International Classification of Diseases for

Oncology-ICD O3

Dr Mohammad Isam El Azhari

Community medicine specialist
Jordan Ministry of Health
Non-communicable Disease
Directorate
Jordan cancer registry
 ICD O3 Structure
• Introduction
It contains introductory texts on the history of ICD-O-3, on the differences
between ICD-O and ICD-10 and on the structure of ICD-O-3.
• Instructions for use
It contains encoding guidelines for topography and morphology together with the most
important rules for the use of ICD-O-3 as well as comments and encoding examples.
• Topography - Numerical list
• Morphology codes- Numerical list
• Alphabetical Index:
1. The alphabetical index is used to encode the site (topography) as well as the histology (morphology).
2. In addition, it contains non-neoplastic tumor-like lesions and precancerous conditions.
3. Topographical codes are indicated by the letter C which is also the first digit in the code numbers of Chapter II
of ICD-10.
4. The prefix M is used to identify the morphological codes.
• The International Classification of Diseases for Oncology, third edition (ICD-O-3),
is designed to categorize tumors.
• Used primarily in tumor or cancer registries for coding the site (topography) and
the histology (morphology) of neoplasms, usually obtained from a pathology
report .
• The ICD-O-3 is a dual classification(‫ )تصنيف ثنائي‬: consisting of two main axes, the
topography and the morphology of tumors.
• The topography :describes the site of the neoplasm; in general.
• The morphology : describes the cell type of the neoplasm and its biological
behavior. It thus characterizes the neoplasm itself.
• Why do we convert the information from the medical record into codes? Coding
allows us to compare the data more easily with reports and analysis.
 ICD 10

• The 10th revision of the International Statistical Classification of Diseases and Related
Health Problems(ICD)
 :Differences between ICD-O & ICD-10

• ICD O3 (just for Oncology) : is just designed to categorize tumors

• ICD10 : A medical classification list by the World Health

Organization (WHO). It contains codes for diseases, signs and
symptoms, abnormal findings, complaints, social circumstances, and
external causes of injury or diseases.

• In ICD 10 , Neoplasm is just in Chapter 2 .

In Chapter II (Neoplasm) of ICD, the topography code describes the behavior of the neoplasm
(malignant, benign, in situ, or uncertain whether malignant or benign) by assigning it to a specific range of
codes identifying each of these types of behavior.
As a result, in ICD-10, five different categories of four characters each are needed to describe
all lung neoplasms. Very few histological types are identified in ICD. As shown in figure below for lung
Neoplasms.
For example, there is no way in ICD to distinguish between an adenocarcinoma of the lung and a squamous
cell carcinoma of the lung: both would be coded to C34.9.

Figure (1) ICD-10 Alphabetic Index Entry for Lung Neoplasms

Malignant Secondary or In situ Benign Uncertain and

Metastatic Unknown

Lung C34.9 C78.0 D02.2 D14.3 D38.1

• In contrast, ICD-O3 uses only one set of four characters for
topography (based on the malignant neoplasm section of ICD-10); the
topography code (C34.9, lung) remains the same for all neoplasms of
that site.
• The behavior code in ICD O3, incorporated as the fifth digit in
the morphology field, identifies whether the neoplasm is malignant,
benign, and so forth.
• ICD-O3 also describes the type or morphology of the neoplasm; an
adenocarcinoma of the lung would thus be coded C34.9, M-8140/3,
and a squamous cell carcinoma of the lung C34.9, M8070/3.
 •
Other Differences
1. In ICD-10, the category C77 is used for secondary and
unspecified malignant neoplasms of lymph nodes.
• In ICD-O3, C77 is used as the topography code for lymph nodes.
• As a result, most of the malignant lymphomas (C81-C85) in ICD-10 are coded to
the topography code C77 in ICD-O.
2. C42 is a vacant category in ICD-10 but is used in ICD-O to designate several
topographic sites within the hematopoietic and reticuloendothelial systems.
This category serves principally as the topography site for most of the leukemias and
related conditions classified to C90-C95 in ICD-10.
The ICD-10 category for malignant neoplasm of the spleen (C26.1) .3
does not appear under digestive organs in ICD-O3. The spleen is assigned
.code C42.2, under the hematopoietic and reticuloendothelial systems
 Hydatidiform Mole and Neurofibromatosis (Von Recklinghausen
disease except bone)
• The final differences between ICD-O and Chapter II of ICD-10 are :
1. that hydatidiform mole, NOS (C58.9 M-9100/0 in ICD-O) is classified
not in Chapter II (Neoplasms) of ICD-10 but in Chapter XV
"Pregnancy, Childbirth and the Puerperium" (Category O01.9,
Hydatidiform mole),
2. Neurofibromatosis including Von Recklinghausen disease
except bone (M-9540/1 in ICD-O) appears in Chapter XVII
"Congenital Malformations, Deformations and Chromosomal
Abnormalities" as Category Q85.0.
 Functions of Neoplasms

• ICD-O does not generally provide code numbers for the function of
neoplasms, for example catecholamine production by a
malignant pheochromocytoma (C74.1, M-8700/3).
• Separate codes, such as those in Chapter IV "Endocrine, Nutritional
and Metabolic Diseases" of ICD-10, can be used to record some of the
functions of neoplasm. Catecholamine production in the example
above would be coded to E27.5.
• ICD O is a dual classification with coding systems for both
topography & morphology.
Topography code : site of origin of the neoplasms .
• The topography tells us where in the body the tumor originated. Originated is a
key word here. We ALWAYS want to code the site where the tumor started
growing.
• These topography terms have four-character codes that run from C00.0 to
C80.9. A decimal point (.) separates subdivisions of the three-character
categories .
 ;Topography ,cont

• Unknown primary site: C80.9

• Leukaemia, primary site is Bone marrow: C42.1
• Lymphoma, primary site is :
1. Lymph node: C77._
2. Extra nodal: like stomach.
Common sites :
 Breast : C50._
 Colon : C18._
 Lung: C34._
 Urinary Bladder: C67._
 Thyroid : C73._
 Prostate :61._
 Stomach: C16._
 LN: C77._
 Kidney: C64._
 Ovary: C56._
 Brain: C71._
 Skin: C44._
 Endometrium: C54.1
 Ill-defined sites: C76._

NOS example
 Stomach NOS: C16.9
Overlapping tumor example
 Breast overlapping lesion example: C50.8
Morphology : Cell type of the tumor & its biologic activity
(characteristic of the tumor).
• NOS : Not otherwise specified.
• Morphology –numerical list :
• It has 5-digit codes ranging from 8000/0 to 9992/3.
Morphology , Examples:
• Cancer, Malignant neoplasm; NOS: 8000/3.
• Carcinoma, NOS –epithelial tumor malignant: 8010/3.
• Carcinoma –basal cell: 8090/3.
• Carcinoma-basal cell – micronodular: 8097/3.
• Carcinoma –squamous cell: 8070/3.
• Adenocarcinoma: 8140/3.
• Adenocarcinoma – Endometrioid type: 8380/3.
• Most common cancer is invasive ductal carcinoma (IDC) of breast
(carcinoma –infiltrating duct): 8500/3.
• Sarcoma (NOS): 8800/3.
 ICD O complete code

• 4 character code C00.0-C80.9 site &subsite.

• The first 4 digits indicate the specific histology term .
• The fifth digit – behavior code .
• A separate one-digit code for histologic or differentiation (for leukemia or lymphoma – identify T- ,B- , Null
- & NK-cell origin) .
• Complete ICD O code requires 10 digit or characters.
Coding Guidelines for Topography and Morphology
RULE A : 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.

• Ill-defined site :
1. Term for area of the body used in a general sense ,Examples: arm,
ankle, face.
2. Has several tissue components.
 Example: Osteosarcoma of ankle.
• Issue: Primary site not fully identified in diagnosis.
• Several non-specific codes available:
1. C76.5 Ankle, NOS.
2. C40.3 Bone of ankle.
3. C44.7 Melanoma of ankle.
4. C49.2 Soft tissue of ankle.
• Use information from the cell type to code primary site.
• Example: Osteosarcoma of ankle
• Code: C40.3 bone of ankle
• Implied rule: Code as specifically as you can with the information you
have.
• Avoid using ill-defined (NOS) site codes if possible.
RULE B :PREFIXES.
• Implied rule: When you can’t find a code for the anatomic site
term, use “ill-defined site of …”
• Do not code to primary site mentioned (such as spine, stomach,
bile duct) when tumor is described as “near” that organ.

• Many prefix terms in index:

1. Periampullary C24.1
2. Periadrenal or perirenal C48.0
3. Retrocecal or peripancreatic C48.0
4. Supratentorial brain C71.0
• When term is not listed, determine the location it describes and code that.
Examples:
• Paraspinal—along the spine: C76.7 Ill-defined site of back
• Perigastric—near the stomach: C76.2 Ill-defined site of abdomen
• Peribiliary—near the biliary tract: C76.2 Ill-defined site of abdomen
RULE C:
• 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.
• Examples:
1. Tumor of breast at 12:00.
2. Glioma involving parietal and Occipital lobes.
3. Large neoplasm of descending and sigmoid colon.
• Examples:
Overlapping lesion of:
1. Tongue C02.8
2. Major salivary glands C08.8
3. Lip, oral cavity and pharynx C14.8
4. Rectum, anus and anal canal C21.8
5. Biliary tract C24.8
6. Digestive system C26.8
7. Female genital organs C57.8
8. Male genital organs C63.8
• Implied rule: If you can’t tell where a single tumor started in an organ
or system, use an “overlapping site code, C_ _ . 8.
• Does not apply to non-adjacent sites:
Example: Squamous cell carcinoma of skin of forehead and skin of
forearm , Primary site: C44.9 Skin, NOS.
(Forearm and forehead are not adjacent sites)
• Does not apply to separate lesions:
Example: Separate tumors in UIQ and LOQ of breast ,Primary site:
C50.9 Breast, NOS
• Note: Multiple primary rules may differ from ICD-O coding
RULE D- TOPOGRAPHY CODES FOR LYMPHOMAS:
• Code lymphomas arising in lymph nodes to C77._.
1. If multiple lymph node regions are involved, code to C77.8 (lymph
nodes of multiple regions).
2. If no site is indicated for a [nodal] lymphoma, code to C77.9 (lymph
node, NOS).
• Code extra nodal 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).
• Implied rule: Code a lymphoma to its site of origin.
• Examples:
1. Hodgkin lymphoma of cervical lymph nodes C77.0
2. MALT lymphoma of stomach C16.9
3. B-cell lymphoma of inguinal and femoral nodes C77.4
4. Primary lymphoma of femur C40.2
• C77.8 Special Rule: Involved lymph node chains do not have to be
adjacent.
• Examples: Code to C77._
1. Cervical and mediastinal nodes C77.8
2. Axillary and periaortic nodes C77.8
RULE E: TOPOGRAPHY CODE FOR LEUKEMIAS:
• Code all leukemia's except myeloid sarcoma (M-9903/3) to C42.1,
bone marrow.
• Myeloid sarcoma: a deposit of malignant myeloid cells in soft tissue.
Code to location of lesion.
• The following are also coded to bone marrow, C42.1
1. Multiple myeloma
2. Refractory anemia's
3. Polycythemia vera
4. Myelodysplastic syndrome
5. Other hematopoietic diseases
RULE F: Behavior code in morphology: Use the appropriate 5th digit
behavior code even if the exact term is not listed in ICD-O3.
• The use of the 5th digit behavior code is explained later.
• The appropriate 5th digit code should be used even if the exact term
is not listed in ICD-O; for example, “benign chordoma” as a diagnosis
should be coded 9370/0. If the pathologist states that the behavior
differs from the usual behavior as given in ICD-O, code as the
pathologist indicates.
RULE G: Grading or differentiation code.

• Assign the highest grade or differentiation code described in the diagnostic

statement.
• The use of the 6th digit for grading or differentiation of solid tumors is explained
later.
• If a diagnosis indicates two different degrees of grade or differentiation (such as
“well and poorly differentiated” or “grades II–III”), code to the higher grade.
• This 6th digit may also be used for identifying the cell origin for lymphomas and
leukemia's. In these lymphatic and hematopoietic diseases, T-cell (code 5), B-cell
(code 6), Null cell (code 7), and NK cell (code 8) take priority over grade codes 1
to 4.
RULE H: 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.
• The appropriate site-specific codes are listed in parentheses after morphology terms for
neoplasms that usually occur in the same site or tissue, for example “retinoblastoma”
(C69.2). If no site is indicated in the diagnosis, use the suggested code.
• If the site given differs from the site-specific code indicated for the morphologic type,
use the appropriate code for the site given. This should be done only after thoroughly
reviewing the case to ascertain that the neoplasm at the site mentioned is not a
metastasis.
RULE J: Compound morphology diagnoses.
• Change the order of word roots in a compound term if the term is
not listed in ICD-O3.
• Not all forms of compound words are listed. For example,
“myxofibrosarcoma” is not in ICD-O but “fibromyxosarcoma” is.
• Check various permutations of the word roots if the first term is not
found.
RULE K: 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.
• An example :
1. “transitional cell epidermoid carcinoma”
2. Two codes are found “Transitional cell carcinoma, NOS” is coded
8120/3 and “epidermoid carcinoma, NOS” is coded 8070/3.
3. When there is no single code that includes all diagnostic elements,
coders should use the numerically higher code number, 8120/3 in
this example, as it is usually more specific.
Behavior:
The behavior of a tumor is the way it acts within the body.

5th digit behavior code for neoplasms

• /0 Benign
• /1 Uncertain whether benign or malignant ,Borderline malignancy,
Low malignant potential ,Uncertain malignant potential.
• /2 Carcinoma in situ ,Intraepithelial, Noninfiltrating ,Noninvasive .
• /3 Malignant, primary site.
• /6* Malignant, metastatic site Malignant, secondary site.
• /9* Malignant, uncertain whether primary or metastatic site.
• * Not used by cancer registries.
Histologic grading & differentiation / 6th digit:
Differentiation describes how much or how little a tumor resembles the
normal tissue from which it arose.
Only malignant tumors are graded.
• 1. Grade I : Well differentiated, NOS.
• 2. Grade II : Moderately differentiated ,Intermediate differentiation.
• 3. Grade III: Poorly differentiated.
• 4 .Grade IV :Undifferentiated, Anaplastic .
• 9. Grade or differentiation : not determined, not Stated or not
applicable.
6th digit –denote cell lineage for leukemia & lymphomas (Immunophenotype
designation ‫)تعيين النمط المناعي‬.

• T-cell (5).
• B-cell (6) /Pre-B / B-precursor.
• Null cell (7) /Non T-non B .
• NK cell/Natural killer cell (8).
• Cell type not determined, not stated or not applicable (9).
The End