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Name
Lymphomatoid granulomatosis grade 3
ICD-O-3 Morphology
9766/3: Lymphomatoid granulomatosis grade 3
Effective
2021 and later
Reportable
for cases diagnosed
2021 and later
Primary Site(s)
No primary site specified
See Module 7
Most common sites of involvement: lung, upper respiratory tract, brain, kidney, liver, skin, gastrointestinal.
Rare involvement of the lymph nodes and spleen
Most common sites of involvement: lung, upper respiratory tract, brain, kidney, liver, skin, gastrointestinal.
Rare involvement of the lymph nodes and spleen
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.)
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 (see 9766/1), LyG presents with pulmonary nodules in an otherwise asymptomatic patient.
A more typical course is characterized by symptoms and multiorgan involvement.
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 (see 9766/1), LyG presents with pulmonary nodules in an otherwise asymptomatic patient.
A more typical course is characterized by symptoms and multiorgan involvement.
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.
Grade
Not Applicable
Module Rule
None
Alternate Names
None
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.
LyP is divided into three different grades
1) Grade 1: see 9766/1
2) Grade 2: see 9766/1
3) Grade 3 lesions show an inflammatory background, but also contain large atypical B cells that are readily identified by CD20 and can form larger aggregates. Markedly pleomorphic and Hodgkin-like cells are often present, and necrosis is usually extensive. EBV-positive cells are numerous, and focally may form small confluent sheets.
LyP is divided into three different grades
1) Grade 1: see 9766/1
2) Grade 2: see 9766/1
3) Grade 3 lesions show an inflammatory background, but also contain large atypical B cells that are readily identified by CD20 and can form larger aggregates. Markedly pleomorphic and Hodgkin-like cells are often present, and necrosis is usually extensive. EBV-positive cells are numerous, and focally may form small confluent sheets.
Definitive Diagnostic Methods
Bone marrow biopsy
Genetic testing
Immunophenotyping
Genetics Data
None
Immunophenotyping
CD3+ (expression/positive)
CD4+ (expression/positive)
CD8+ (expression/positive)
CD15- (no expression/negative)
CD20+ (expression/positive)
CD30+ (expression/positive)
EBNA2+ (expression/positive)
LMP1+ (expression/positive)
Treatments
Chemotherapy
Immunotherapy
Transformations to
Transformations from
None
Same Primaries
Corresponding ICD-9 Codes
200.8 Other named variants of lymphosarcoma and reticulosarcoma
Corresponding ICD-10 Codes
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)
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: Usually presents in adults, but may be seen in children
Incidence: Rare condition, female predominance
Survival: Up to 5 year survival rate of 70%
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
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, Second Revision. Geneva: World Health Organization, 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
National Cancer Institute
Section: General Information About Adult Non-Hodgkin Lymphoma (NHL)
Pages: https://www.cancer.gov/types/lymphoma/hp/adult-nhl-treatment-pdq
Section: General Information About Adult Non-Hodgkin Lymphoma (NHL)
Pages: https://www.cancer.gov/types/lymphoma/hp/adult-nhl-treatment-pdq
