Name

Nodular sclerosis classic Hodgkin lymphoma

ICD-O-1 Morphology

9656/3: Hodgkin disease, nodular sclerosis, NOS
Effective 1978 - 1991

ICD-O-2 Morphology

9663/3: Hodgkin lymphoma, nodular sclerosis, NOS
Effective 1992 - 2000

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 (C770-C779) are the usual primary sites; however, involvement in other sites is possible. If you have confirmation that the only involved site is something other than the lymph nodes, then code to that primary site. See Module 7.

Grade

Not Applicable

Module Rule

None

Alternate Names

Classical Hodgkin lymphoma, nodular sclerosis, cellular phase
Classical Hodgkin lymphoma, nodular sclerosis, grade 1
Classical Hodgkin lymphoma, nodular sclerosis, grade 2
Classical Hodgkin lymphoma, nodular sclerosis, NOS
Classical Hodgkin lymphoma, nodular sclerosis, syncytial variant
Hodgkin disease, nodular sclerosis, lymphocyte predominance
Hodgkin disease, nodular sclerosis, lymphocyte depletion
Hodgkin disease, nodular sclerosis, mixed cellularity
Hodgkin disease, nodular sclerosis, NOS
Hodgkin lymphoma, nodular sclerosis, grade 1
Hodgkin lymphoma, nodular sclerosis, grade 2
NS-HL
NSCHL

Definition

Nodular sclerosis classic Hodgkin lymphoma (NSCHL) is a subtype of classic Hodgkin lymphoma (CHL) characterized by collagen bands that surround at least one nodule, and by HRS cells with lacunar-type morphology.

Abstractor Notes

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.

Definitive Diagnostic Methods

Histologic confirmation
Immunophenotyping

Genetics Data

None

Immunophenotyping

CD2 expressed
CD4 expressed
CD15 negative
CD20 variably expressed
CD30 expression
CD45-
CD79a positive
EBV-encoded small RNA (EBER)

Treatments

Chemotherapy
Hormone therapy
Radiation therapy

Transformations to

None

Corresponding ICD-9 Codes

201.5 Hodgkin's disease, nodular sclerosis

Corresponding ICD-10 Codes

C81.1 Hodgkin's disease, nodular sclerosis

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

C81.1 Nodular sclerosis classical Hodgkin lymphoma (effective October 01, 2015)

Signs and Symptoms

Drenching night sweats
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

CT (CAT) scan
MRI (magnetic resonance imaging)
PET (positron emission tomography) scan
Physican exam and history (H&P)

Progression and Transformation

None

Epidemiology and Mortality

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

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: Hodgkin lymphomas
Pages: 435-437

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 Hodgkin Lymphoma (HL)
Pages: https://www.cancer.gov/types/lymphoma/hp/adult-hodgkin-treatment-pdq
Glossary