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This neoplasm is not reportable.
Name
Lymphomatoid granulomatosis, NOS (LYG)
ICD-O-3 Morphology
9766/1: Angiocentric immunoproliferative lesion
Effective
2001 and later
Reportability
This neoplasm is not reportable
Primary Site(s)
No primary site specified
Lung primary sites only (C340-C349)
This hematopoietic disease is not reportable for the U.S.
Canada, refer to your specific province for reportability requirements.
This hematopoietic disease is not reportable for the U.S.
Canada, refer to your specific province for reportability requirements.
Abstractor Notes
Lymphomatoid granulomatosis, NOS (LYG) 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.
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.
Alternate Names
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 edition)
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-10 Codes (Cause of Death codes only)
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
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: 489-492
Section: Large B-cell lymphomas
Pages: Part B: 489-492
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
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
