Search Database ICD-O-3 Code Lists

Name

Primary effusion lymphoma (PEL)

ICD-O-3 Morphology

9678/3: Primary effusion lymphoma
Effective 2001 and later

Reportable

for cases diagnosed 1978 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.

See abstractor notes

Coding Manual: Hematopoietic Coding Manual (PDF)

Abstractor Notes

Primary effusion lymphoma (PEL) is part of the Mature B-cell neoplasms lineage table in the WHO 5th edition of Hematolymphoid Tumors. (See Appendix B in the Hematopoietic Manual, Table B15)

For primary effusion lymphoma, these arise in the pleural, pericardial and abdominal body cavities. Extracavitary primary effusion lymphoma involves lymph nodes and extranodal sites. Associated with HIV/AIDS.

Extracavitary PEL (EC-PEL) is a related entity presenting with a tumour mass, often at extranodal sites, or lymph nodes.

For fibrin-associated large B-cell lymphoma (FA-LBCL), these arise in sites of chronic fibrin deposition including cyst and pseudocyst cavities, in the peri-implant space of breast implants, and in chronic hematomas, as well as in intravascular or intracardiac locations, such as the surface of cardiac myxomas}, endovascular grafts, and prosthetic cardiac valves.

For fluid overload-associated large B-cell lymphoma (FL-LBCL), a majority of cases occur in the pleural cavity, followed by the pericardial and abdominal cavities.

Diagnostic Confirmation

This histology can be determined by positive histology (including peripheral blood) with or without genetics and/or immunophenotyping. Review the Definitive Diagnostic Methods, Immunophenotyping and Genetics Data sections below, and the instructions in the Hematopoietic Manual for further guidance on assigning Diagnostic confirmation.

Module Rule

None

Alternate Names

Body-cavity based lymphoma [OBS]
Extracavitary primary effusion lymphoma (EC-PEL)
KSHV/HHV8-negative effusion based lymphoma

Definition

Primary effusion lymphoma (PEL) is a large B-cell lymphoma presenting as a pleural, pericardial, and/or peritoneal serous effusion in the absence of lymph node involvement or an extranodal mass lesion, except for the rare presence of a body cavity–related tumor mass. It is consistently associated with Kaposi sarcoma–associated herpesvirus / human herpesvirus 8 (KSHV/HHV8) and usually coinfected with EBV. Extracavitary PEL (EC-PEL) is a related entity presenting with a tumor mass, often at extranodal sites. (WHO 5th ed)

Fibrin associated large B-cell lymphoma (FA-LBCL) arises in sites of chronic fibrin deposition including cyst and pseudocyst cavities}, in the peri-implant space of breast implants, and in chronic hematomas, as well as in intravascular or intracardiac locations, such as the surface of cardiac myxomas, endovascular grafts, and prosthetic cardiac valves. (WHO 5th edition).

Fluid overload–associated large B-cell lymphoma (FO-LBCL) is a B-cell neoplasm presenting as serous effusions without detectable tumor masses, often in patients with fluid overload states. It is not associated with KSHV/HHV8. (WHO 5th edition)

Definitive Diagnostic Methods

Histologic confirmation
Immunohistochemistry
Immunophenotyping

Genetics Data

None

Immunophenotyping

CD20- (no expression/negative)
CD30+ (expression/positive)
CD138+ (positive/expression)
EBER+ (positive/expression) (EBV-encoded small RNA is nearly always positive)
EMA+ (expression/positive)
IRFR (MUMI)+ (expression/positive)
KSHV/HHV8+ (expression/positive)
CD45- (no expression/negative)
HAL-DR+ (expression/positive)
VS38c+ (expression/positive)

Treatments

Chemotherapy
Immunotherapy
Other therapy

Transformations to

There are no known transformations

Transformations from

There are no known transformations

Same Primaries

Corresponding ICD-10 Codes (Cause of Death codes only)

C83.8 Other types of diffuse non-Hodgkin lymphoma

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

C83.8_ Other non-follicular lymphoma (effective October 01, 2015)
C83.8A Other non-follicular lymphoma, in remission (effective October 01, 2024)

Signs and Symptoms

Drenching night sweats
Fatigue
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

Blood chemistry studies
Bone marrow aspiration and biopsy
CT (CAT) scan
Cytogenetic analysis
Flow cytometry
Immunohistochemistry
Immunophenotyping
Laparoscopy (rarely performed)
Laparotomy (rarely performed)
Lymph node biopsy
MRI (magnetic reasonance imaging)
PET (positron emission tomography) scan

Progression and Transformation

None

Epidemiology and Mortality

Age: wide age range (25-91)
Sex: some specific anatomic locations have a sex bias
Survival: <6 months

Sources

WHO Classification of Tumours Editorial Board. Haematolymphoid tumours. Lyon (France): International Agency for Research on Cancer; 2024. (WHO classification of tumours series, 5th ed.; vol. 11). https://publications.iarc.who.int/637.
Section: Large B-cell lymphomas/KSHV HHV8-associated B-cell lymphoid proliferations and lymphomas
Pages: Part B: 500-505, 541-543

International Classification of Diseases for Oncology, 3rd edition (including revisions). Geneva: World Health Organization, 2001, 2011, 2020.
Section: ICD-O-3.2 (2020) Morphological Codes
Pages: http://www.iacr.com.fr/index.php?option=com_content&view=category&layout=blog&id=100&Itemid=577

PDQ® Adult Treatment Editorial Board. PDQ Aggressive B-Cell Non-Hodgkin Lymphoma Treatment. Bethesda, MD: National Cancer Institute. Updated <02/24/2025>. Available at: https://www.cancer.gov/types/lymphoma/hp/aggressive-b-cell-lymphoma-treatment-pdq. Accessed <03/19/2025>.
Section: Aggressive B-Cell Non-Hodgkin Lymphoma Treatment (PDQ®)–Health Professional Version
Pages: https://www.cancer.gov/types/lymphoma/hp/aggressive-b-cell-lymphoma-treatment-pdq
Glossary