Name

Primary effusion lymphoma

ICD-O-3 Morphology

9678/3: Primary effusion lymphoma
Effective 2001 and later

Reportable

for cases diagnosed 2001 and later

Primary Site(s)

See Module 7
Most common sites of involvement: body cavities (pleural, pericardial, and peritoneal). Extracavity lesions may occur in: GI tract, skin, lung, and CNS, and lymph nodes.

Grade

Not Applicable

Module Rule

None

Alternate Names

Body-cavity based lymphoma [OBS]
Extracavitary PEL
PEL

Definition

Primary effusion lymphoma (PEL) is a large B-cell neoplasm usually presenting as serous effusions without detectable tumor masses. It is universally associated with the human herpesvirus 8 (HHV8). It most often occurs in the setting of immunodeficiency. Some patients with PEL secondarily develop solid tumors in adjacent structures such as the pleura. Rare HHV8-positive lymphomas indistinguishable fro PEL present as solid tumor masses and have been termed extracavitary PEL.

Abstractor Notes

PEL is universally associated with human herpes virus 8 (HH8).

PEL is unusual in that the majority of cases arise in body cavities (typically only one body cavity is involved). Because there are no ICD-O-3 codes for the pleural space, pericardium, or peritoneal cavity, code the primary site to:
1. Pleura (C384) when it occurs in the pleural cavity
2. Pericardium (C380) when it occurs in the pericardium
3. Peritoneum (C482) when it occurs in the peritoneal cavity.

PEL is generally resistant to cancer chemotherapy drugs that are active against other lymphomas, and carries a poor prognosis.

Definitive Diagnostic Methods

Cytology
Genetic testing
Histologic confirmation
Immunophenotyping

Genetics Data

IG genes (so-called genotypic infidelity) rearrangement
Immunoglobulin genes clonally rearranged and hypermutated
TR genes are clonally rearranged

Immunophenotyping

BCL6 absent
CD19 lacking
CD20 lacking
CD30 (often demonstrable)
CD38 (often demonstrable)
CD45 expression
CD79a lacking
CD138 (often demonstrable)
EMA (often demonstrable)
HLA-DR (often demonstrable)
LANA (ORF 73) (HHV8-associated latent protein)
Surface and cytoplasmic immunoglobulin absent
T-NK cell antigens expressed (in extracavitary PEL)
VS38c (often demonstrable)

Treatments

Chemotherapy
Immunotherapy
Other therapy

Transformations to

There are no known transformations

Transformations from

There are no known transformations

Corresponding ICD-9 Codes

200.8 Other named variants, lymphoma

Corresponding ICD-10 Codes

C83.8 Other types of diffuse non-Hodgkin's lymphoma

Corresponding ICD-10-CM Codes (U.S. only)

C83.8 Other non-follicular lymphoma (effective October 01, 2015)

Signs and Symptoms

Drenching night sweats
Fever (for no known reason)
Pain in the chest, abdomen, or bones (for no known reason)
Painless swelling in the lymph nodes
Skin rash or itchy skin
Weight loss (for no known reason)

Diagnostic Exams

CT (CAT) scan
Complete blood count (CBC)
Laparoscopy (rarely performed)
Laparotomy (rarely performed)
MRI (magnetic reasonance imaging)
PET (positron emission tomography) scan

Progression and Transformation

None

Epidemiology and Mortality

Age: young or middle aged HIV patients
Sex: male predominance
Survival: <6 months

Sources

Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J (Eds):
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues (Revised 4th edition)
IARC: Lyon 2017
Section: Mature B-cell neoplasms
Pages: 323-324

International Classification of Diseases for Oncology, Third Edition, First Revision. Geneva: World Health Organization, 2013.
Section: ICD-O-3.1 (2011) Morphological Codes
Pages: http://codes.iarc.fr/codegroup/2

National Cancer Institute
Section: General Information About Adult Non-Hodgkin Lymphoma (NHL)
Pages: https://www.cancer.gov/types/lymphoma/hp/adult-nhl-treatment-pdq
Glossary