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International Classification of Diseases for Oncology (3 rd Edition). History. Brief History leading to ICD-O- 1893 WHO responsible for publishing international classification of Mortality

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International Classification

of Diseases for Oncology

(3rd Edition)

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  • Brief History leading to ICD-O-

  • 1893 WHO responsible for publishing international classification of Mortality

  • 1948 6th Revision of International Statistical Classification of Diseases, Injuries, and cause of death ICD) (9) Used to code mortality and Morbidity data

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History cont’d

  • Classification of Neoplasm was based on site and behavior (whether malignant benign or not otherwise specified).

  • 1951- 1st coding manual for Morphology by American Cancer Society

  • 1971 Recommendation for ICDO was approved by WHO- 2nd edition of ICDO published in 1990 to be used by cancer registries

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What is ICD-O?

  • Not a true classification system

    • Includes codes from multiple classification systems

  • Actually a coded nomenclature for :

    • Topography (primary site of origin)

    • Morphology (histology, behavior, grade)

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Rationale for ICD-O

  • To standardize cancer data collection

  • To compare data

    • Regional

    • National

    • International

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Why a new edition?

  • Immense changes over the past decade in techniques for diagnosing neoplasms

  • Science has evolved (cytogenetics, immunophenotyping, etc)

  • 1998 – IARC task force

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ICD-10 vs ICD-O

  • ICD-10 topography code describes the behavior of the neoplasm

    Ex) Lung neoplasm (ICD-10)

    C34.9 malignant D02.2 in situ D14.3 benign

    Ex) Lung neoplasm (ICD-O-3), carcinoma

    C34.9 lung, NOS

    8010 / __ (3, 2, 0 )

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ICD-10 vs ICD-O

Behavior ICD-10 Category Term

/0 D10-D36 Benign

/1 D37-D48 Borderline

/2 D00-D09 In situ

/3 C00-C76, Invasive


/6 C77-C79 Metastatic

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Contents of ICD-O-3

pp. 001-044) Introduction & Instructions for Use

pp. 045-068) Topography (site) - Numerical List

pp. 069-104) Morphology (type) - Numerical List

pp. 105-218) Alphabetic Index (site & type)

pp. 219-240) Appendices

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Topography – Numerical List

  • Structure C__ __ . __

    • Example) LIP

  • Range : C00.0 – C80.9

    • Grouped by systems / common organs

    • Numerical order

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Major Topography Groups

  • C00-14 Lip, Oral Cavity, Pharynx

  • C15-26 Digestive Organs

  • C30-39 Respiratory System

  • C40-41 Bones, Joints, Cartilage

  • C44 Skin

  • C47 Nerves

  • C49 Soft Tissues

  • C50 Breast

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Major Topography Groups

  • C51-58 Female Genital Organs

  • C60-63 Male Genital Organs

  • C64-68 Urinary Tract

  • C69-72 Eye, Brain, CNS

  • C73-75 Thyroid and Endocrine Glands

  • C76 Other Ill defined sites

  • C77 Lymph Nodes

  • C80 Unknown

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Topography – Numerical List

  • Preferred terms in boldface

  • Synonyms indented

  • Equivalent terms not indented

    --subdivision of preferred term not sufficiently different to warrant own code

    Example) C07 Parotid Gland --- page 46

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Morphology - Numerical List

  • Structure __ __ __ __ / __

    • Example) Neoplasm

  • Range : 8000/0 to 9989/3

    • Grouped by common cell types

    • Numerical order

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Major Morphology Groups

  • 800 Unspecified

  • 801-867 Carcinomas

  • 872-879 Melanomas

  • 880-899 Sarcomas

  • 906-909 Germ Cell

  • 938-948 Gliomas

  • 959-972 Lymphomas

  • 980-994 Leukemias

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Morphology – Numerical List

  • Preferred terms in boldface

  • Synonyms indented

  • Equivalent terms not indented

    --subdivision of preferred term not sufficiently different to warrant own code

    Example) 8045/3 Combined small cell carcinoma --- page 70

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Behavior, Grade/Differentiation

  • Located on pages between numerical topography and morphology lists

  • Place purple ribbon here for quick reference!

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Morphology - Behavior

  • Behavioris the fifth digit (after the “/”) of the morphology code

  • Behavior code indicates the pathologic behavior of the neoplasm - benign, in situ, malignant

  • Most registries in the world only collect cancer with behavior codes 2 (in situ) and 3 (malignant)

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Morphology – Grade/Differentiation

  • Grade/Differentiation is the sixth digit (after the /) of the morphology code

  • Describes how much or how little a tumor resembles the normal tissue from which it arose

  • Code the grade of the primary tumor. Do code the grade if given for an in situ neoplasm. Do not code grade for intraepithelial neoplasias

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ICD-O-3 Code = 10 Digit Code

  • TOPOGRAPHY 4 Digits


  • MORPHOLOGY 4 Digits


  • BEHAVIOR 1 Digit


  • GRADE 1 Digit

    10 Digits

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Alphabetic Index

  • Topography (C) and morphology (M) terms listed together alphabetically but not mixed. Space before and after each group.

  • Boldface header for any word that appears as part of 3 or more terms (terms indented)

  • NOS terms listed first

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Meaning and Use of NOS

  • Not Otherwise Specified

  • Printed after a term when it :

    • lacks a modifying or more explicit word or phase

    • contains an adjective that is not listed

    • used in a general sense

      Examples) Adenocarcinoma & Endocrine Gland

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General Coding Guidelines -Topography

  • Code the site in which the primary tumor originated, even if it extends to an adjacent subsite.

  • Code subsite to .9 for single primaries when multiple tumors arise in different subsites of the same anatomic site.

    • Infiltrating duct carcinoma in UOQ of R Breast and infiltrating duct carcinoma in LIQ R Breast

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General Coding Guidelines - Site

  • If a tumor is metastatic and the primary site is unknown, code primary site to C80.9

  • If the medical record does not contain enough info to code site:

    • Consult physician advisor

    • Use NOS for organ system or ILL defined site code

    • Code unknown C80.9

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Coding Guidelines

  • Topography – p23-24

    • Where the tumor arose

    • No changes from ICD-O-2

    • Noun form appears in manual

      • Glioma of pons vs pontine glioma

    • Special Codes

      • Esophagus

      • Branchial Cleft, Meckel diverticulum

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Topography - Ill-Defined Sites (A)

Codes C76._

  • If the diagnosis specifies the tissue of origin, code to the tissue suggested in the alphabetic index in preference to NOS

  • C76._ codes should be used very rarely as the histology usually will allow you to use a more specific code

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Example of Possible Codes Use of NOS for Ill-defined Sites


C76.0 NOS

C44.4 NOS (carcinoma, melanoma, nevus)

C49.0 NOS (sarcoma, lipoma)

C49.0 adipose tissue

C47.0 autonomic nervous system

C49.0 connective tissue

C44.4 skin

C49.1 tendon

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Example of Rule A

Code : Malignant carcinoma of the arm

Answer : C44.6 (skin of upper limb)

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Topography – Prefixes (B)

  • Prefixes are often used to describe topographic sites and various organs (ex. peri-, para-, pre-, supra-, etc)

  • Some prefixes modify the topographic site and are listed with specific codes (ex. C77.2 Para-aortic lymph node)

  • Use Ill-defined site code C76._ for terms such as those above if not specifically listed in ICD-O (only if Rule A does not apply)

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Example of Rule B

Code : periabdominal cystadenocarcinoma

Answer : C76.2 (abdomen, NOS)

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Topography - Overlapping Sites (C)

  • A single neoplasm that overlaps two contiguous sites and whose point of origin cannot be determined is coded to

    ‘C__ __ .8’ unless..

  • The combination is listed elsewhere

Carcinoma of tip and ventral surface of the tongue

C02.8 Overlapping lesion of the tongue

Carcinoma of esophagus and stomach

C16.0 Cardioesophageal junction

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Example of Rule C

Code : Carcinoma overlapping the stomach and small intestine?

Answer : C26.8 (overlapping digestive)

Code : Overlapping tumor of the cervix and uterus?

Answer : C57.8 (overlapping female genital)

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Topography – Lymphomas (D)

  • Always code site of origin (extranodal/nodal)

    • malignant lymphoma of the stomach = C16.9

    • malignant lymphoma of lymph node = C77._

    • spleen and splenic lymph nodes = C42.2

    • spleen and non-regional nodes = consult(C77.9)

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Topography – Lymphomas (D)

  • If no site of origin is given, code to lymph nodes, NOS (C77.9);– C80.9 if suspected to be extranodal but site is not stated.

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Topography – Lymphomas (D)

  • When multiple lymph node chains are involved:

    • Code where originated

    • If all involved chains are in the same lymph node region, code to C77.__

    • If involved chains are in different lymph node regions, code to C77.8

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Example of Rule D

Code : Lymphoma of inguinal and iliac lymph nodes

Answer : C77.8 (multiple regions)

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Topography - Bone Marrow C42.1 (E)

  • All leukemias have a site-specific code of bone marrow (C42.1)except:

    • Myeloid sarcoma (rare) – leukemic deposit in an organ and should be coded to that organ

  • Multiple myeloma – code site to C42.1

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Group Exercise - Topography

  • Ascending colon

    • C18.2

  • Axillary lymph node

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Group Exercise - Topography

  • Ascending colon

    • C18.2

  • Axillary lymph node

    • C77.3

  • UOQ right breast

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Group Exercise - Topography

  • Ascending colon

    • C18.2

  • Axillary lymph node

    • C77.3

  • UOQ right breast

    • C50.4

  • Temporal and parietal lobes (1 tumor)

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Group Exercise - Topography

  • Ascending colon

    • C18.2

  • Axillary lymph node

    • C77.3

  • UOQ right breast

    • C50.4

  • Temporal and parietal lobes (1 tumor)

    • C71.8

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Individual Exercises

Practice Topography Coding

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General Coding Guidelines - Histology

  • Histology can only be coded after multiple primary determination has been made

  • If there is no tissue, code the histology the medical practitioner uses to describe the tumor

  • Use the histology stated in the final diagnosis from the pathology report, unless a more specific histology is definitively described elsewhere (i.e. micro, comment)

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General Coding Guidelines - Behavior

  • Collect in situ and malignant always

  • Collect benign and borderline intracranial and CNS effective 1/1/2004

  • Code the behavior as /3 if any portion of the primary tumor is invasive

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General Coding Guidelines - Grade

  • Code the grade from the final diagnosis of the pathology report.

  • Code grade from primary tumor, not from a metastasis or recurrence

  • If tumor has invasive and in situ components, code grade of invasive component of tumor. If invasive component unknown then code 9

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Morphology – Matrix Concept (F)

  • Use the appropriate 5th digit behavior code even if the exact term is not listed in ICD-O

  • It is okay to over-ride suggested code if pathology report says something different (“Matrix Principle”)

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Example of Rule F

Code : Benign chordoma

Answer : 9370/0

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Morphology-Grade/Differentiation (G)

  • If a pathology report gives more than one grade/diff, code to the highest grade/diff

  • If the morphology code includes grade, repeat grade code as sixth digit

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Example of Rule G

Code : moderately to poorly differentiated ` adenocarcinoma

Answer : 8140/33

Code : anaplastic malignant teratoma

Answer : 9082/34

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Morphology - 6th Digit for Lymphomas and Leukemias

Grade - Description

5 T-cell

6 B-cell,Pre-B,B-precursor

7 Null cell, non T or non B

8 N K cell (natural killer)

9 Unknown, Undetermined

  • Codes for cell lineage take precedence over grade/diff codes 1-4. Based on path/lab report.

  • Do not borrow from bold face headers (9680/3)

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Leukemias and Lymphomas

  • ICD-O-2 Classification based on Working Formulation (means of translating terminology among various other classification schemes – grouped by morphologic characteristics)

    low grade – slow progressing, most favorable

    intermediate grade

    high grade – fast progressing, least favorable

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Using grade to assign 6th digit

  • Errata # 1 – Read Clarifications!

  • Certain instances, term grade does not represent diff. but instead is a syn for “category”. 6th digit should not be coded as grade 1,2,3 for these cases

    • Ex. Follicular lymphomas (don’t use grade)

    • Ex. Low grade endometrial stromal sarcoma (do use grade)

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More on Grade

  • Lymphomas and Leukemias

    • Do not use the terms “high grade”, “low grade” and “intermediate grade” to code differentiation. These terms refer to histology in the Working Formulation.

  • Lymphomas

    • Do not use the descriptions “grade 1”, “grade 2” and “grade 3” to code differentiation. These represent lymphoma types

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Lymphoma grades

Code : Follicular Lymphoma, grade 1

Answer : 9695/39

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Bold face headers

Code : Diffuse centroblastic lymphoma

Answer : 9680/39 NOT 9680/36

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Morphology - Site Specific (H)

  • Some site specific morphology terms have suggested primary site codes in the index

  • If no specific primary site is listed in the diagnosis, default to the suggested code

    • Disregard suggested code if tumor is known to arise at another primary site

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Example of Rule H

Code : Pathology reports states infiltrating duct carcinoma with no mention of primary site

Answer : C50.9 (Breast, nos)

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Morphology – Compound Terms (J)

  • Some tumors have more than one histologic pattern

  • Common combinations are listed and given a specific code

    • Two new codes for mixed breast subtypes

      (8523/_ And 8524/_)

    • If any part of the tumor is invasive, the behavior must be coded to 3

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Morphology – Compound Terms (J)

Code : Mixed adenocarcinoma & squamous cell carcinoma

Answer : 8560/3 (adenosquamous carcinoma)

Code : Papillary and follicular carcinoma

Answer : 8340/3

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Morphology – Compound Terms (J)

  • Compound terms with multiple prefixes may not be in the index - check for alternate permutations of the prefix(es) and the base term

  • Myxofibrosarcoma vs. Fibromyxosarcoma

  • Look up myxofibrosarcoma

  • Not there?

  • Try moving around the prefixes

  • Look up Fibromyxosarcoma

  • Fibromyxosarcoma = 8811/3

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Morphology - Multiple Terms (K)

  • If two or more modifying terms describe the same single tumor and no single code exists, use the higher morphology code (more specific)

  • Ex. Transitional cell epidermoid carcinoma

    (1 tumor)

    Epidermoid carcinoma - 8070/3

    Transitional cell carcinoma, NOS - 8120/3

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Morphology - Multiple Terms (K)

  • Rule K does not apply to separate tumors nor to the hematopoetic diseases in general.

  • For the hematopoetic diseases, code the more specific histology over the higher code.

    Example) Diffuse large B-Cell lymphoma (9680/3)

    Mantle cell lymphoma (9673/3)

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Morphology - Multiple Terms (K)

  • 9680/3 – includes NOS term and 27 synonyms (non-specific diagnosis)

  • 9673/3 – does not include NOS and has many fewer synonyms (more specific)

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Exception to rule (K) – errata!

  • With ICD-O-3, many new subtypes of tumors were added. Some subtypes have lower codes than the NOS term.

  • Code the subtype of the tumor even if it is lower than the NOS term

  • Ex) Ductal carcinoma, cribriform type

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Coding Order of Single Tumor

  • Code histology if only 1 type mentioned

  • Code invasive histology if invasive and insitu

  • Use a mixed code if one exists

  • Use a combination code if one exists

  • Code the more specific term

  • Code the majority of the tumor

  • Code the numerically higher ICD-O-3 code

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Group Exercise - Morphology

  • Medullary adenocarcinoma

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Group Exercise - Morphology

  • Medullary adenocarcinoma

    • 8510/3

  • Acute myelomonocytic leukemia

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Group Exercise - Morphology

  • Medullary adenocarcinoma

    • 8510/3

  • Acute myelomonocytic leukemia

    • 9867/3

  • Malignant neuroastrocytoma

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Group Exercise - Morphology

  • Medullary adenocarcinoma

    • 8510/3

  • Acute myelomonocytic leukemia

    • 9867/3

  • Malignant neuroastrocytoma

    • 9505/3

  • Seminoma and teratocarcinoma

    (1 tumor)

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Group Exercise - Morphology

  • Medullary adenocarcinoma

    • 8510/3

  • Acute myelomonocytic leukemia

    • 9867/3

  • Malignant neuroastrocytoma

    • 9505/3

  • Seminoma and teratocarcinoma (1 tumor)

    • 9081/3 (higher than 9061/3 and more specific)

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Individual Exercises

Practice Morphology Coding

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Multiple Neoplasms

  • A primary cancer is one that originates in a primary site or tissue

  • Code primary cancer, not extension, recurrence or metastasis

  • Consult an oncologist for difficult cases

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Rules for Multiple Primaries

  • How to know if we are talking about two or more separate primaries?

    Follow rules – In the U.S. registries use the SEER rules for determining multiple primaries.

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Hematopoietic Multiple Primaries

  • If the physician states that a hematopoietic diagnosis is a new primary, use that information. Otherwise, refer to the 52x52 table.

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Basis of DiagnosisCoding the Diagnosis

Diagnostic Confirmation

  • Microscopic diagnosis preferred

    • Positive histology, cytology, microscopic NOS

  • Use Non-microscopic diagnosis only if microscopic does not exist

    • Lab test, direct visualization without mc, radiology, other clinical diagnosis

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  • 1. New ICD-O-3 codes

  • 2. New terms & synonyms (codes were in ICD-O-2)

  • 3. Terms that changed morphology codes

  • 4. Terms that changed from tumor-like lesions to neoplasms

  • 5. Deleted terms

  • 6.Terms that changed behavior code

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ICD-O-3 Training

ICD-O-3 Training Web Site

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  • Many thanks to Professor John Young of the Rollins Schools of Public Health at Emory University who assisted with material for this presentation.

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Time for Exercises!

Now, let’s look at some more exercises and practice coding site and morphology using ICD-O-3