Introducing ICD-0

Introducing ICD-O-3
The International Classification of Diseases for Oncology,
Third Edition
April Fritz, RHIT, CTR
SEER Program, National Cancer Institute

This presentation covers
• Background for developing ICD-O-3
• What’s new in ICD-O-3
• What’s not new
• Coding guidelines and issues
• Newly reportable hematopoietic diseases

ICD-O-3 Editorial Committee

April Fritz and Constance Percy
National Cancer Institute, USA
Dr Leslie Sobin
Armed Forces Institute of Pathology, USA
Dr K. Shanmugaratnam
National University of Singapore
Dr Andrew Jack
Leeds Teaching Hospitals, UK
Dr Max Parkin and Sharon Whelan
International Agency for Research on Cancer, France

Development of ICD-O-3
• Evolution of cytogenetic and molecular markers
• Identification of morphologic subtypes
• Changes in terminology
• Publication of REAL Classification, 1997
• Publication of WHO/ISUP consensus paper, 1998
• Working party convened, 1998
• Submission of new terms
• Field testing, 1999
• Implementation

What’s New?
• ~550 new morphology terms and synonyms added 8000-9582
• ~200 new morphology terms added to lymphomas and leukemias

• NO changes to topography

Equivalent Terms
• Mixed/combined small cell ca = large cell-small cell carcinoma
• Sarcomatoid = spindle cell
• Urothelial = transitional cell
• Primary cutaneous neuroendocrine carcinoma = Merkel cell
• Malignant peripheral nerve sheath tumor (MPNST) = neurofibrosarcoma

What’s New?
• Terms from International Histological Typing of Tumours (WHO Blue Books) included
• Major overhaul of lymphomas and leukemias
• Some existing codes moved (combined with other codes)

What’s New?
• More acronyms
» MALT, SALT, BALT mucosa-, skin-, bronchus-associated lymphoid tissue
» PIN, AIN prostatic, anal intraepithelial neoplasia
» DCIS duct carcinoma in situ
» PNET, CPNET, PPNET primitive, central primitive, peripheral primitive
neuroectodermal tumor
» MPNST malignant peripheral nerve sheath tumor

What’s New?
• More specific designation of NOS terms as /0, /1 or /3
• “Crossover” terminology
» Borderline terms that sound malignant
» Malignant terms that don’t sound malignant

Reportable Terms
• Atypical carcinoid
• SETTLE, CASTLE Spindle epithelial tumor showing thymus-like element,
Carcinoma showing thymus-like element
• Rhabdoid tumor, NOS
• Intratubular germ cell neoplasia
• Atypical teratoid/rhabdoid tumor
• Pagetoid reticulosis
• Agnogenic myeloid metaplasia
• Heavy chain disease
• Therapy related myelodysplastic syndrome

What’s New?
• Codes for non-specific diagnoses
» 8046/3 Non-small cell carcinoma
» 8935/3 Stromal sarcoma, NOS
» 9473/3 PNET, NOS
» 9591/3 B-cell lymphoma, NOS
» 9702/3 T-cell lymphoma, NOS
» 9801/3 Stem cell leukemia
» 9860/3 Non-lymphocytic leukemia, NOS

What’s NOT New?
• Matrix principle
• No rule changes
• Older terms retained

Behavior Code Changes
Malignant /3 to Borderline /1
• Borderline tumors of ovary
» 8442/1 serous cystadenoma
» 8451/1 papillary cystadenoma
» 8462/1 serous papillary cystadenoma
» 8472/1 mucinous cystadenoma
» 8473/1 papillary mucinous cystadenoma

Behavior Code Changes
Malignant /3 to Borderline /1
• Pilocytic/juvenile astrocytoma
» Listed as 9421/1 in ICD-O-3
» Will remain reportable as 9421/3
» Sequenced as malignant

Behavior Code Changes
Borderline /1 to Malignant /3
• 8931/3 Endolymphatic stromal myosis (C54.1)
– now a synonym for endometrial stromal sarcoma, low grade

• 9538/3 Papillary meningioma
– malignant version of clear cell/chordoid meningioma

Behavior Code Changes
Borderline /1 to Malignant /3
Myeloproliferative Disorders
• 9950/3 Polycythemia vera
• 9960/3 Chronic myeloproliferative disease
• 9961/3 Myelosclerosis with myeloid metaplasia
• 9962/3 Essential thrombocythemia

Behavior Code Changes
Borderline /1 to Malignant /3
Myelodysplastic Syndromes
• 9980/3 Refractory anemia
• 9982/3 Refractory anemia with sideroblasts
• 9983/3 Refractory anemia with excess blasts
• 9984/3 Refractory anemia with excess blasts in transformation

Additional ICD-9 Casefinding Codes(1)
238 Neoplasm of uncertain behavior of other and unspecified sites and tissues
.4 Polycythemia vera
.5 Histiocytic and mast cells
.6 Plasma cells (plasmacytoma, NOS, solitary myeloma)
.7 Other lymphatic and hematopoietic tissues…

Additional ICD-9 Casefinding Codes(2)
238.7 Other lymphatic and hematopoietic tissues, continued
Chronic lymphoproliferative disease
Chronic myeloproliferative disease
Idiopathic thrombocythemia
Megakaryocytic myelosclerosis
Myelodysplastic syndrome (NOS and therapy related)
Myelosclerosis with myeloid metaplasia
Acute panmyelosis
[Refractory cytopenia with multilineage dysplasia]
[Myelodysplastic syndrome with 5q- syndrome]

Additional ICD-9 Casefinding Codes(3)
273 Disorders of plasma protein metabolism
.2 Franklin’s disease, gamma heavy chain disease
.3 Macroglobulinemia (Waldenstrom’s macroglobulinemia, primary (idiopathic)
.9 Unspecified disorder of plasma protein metabolism
(screen for potential 273.3 miscodes)

Additional ICD-9 Casefinding Codes(4)
284.9 Aplastic anemia
Aplastic anemia, unspecified
Refractory anemia
Medullary hypoplasia
Aregenerative anemia
Hypoplastic anemia, NOS

285.0 Sideroblastic anemia
Refractory sideroblastic anemia
[Refractory anemia with sideroblasts]
[Refractory anemia with excess blasts]
[Refractory anemia with excess blasts in transformation]

Additional ICD-9 Casefinding Codes(5)
288.3 Eosinophilia
Idiopathic eosinophilia
[Hypereosinophilic syndrome]

289.8 Other specified diseases of blood and blood-forming organs
Pseudocholinesterase deficiency
Acute myelofibrosis

What’s New?
Genetic Terminology

p short arm
q long arm

– deletion
Chromosome t translocation (swap)

Cytogenetic and Molecular Descriptors
Acute promyelocytic leukemia t(15;17)(q22;q11-12)
Acute promyelocytic leukemia, PML/RAR-alpha
Acute promyelocytic leukemia, NOS

Cytogenetic and Molecular Descriptors
Chronic myelogenous leukemia, BCR/ABL positive
Chronic granulocytic leukemia, t(9;22)(q34;q11)
Chronic granulocytic leukemia, Philadelphia chromosome (Ph1) positive

Leukemia Oncogenes Appearing in ICD-O-3
ABL Abelson murine leukemia oncogene
BCR breakpoint cluster region
CBF core binding factor
ETO eight twenty one (8;21)
MYH11 myosin, heavy polypeptide 11
PML promyelocytic leukemia
RARA retinoic acid receptor, alpha

What’s New?
9728/3 Precursor B-cell lymphoblastic lymphoma (see also 9836/3)
9836/3 Precursor B-cell lymphoblastic leukemia (see also 9728/3)

9671/3 Malignant lymphoma, lymphoplasmacytic (see also 9761/3)
9761/3 Waldenstrom macroglobulinemia (C42.0) (see also 9671/3)

“B-cell chronic lymphocytic leukemia/
small lymphocytic lymphoma”
• WHO formal name for disease entity
» had to be split in ICD-10
• 9823/3 cross-referenced to 9670/3
» Malignant lymphoma, small B lymphocytic
• If diagnosed in blood, code 9823/3
• If diagnosed in tissue, code 9670/3

What’s New?
• Instructions rewritten
» More user friendly
» Active verbs
» Simpler statements
» NO change in meaning
» More visual

What’s New?
Table 8. Structure of a Morphology Code

___ ___ ___ ___ / ___ ___
histology behavior differentiation

Example: well-differentiated adenocarcinoma

8 1 4 0 / 3 1

Tumor/cell type Behavior Differentiation
[adeno-] [carcinoma] [well-differentiated]

ICD-O Coding Rules
• Clarified and simplified
• Reorganized
» topography first, then morphology
• Assigned alphabetic labels

Topographic regions and ill-defined sites
If the diagnosis does not specify the tissue of origin, use the appropriate tissues suggested in the alphabetic index for each ill-defined site in preference to the “NOS” category.

If a topographic site is modified by a prefix (such as peri-, para-, or the like) which
is not specifically listed in ICD-O, code to the appropriate ill-defined subcategory C76
(ill-defined site), unless the type of tumor indicates origin from a particular tissue.

Tumors involving more than one topographic category
or subcategory
Use subcategory “.8” when a tumor overlaps the boundaries of two or more categories
or subcategories and its point of origin cannot be determined.

Topography codes for lymphomas
If multiple lymph node regions are involved by lymphoma, code to C77.8 (lymph nodes of multiple regions).

If no site is indicated for a [nodal] lymphoma, code to C77.9 (lymph node, NOS).

Code extranodal lymphomas to the site of origin, which may not be the site of the biopsy.

If no site is indicated for a lymphoma and it is suspected to be extranodal, code to C80.9 (unknown primary site).

Topography code for leukemias
Code all leukemias except myeloid sarcoma (M-9903/3) to C42.1, bone marrow.

Behavior code in morphology
(The “Matrix Principle”)
Use the appropriate 5th digit behavior code even if the exact term is not listed in ICD-O.

Grading or differentiation code
Assign the highest grade or differentiation code described in the diagnostic statement.

Site-associated morphology terms
Use the topography code provided when a topographic site is not stated in the diagnosis.

This topography code should be disregarded if the tumor is known to arise at another site.

Compound morphology diagnoses
Change the order of word roots in a compound term if the term is not listed in ICD-O-3.

Coding multiple morphology terms
When no single code includes all diagnostic terms, use the numerically higher code number if the diagnosis of a single tumor includes two modifying adjectives with different code numbers.

Other Coding Issues
• Coding of subtypes
» “ductal” (breast)
» “renal cell” (kidney)
» “urothelial” (bladder)
• Definitions of certain terms
• Use of obsolete or archaic terms

Morphology Coding Rules
1. Determine whether single primary or multiple primaries.
» Same first three digits (single)
» Different first three digits (multiple)
° Except lymphatic and hematopoietic diseases

Single Morphology
• If diagnosis is “generic cancer of something type,” code the type.
Example: Duct carcinoma, cribriform type
Code to 8201/3 Cribriform carcinoma

Morphology Coding Rules
2. Coding mixed or multiple morphologies in a single lesion
These rules are in priority order:
a. Use a combination code
b. Code the more specific term
c. Code the majority of the tumor
d. Code the morphology with the highest code

A. Combination Codes
• 8522/_ Ductal and lobular
• 8523/_ Infiltrating duct mixed with other types
• 8524/_ Infiltrating lobular mixed with other types

B. Code the More Specific Morphology
• Non-specific morphologies:
»carcinoma, adenocarcinoma, melanoma, sarcoma
Example: Poorly differentiated carcinoma, probably squamous in origin
Code to 8070/3 Squamous cell carcinoma

C. Majority of Tumor
Majority words
“with features of”

NOT majority words
“with foci of”
“with areas of”
“with elements of”

D. Code the Higher Number
• Used infrequently—rule with lowest priority
Example: Pleural tumor containing malignant mesothelioma and neuroendocrine
Code to 9050/3 Malignant mesothelioma

Format of Index
• NOS term listed first in index
• Most terms indexed under each word
• Lymphomas listed only once
• Leukemias listed only once
• Single instance of a word not bolded
• If 3 or more instances of a word, a bold header is added
• Empty line when list changes from morphology to topography

Structure of ICD-O-3 Index
M-8720/3 NOS
M-8771/3 epithelioid cell
M-8745/3 desmoplastic
M-8723/3 regressing

M———- Melanosis, congenital (see SNOMED)

C70.9 NOS
C70.0 cerebral
C70.1 spinal

M-8247/3 Merkel cell carcinoma (C44._)
M-8247/3 Merkel cell tumor (C44._)

C49.4 Mesenteric artery

Meaning of NOS
• Not Otherwise Specified
• Not Elsewhere Classified
• Term used in a general sense

Terms Requiring Review
8580/0 Thymoma, NOS (to 8580/1)
8580/3 Thymic carcinoma (to 8586/3)
8930/3 Stromal sarcoma (to 8935/3)
9190/3 Juxtacortical osteosarcoma (to 9192/3)
9190/3 Periosteal osteogenic sarcoma (to 9193/3)
9731/3 Extramedullary plasmacytoma (to 9734/3)
9960/1 Myeloproliferative disease [obs] (to 9975/1)

Ancillary ICD-O-3 Materials Available from SEER
• Conversion tables and programs
» ICD-O-2 to ICD-O-3
» ICD-O-3 to ICD-O-2
» ICD-O-3 to ICD-10
• Hematopoietic primaries table
• Site-type table
• Edits

ICD-O-3 Web Sites
ICD-O-3 Training Web Site

ICD-O-3 Download Site

Implementation Work Group
• Joint effort of Uniform Data Standards, Information and Technology, Registry Operations and Education committees of NAACCR
• Joint effort of SEER, NPCR, COC, state central registries, vendors
• Guidelines mailed and e-mailed
• Download from

Ordering Information
Single hardcover copy price: $54.00.
• Quantity discounts available

Order from:
WHO Publications Center USA
49 Sheridan Avenue
Albany, NY 12210
Tel: (518) 436 9686
Fax: (518) 436 74 33

Understanding the Newly Reportable
Hematopoietic Diseases

From Stem Cell to Mature Blood Cell


WHO Classification of Hematopoietic and Lymphoid Neoplasms
Lymphoid neoplasms
B-cell, T-cell, NK-cell lymphomas
Hodgkin lymphoma
Lymphocytic leukemias
Other lymphoid malignancies

Myeloid Neoplasms
Myeloproliferative diseases
Myelodysplastic diseases and syndromes
Myeloid leukemias
Acute biphenotypic leukemias

The Lymphomas
• Non-Hodgkin Lymphoma
» WHO Classification–T vs B cell
» De-emphasizing Rappaport, Kiel, Lukes and Collins, Working Formulation

• Hodgkin Lymphoma
» formerly Hodgkin’s Disease
» “classic Hodgkin Lymphoma”– 4 types
» “Hodgkin Lymphoma, nodular lymphocyte predominance”
Equivalent Terms
• Follicular lymphoma = follicle center cell lymphoma
• Mantle cell lymphoma = mantle zone lymphoma
• Anaplastic large B-cell lymphoma = diffuse large cell lymphoma
• Mature T-cell lymphoma, NOS = peripheral T-cell lymphoma

Leukemia Cell Lines



Leukemia Terminology
-blast early form
-cyte mature form
phenotype cell line

Equivalent Terms
Myeloid Lymphoid
Non-lymphocytic Lymphoblastic
Granulocytic Lymphocytic
Myelogenous Lymphatic

Other Lymphoid Malignancies
• Solitary plasmacytoma 9731/3
• Multiple myeloma 9732/3
• Plasma cell leukemia 9733/3
• Extramedullary plasmacytoma 9734/3
• Waldenstrom’s macroglobulinemia 9761/3
• Monoclonal gammopathy of
unknown significance 9765/1

Histiocytic and Dendritic Cells
(Accessory Lymphoid Cells)
• Malignant histiocytosis 9750/3
• Langerhans cell histiocytosis, generalized 9752/3
• Histiocytic sarcoma 9755/3
• Langerhans cell sarcoma 9754/3
• Interdigitating dendritic cell sarcoma 9757/3
• Follicular dendritic cell sarcoma 9758/3

Chronic Myeloproliferative Diseases and Disorders
Polycythemia vera 9950/3
Chronic myeloproliferative disease 9960/3
Myelosclerosis with myeloid metaplasia 9961/3
Essential thrombocythemia 9962/3
Chronic neutrophilic leukemia 9963/3
Hypereosinophilic syndrome 9964/3

Definition Overproduction of one or more cell lines developing in bone marrow

Disease Cell Line
Polycythemia vera Erythrocytes
Thrombocythemia Platelets
CMD Multiple cell lines

Polycythemia Vera
Affects Erythrocytes
ICD-9 code 238.4
ICD-O-3 code M-9950/3
Treated by Blood removal, P32 or chemo
Other names Polycythemia rubra vera, p. vera, chronic erythremia (obs),
proliferative polycythemia, primary polycythemia, splenomegalic polycythemia, Vaquez-Osler disease

Essential Thrombocythemia
Affects Platelets/thrombocytes
ICD-9 code 238.7
ICD-O-3 code M-9962/3
Treated by Aspirin, chemo
Other names Idiopathic thrombocythemia, essential hemorrhagic
thrombocythemia, idiopathic hemorrhagic thrombocythemia,
primary thrombocythemia
Chronic Myeloproliferative Disorder
Affects Multiple cell lines
ICD-9 code 238.7
ICD-O-3 code M-9960/3
Treated by Observation; chemo, RT to relieve symptoms
Other names Chronic myeloproliferative disease

Myelosclerosis with Myeloid Metaplasia
Affects Bone marrow stem cells
ICD-9 code 238.7
ICD-O-3 code M-9961/3
Treated by Observation; RT to spleen; transfusions; chemo; splenectomy
Other names Myelofibrosis with myeloid metaplasia, primary myelofibrosis (PMF),
myelofibrosis as a result of myeloproliferative disease,
megakaryocytic myelosclerosis, agnogenic myeloid metaplasia

Definition Defective development of blood cell precursors in the bone marrow
resulting in qualitative and quantitative changes in blood cells.

Other names Pre-leukemia, preleukemic disorders, smoldering leukemia,
oligoblastic leukemia, subacute myeloid leukemia, de novo myelodysplastic syndrome, dysmyelopoietic syndrome, hemopoietic dysplasia

Myelodysplastic Syndromes
Refractory anemia 9980/3
Refractory anemia with sideroblasts 9982/3
Refractory anemia with excess blasts 9983/3
Refractory anemia with excess blasts
in transformation [obs] 9984/3

Refractory Anemia
Affects Reticulocytes
ICD-9 code 284.9
ICD-O-3 code M-9980/3
Treated by Supportive measures
Other names RA, refractory anemia without sideroblasts

Refractory Anemia with Sideroblasts
Affects Reticulocytes
ICD-9 code 285.0
ICD-O-3 code M-9982/3
Treated by Supportive measures
Other names Refractory anemia with ringed sideroblasts, RARS

Refractory Anemia with Excess Blasts
Affects Myeloblasts
ICD-9 code 285.0
ICD-O-3 code M-9983/3
Treated by Transfusions; chemo
Other names RAEB

Refractory Anemia with Excess Blasts in Transformation
Affects Myeloblasts
ICD-9 code 285.0
ICD-O-3 code M-9984/3
Treated by Transfusions; chemo
Other names RAEB-T

New Myelodysplastic Syndromes
Refractory cytopenia with multilineage dysplasia 9985/3
Myelodysplastic syndrome with 5q- (5q deletion) syndrome 9986/3
Therapy related myelodysplastic syndromes 9987/3

Refractory Cytopenia with Multilineage Dysplasia
Affects Multiple cell lines
ICD-9 code 238.7
ICD-O-3 code M-9985/3
Treated by Transfusions

Myelodysplastic Syndrome with 5q- (5q deletion) Syndrome
Affects Myeloid cells
ICD-9 code 238.7
ICD-O-3 code M-9986/3
Treated by Transfusions; chemo

Therapy Related Myelodysplastic Syndrome
Affects Myeloid cells
ICD-9 code 238.7
ICD-O-3 code M-9987/3
Results from treatment with alkylating agents or
Other names Secondary myelodysplasia, secondary MDS

Miscellaneous Reportable Terms
Heavy chain disease 9762/3
Immunoproliferative small intestinal disease 9764/3
Myeloid sarcoma 9930/3
Hairy cell leukemia 9940/3
Juvenile myelomonocytic leukemia 9946/3

Record as Treatment
• Chemotherapy
• Radiation therapy
• Surgery
• Immunotherapy
• Bone marrow transplants
• Clinical trials

DO NOT Record as Treatment
• Transfusions
• Phlebotomy/blood removal
• Supportive care
• Aspirin
• Observation

• Do include in remarks field

Registering the Newly Reportable Hematopoietic Diseases (NRHD)
• Accession and abstract only NEW NRHD diagnoses.
• Disregard NRHD cases diagnosed prior to 01/01/01 undergoing active treatment.
• Monitor for transition to another hematopoietic disease.

Abstracting NRHD
Example 1
Refractory anemia with excess blasts diagnosed 6/25/99.
Still receiving transfusions 2/24/01.

Do not abstract.

Abstracting NRHD
Example 2
Refractory anemia with excess blasts diagnosed 6/25/99.
Bone marrow biopsy 3/20/01 shows acute myelogenous leukemia.

Abstract as acute myelogenous leukemia (sequence 00).
Note prior diagnosis in remarks.

Abstracting NRHD
Example 3
Polycythemia vera diagnosed 1/21/01 following 1997 colon cancer.

Abstract polycythemia as a new cancer (sequence 02).

Abstracting NRHD
Example 4
Essential thrombocythemia diagnosed 1/21/01.
Acute myelofibrosis diagnosed 5/19/01.

Abstract essential thrombocythemia as initial cancer. Acute
myelofibrosis is presumably not a second primary according to the
hematopoietic multiple primaries table, but transition should be
noted in remarks.

Hematopoietic Primaries
• New table prepared for ICD-O-3
» Includes myelodysplasias and myeloproliferative disorders
» Based on new medical understanding of cell line diseases
» Not sequence dependent

Table Example
@ one primary only # presumably a second primary

• Implementation with cases diagnosed on or after 01/01/01
• Contemporary pathologic terminology
• Easier to find diagnoses
• No rules changes

Questions or Comments?
April Fritz, RHIT, CTR
SEER Program, NCI
Room 343J, Executive Plaza North
6130 Executive Blvd Mail Stop 7352
Rockville, MD 20852

This presentation has been APPROVED
for 2 continuing education credits by the
National Cancer Registrars Association.

Program Recognition # 2000217

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