Overview

NCI Definition: A primary cutaneous T-cell lymphoma. It usually presents with a solitary plaque or tumor on the face, neck, or upper trunk. Morphologically, it is composed of small to medium-sized CD4-positive, CD8-negative, and CD30-negative pleomorphic T-lymphocytes. A small number of large pleomorphic T-lymphocytes may also be present. The lymphocytic infiltrate is dermal. Focal epidermotropism and subcutaneous involvement may be present. The prognosis is favorable. [1]

Primary cutaneous CD4-positive small/medium T-cell lymphoproliferative disorders most frequently harbor alterations in SMARCA4 [2].

Most Commonly Altered Genes in Primary Cutaneous CD4-Positive Small/Medium T-Cell Lymphoproliferative Disorder

SMARCA4 R1093* and SMARCA4 Mutation are the most common alterations in primary cutaneous CD4-positive small/medium T-cell lymphoproliferative disorder [2].

Top Alterations in Primary Cutaneous CD4-Positive Small/Medium T-Cell Lymphoproliferative Disorder

Disease Details

Synonyms
Primary Cutaneous CD4-Positive Small/Medium-Sized Pleomorphic T-Cell Lymphoproliferative Disorder, Primary Cutaneous CD4 Positive Small/Medium T-Cell Lymphoproliferative Disorder, Primary Cutaneous CD4-Positive Small/Medium-Sized T-Cell Lymphoproliferative Disorder
Parent(s)
Mature T-Cell and NK-Cell Neoplasm
OncoTree Name
Primary Cutaneous CD4 Positive Small/Medium T-Cell Lymphoproliferative Disorder
OncoTree Code
PCSMTPLD

References

1. National Cancer Institute. NCI Thesaurus Version 18.11d. https://ncit.nci.nih.gov/ncitbrowser/ [2018-08-28]. [2018-09-21].

2. The AACR Project GENIE Consortium. AACR Project GENIE: powering precision medicine through an international consortium. Cancer Discovery. 2017;7(8):818-831. Dataset Version 8. This dataset does not represent the totality of the genetic landscape; see paper for more information.

3. All assertions and clinical trial landscape data are curated from primary sources. You can read more about the curation process here.