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.
Coding Manual:
Hematopoietic Coding Manual (PDF)
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.
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.
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
Hodgkin disease, nodular sclerosis, lymphocyte predominance
Hodgkin disease, nodular sclerosis, lymphocyte depletion
Hodgkin disease, nodular sclerosis, mixed cellularity
Hodgkin disease, nodular sclerosis, NOS
Nodular sclerosis Hodgkin lymphoma
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.
Definitive Diagnostic Methods
Histologic confirmation
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
Transformations from
Same Primaries
Corresponding ICD-9 Codes
201.5 Hodgkin's disease, nodular sclerosis
Corresponding ICD-10 Codes
C81.1 Hodgkin nodular sclerosis
Corresponding ICD-10-CM Codes (U.S. only)
C81.1 Nodular sclerosis Hodgkin lymphoma (effective October 01, 2015)
Signs and Symptoms
Drenching night sweats
Fatigue
Fever (for no known reason)
Mediastinal mass (bulky disease)
Painless swelling in the lymph nodes
Skin rash or itchy skin
Weight loss (for no known reason)
Diagnostic Exams
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
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, 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 Hodgkin Lymphoma (HL)
Pages: https://www.cancer.gov/types/lymphoma/hp/adult-hodgkin-treatment-pdq
Section: General Information About Adult Hodgkin Lymphoma (HL)
Pages: https://www.cancer.gov/types/lymphoma/hp/adult-hodgkin-treatment-pdq