Search Database ICD-O-3 Code Lists

Name

Lymphomatoid granulomatosis (LyG) grade 3 (see 9680/3 prior to 2021)

ICD-O-3 Morphology

9766/3: Lymphomatoid granulomatosis grade 3
Effective 2001 and later

Reportable

for cases diagnosed 1978 and later

Primary Site(s)

See Module 7
This disease always involves the lung (primary site). Other involvement is usually metastatic disease.

Coding Manual: Hematopoietic Coding Manual (PDF)

Abstractor Notes

(This code is effective for cases diagnosed 2021 and later. For cases diagnosed prior to 2021 see code: 9680/3.)

Lymphomatoid granulomatosis (LyG) grade 3 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)

Lymphomatoid granulomatosis is divided into three different grades, and NOS

1. Lymphomatoid granulomatosis, NOS (9766/1)
2. Lymphomatoid granulomatosis, grade 1 (9766/1/)
3. Lymphomatoid granulomatosis, grade 2 (9766/1)
4. Lymphomatoid granulomatosis, grade 3 (9766/3)

Lymphomatoid granulomatosis always involves the lung, and frequently involves the CNS, skin, kidneys, and liver, but it can occur in essentially any organ. Involvement of lymph nodes and/or bone marrow is rare.

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

None

Definition

Lymphomatoid granulomatosis (LYG) is an EBV-associated angiocentric and angiodestructive B-cell lymphoproliferative disorder involving extranodal sites, composed of EBV-positive atypical large B cells usually admixed with a large number of reactive T cells. It occurs in patients lacking evidence of inborn or acquired immune deficiency/dysregulation other than immunosenescence. (WHO 5th ed)

Definitive Diagnostic Methods

Cytogenetics
Genetic testing
Histologic confirmation
Immunohistochemistry
Immunophenotyping

Genetics Data

None

Immunophenotyping

CD15- (no expression/negative)
CD30+ (expression/positive)
EBNA2+ (expression/positive)
LMP1+ (expression/positive)

Treatments

Chemotherapy
Immunotherapy

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 node
Shortness of breath

Diagnostic Exams

Bone marrow or aspiration and biopsy
CT (CAT) scan
Flow cytometry
Immunohistochemistry
Immunophenotyping
Lymph node biopsy
PET (positron emission tomography) scan

Progression and Transformation

None

Epidemiology and Mortality

Age: Median age between fourth and sixth decades of life
Incidence: Rare
Sex: M:F ratio is 2:1
Survival: Median overall survival of 14 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
Pages: Part B:

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 <03/20/2025>.
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