This neoplasm is not reportable.

Name

Lymphomatoid granulomatosis

ICD-O-2 Morphology

9766/1: Angiocentric immunoproliferative lesion
Effective 1992 - 2000

ICD-O-3 Morphology

9766/1: Angiocentric immunoproliferative lesion
Effective 2001 and later

Not Reportable

This neoplasm is not reportable

Primary Site(s)

See Abstractor Notes

Alternate Names

Angiocentric immunoproliferative lesion [OBS]
LYG
Lymphoid granulomatosis grade 1
Lymphoid granulomatosis grade 2
Lymphomatoid granulomatosis grade 1 or 2

Definition

Lymphoid granulomatosis (LyG) is an angiocentric and angiodestructive lymphoproliferative disease involving extranodal sites, composed of EBV-positive B cells admixed with reactive T cells, which usually predominate. The lesion has a spectrum of histological grade and clinical aggressiveness, which is related to the proportion of large B cells.

LyG is divided into three different grades

1) Grade 1 lesions contain a polymorphous lymphoid infiltrate without cytological atypia. Large transformed lymphoid cells are absent or rare, and are better appreciated by immunohistochemistry. When present, necrosis is usually focal. In some cases, EBV-positive cells may be absent, in this setting, the diagnosis should be made with caution, with studies to rule out other inflammatory or neoplastic conditions.

2) Grade 2 lesions contain occasional large lymphoid cells or immunoblasts in a polymorphous background. Small clusters can be seen, in particular with CD20 staining. Necrosis is more commonly seen.

3) Grade 3-see 9680/3.

Abstractor Notes

If angiocentric immunoproliferative lymphoma, code 9719/3. Angiocentric immunoproliferative lesion is coded 9766/1.

The clinical behavior of LyG varies widely; the disease ranges from an indolent process to an aggressive large B-cell lymphoma. In its most indolent form, LyG presents with pulmonary nodules in an otherwise asymptomatic patient.

This disease can transform to EBV+ diffuse large B-cell (alternate name for 9680/3). If disease does transform to 9680/3 this would be one primary since 9766/1 is not reportable.

Genetics Data

This data item does not apply

Immunophenotyping

This data item does not apply

Transformations to

This data item does not apply

Corresponding ICD-9 Codes

238.79 Other lymphatic and hematopoietic tissues

Corresponding ICD-10 Codes

D47.7 Other specified neoplasms of uncertain or unknown behavior of lymphoid, hematopoietic, and related tissue

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

D47.Z9 Other specified neoplasms of uncertain behavior of lymphoid, hematopoietic and related tissue (effective October 01, 2015)

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: 312-314

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