrates that are composed of atypical lymphocytes that are medium to large in size, with irregular nuclear contours
  • Necrosis may be present
  • Epitheliotropism can be present
  • References: Diagn Pathol 2022;17:53, Clin Case Rep 2022;10:e05546
Microscopic (histologic) images

Contributed by Carlos A. Murga-Zamalloa, M.D.
Epitheliotropism of atypical infiltrates

Epitheliotropism of atypical infiltrates

Infiltrates adjacent to ulcer site

Infiltrates adjacent to ulcer site

Submucosal extension of infiltrates

Submucosal extension of infiltrates

CD3 positive infiltrates

CD3 positive infiltrates

Aberrant CD5 loss

Aberrant CD5 loss

Positive stains
Negative stains
Sample pathology report
  • Small intestine, biopsies:
    • Atypical lymphocytic infiltrates, consistent with involvement by T cell lymphoma (see comment)
    • Comment: The overall morphological and immunohistochemical findings are most consistent with T cell lymphoma unclassified (not otherwise specified, NOS). Correlation with staging and imaging studies is required. In the right clinical context, the findings can be consistent with primary intestinal T cell lymphoma, not otherwise specified (ITCL, NOS).
Differential diagnosis
Additional references
Board review style question #1

Which of the following is the correct statement regarding intestinal T cell lymphoma, not otherwise specified?

  1. All patients characteristically feature persistent diarrhea
  2. Clinical course is indolent
  3. Diagnosis requires the exclusion of celiac disease
  4. Loss of the expression of pan T cell markers is rarely observed
  5. Lymphocytic infiltrates are benign appearing and indistinguishable from inflammatory bowel disease
Board review style answer #1
C. Diagnosis requires the exclusion of celiac disease

Comment Here

Reference: Intestinal T cell lymphoma, NOS
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