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Primary Cutaneous CD8-Positive Aggressive Epidermotropic Cytotoxic T-Cell Lymphoma

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Overview

NCI Definition: An aggressive, epidermotropic primary cutaneous T-cell lymphoma. It is characterized by the presence of eruptive papules and nodules, or tumors, which may be localized or disseminated. Morphologically, it is composed of small to medium-sized, or medium-sized to large, CD8-positive T-lymphocytes with pleomorphic or blastic nuclei. Epidermotropism is often pronounced. Angiocentricity and angioinvasion may be present. [1]

Primary cutaneous CD8-positive aggressive epidermotropic cytotoxic T-cell lymphomas most frequently harbor alterations in TP63, TET2, SMAD2, ROBO1, and RHOA [2].

Most Commonly Altered Genes in Primary Cutaneous CD8-Positive Aggressive Epidermotropic Cytotoxic T-Cell Lymphoma

TP63 V179M, TP63 Mutation, TET2fs, TET2 R686*, and TET2 Nonsense are the most common alterations in primary cutaneous CD8-positive aggressive epidermotropic cytotoxic T-cell lymphoma [2].

Top Alterations in Primary Cutaneous CD8-Positive Aggressive Epidermotropic Cytotoxic T-Cell Lymphoma

Clinical Trials

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Disease Details

Synonyms
Primary Cutaneous CD8 Positive Aggressive Epidermotropic Cytotoxic T-Cell Lymphoma, Primary Cutaneous CD8-Positive Aggressive Epidermotropic T-Cell Lymphoma, Primary Cutaneous Aggressive Epidermotropic CD8-Positive T-Cell Lymphoma
Parent(s)
Primary Cutaneous T-Cell Non-Hodgkin Lymphoma
OncoTree Name
Primary Cutaneous CD8 Positive Aggressive Epidermotropic Cytotoxic T-Cell Lymphoma
OncoTree Code
PCAECTCL

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.