Overview

NCI Definition: An aggressive non-Hodgkin B-cell lymphoma composed of large cells, presenting as a serous effusion without detectable tumor masses. It is universally associated with human herpes virus 8 (HHV-8)/Kaposi sarcoma herpes virus (KSHV) [HHV-8/KSHV]. It mostly occurs in the setting of immunodeficiency; most cases have been reported in HIV positive patients. The most common sites of involvement are the pleural, pericardial, and peritoneal cavities. The prognosis is extremely unfavorable. [1]

Primary effusion lymphomas most frequently harbor alterations in WRN, TET2, STAT3, SETD2, and PIK3C2B [2].

Most Commonly Altered Genes in Primary Effusion Lymphoma

WRN Mutation, WRN K541E, TET2 P1012L, TET2 Mutation, and STAT3 Mutation are the most common alterations in primary effusion lymphoma [2].

Top Alterations in Primary Effusion Lymphoma

Significant Genes in Primary Effusion Lymphoma

AFF1 +

ALK +

BCL2 +

BCL6 +

ELL +

IRF4 +

KMT2A +

MLLT1 +

MLLT10 +

MLLT3 +

MLLT4 +

MYC +

NRIP3 +

Disease Details

Synonyms
PEL, Primary effusion lymphoma, primary effusion lymphoma
Parent(s)
Diffuse Large B-Cell Lymphoma
OncoTree Name
Primary Effusion Lymphoma
OncoTree Code
PEL

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.