Name

Classic Hodgkin lymphoma, nodular sclerosis, NOS (NSCHL)

ICD-O-3 Morphology

9663/3: Hodgkin lymphoma, nodular sclerosis, NOS
Effective 2001 and later

Reportable

for cases diagnosed 1978 and later

Primary Site(s)

C770-C779
Lymph nodes are the most common primary sites.
Common metastatic sites include the bone, CNS, liver, lung, and bone marrow.
Spleen involvement is common due to dissemination of disease

Assign C779 if specific primary site cannot be determined.

See abstractor notes

Abstractor Notes

Classic Hodgkin lymphoma, nodular sclerosis, NOS (NSCHL) is part of the Hodgkin lymphoma neoplasms lineage table in the WHO 5th edition of Hematolymphoid Tumors. (See Appendix B in the Hematopoietic Manual, Table B16)

Nodular sclerosis classical Hodgkin lymphoma (NSCHL) occurs in the mediastinum in 90% of the cases, bulky involvement in 54%, splenic and/or lung involvement in 8-10%, bone involvement in 5%, bone marrow involvement in 3%, and liver involvement in 2%.

Most patients present with stage II involvement. B symptoms are encountered in approximately 40% of cases. Massive mediastinal involvement is an adverse prognostic factor.

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

Classical Hodgkin lymphoma, nodular sclerosis, cellular phase (see 9664/3 prior to 2010)
Classical Hodgkin lymphoma, nodular sclerosis, grade 1 (see 9665/3 prior to 2010)
Classical Hodgkin lymphoma, nodular sclerosis grade 2 (see 9667/3 prior to 2010)
Hodgkin disease, nodular sclerosis, lymphocyte depletion (see 9667/3 prior to 2010)
Hodgkin disease, nodular sclerosis, lymphocyte predominance (see 9665/3 prior to 2010)
Hodgkin disease, nodular sclerosis, mixed cellularity (see 9665/3 prior to 2010)
Hodgkin disease, nodular sclerosis, syncytial variant (see 9667/3 prior to 2010+
Hodgkin lymphoma, nodular sclerosis, NOS
Hodgkin lymphoma, nodular sclerosis, grade 1 (see 9665/3 prior to 2010)
Hodgkin lymphoma, nodular sclerosis, grade 2 (see 9667/3 prior to 2010)

Definition

Classic Hodgkin lymphoma (CHL) is a neoplasm derived from germinal-centre B cells, characterized by a low fraction of tumour cells embedded in a reactive microenvironment rich in immune cells. The large neoplastic Hodgkin and Reed–Sternberg cells show a defective B-cell expression programme. (WHO 5th edition).

Definitive Diagnostic Methods

Histologic confirmation
Immunohistochemistry
Immunophenotyping

Genetics Data

None

Immunophenotyping

CD2+ (expression/positive)
CD4+ (expression/positive)
CD15-(no expression/negative)
CD20+ (expression/positive)
CD30+ (expression/positive)
CD45- (no expression/negative)
CD79a+ (expression/positive)
EBV-encoded small RNA (EBER)

Treatments

Chemotherapy
Hormone therapy
Radiation therapy

Transformations to

None

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

C81.1 Hodgkin nodular sclerosis

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

C81.1_ Nodular sclerosis Hodgkin lymphoma (effective October 01, 2015)
C81.1A Nodular sclerosis Hodgkin lymphoma, in remission (effective October 01, 2024)

Signs and Symptoms

Drenching night sweats
Fatigue
Fever (for no known reason)
Mediastinal mass (bulky disease)
Pain in the chest, abdomen, 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
Erythrocyte sedimentation rate
Immunophenotyping
Lymph node biopsy
PET (positron emission tomography) scan
Physican exam and history (H&P)

Progression and Transformation

None

Epidemiology and Mortality

Incidence: 70% of classical Hodgkin lymphomas, most common subtype of Hodgkin lymphoma in developed countries
Age: peaks at 15-34 years
Sex: no male or female predilection
Survival: better survival than other types of CHL

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: Hodgkin lymphomas
Pages: Part B: 580-590

International Classification of Diseases for Oncology, Third Edition, Second Revision. 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 Hodgkin Lymphoma Treatment. Bethesda, MD: National Cancer Institute. Updated <02/12/2025>. Available at: https://www.cancer.gov/types/lymphoma/hp/adult-hodgkin-treatment-pdq. Accessed <03/30/2025>. [PMID: 26389473]
Section: Hodgkin Lymphoma Treatment (PDQ®)–Health Professional Version
Pages: https://www.cancer.gov/types/lymphoma/hp/adult-hodgkin-treatment-pdq
Glossary