2007 Multiple Primary and

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1 2007 Multiple Primary and Histology Coding Rules Beyond the Basics Florida Cancer Data System Annual Conference Tampa, FL July 26,2007 Steven Peace, CTR Westat Carol Johnson, CTR NCI SEER Peggy Adamo, RHIT, CTR NCI SEER 1

2 Introducing 2007 MP/H Rules 2

3 Old Rules 3

4 New Rules 4

5 When to Use 2007 Rules The rules are effective for cases diagnosed January 1, 2007 and after Do not use these rules to abstract cases diagnosed prior to January 1,

6 When to Use 2007 Rules New tumor dx 2007 Subsequent tumor dx

7 Compare Original to 2007 Timing: dx date original tumor Use MP rules to determine MP status 7

8 Do Not Use For Metastatic Ds. Hx of melanoma presents with positive nodes Mets Hx breast ca presents with multiple lung metastasis 8

9 Stay on Track Use the rules in hierarchical order Don t berry pick Don t go directly to the rule you think applies 9

10 Stay on Track STOP when you find the rule that fits So, what is a fit? Example: Tumors on both sides (right and left breast) are multiple primaries 10

11 General Instructions Reference: B.4.b.II.iii When there is a tumor or tumors with separate microscopic foci, ignore the separate microscopic foci and use the Single Tumor or Multiple Tumor modules as appropriate. 11

12 Definitions Focus: A term used by pathologists to describe a group of cells that can be seen only by a microscope. The cells are noticeably different from the surrounding tissue either by their appearance, chemical stain, or other testing. Foci: Pluraloffocus focus 12

13 Not to be Confused with Focal: Adjective meaning limited to a Specific area Specific organ May be microscopic or macroscopic 13

14 Choosing Tumor Module Just like always Single biopsy single tumor Multiple biopsies multiple tumors Multiple foci multiple tumors 14

15 Choosing Tumor Module Just like always Count the number of primary tumors Do not count metastatic tumors Do not mix benign with malignant 15

16 Never Change Remember Always record the histology at diagnosis Histology may evolve unlikely Recurrence Transformation Grade/differentiation more likely to change with disease progression 16

17 Most Representative Specimen Most TUMOR tissue Cannot determine - use best info 17

18 Invasive and In Situ Histology Code the invasive histology What if what if you also have more than one invasive histology in the mix? Use invasive/in situ rule/module 2 nd pass histology rules 18

19 What Happened to Recurrence? Single primary = recurrence.. Record date of dx Record other recurrence info 19

20 20

21 Histology Chart - Lung Malignant neoplasm, NOS and Malignant tumor cells (8000 and 8001) Carcinoma, NOS, Carcinoma, undifferentiated, NOS and Carcinoma, anaplastic, NOS (8010, 8020 and 8021) Neuroendocrine CA, NOS (8246) Non-Small Cell CA (8046) Carcinoid, NOS (8240) Small Cell CA, NOS (8041) Sarcomatoid CA (8033) Pleomorphic CA (8022) Large Cell CA, NOS (8012) AdenoCA, NOS (8140) Squamous Cell CA, NOS (8070) 21

22 Histology Chart - Lung Chart Instructions: Use this chart with multiple primary rule M10 to identify types of non-small cell carcinoma. Use this chart with the histology rules to code the most specific histologic term. The tree is arranged in descending order. Each branch is a histology group, starting with the NOS or group terms and descending into the specific types for that group. As you follow the branch down, the terms become more specific. Neuroendocrine CA, NOS (8246) Malignant neoplasm, NOS and Malignant tumor cells (8000 and 8001) Carcinoma, NOS, Carcinoma, undifferentiated, NOS and Carcinoma, anaplastic, NOS (8010, 8020 and 8021) Non-Small Cell CA (8046) Sarcomatoid CA (8033) Carcinoid, NOS (8240) Combined Small Cell CA (8045) Small Cell CA, NOS (8041) Carcinosarcoma (8980) Giant cell CA (8031) Pleomorphic CA (8022) Pulmonary Blastoma (8972) Spindle cell CA (8032) Atypical carcinoid (8249) Fusiform cell CA (8043) Large Cell CA, NOS (8012) AdenoCA, NOS (8140) Adenoid cystic CA (8200) and Mucoepidermoid CA (8430) Adenosquamous (8560) Squamous Cell CA, NOS (8070) Acinar cell CA (8550) Adenocarcinoma, mixed subtypes (8255) Alveolar adenocarcinoma (8251) Bronchioloalveolar CA, NOS (8250) Bronchioloalveolar CA, non mucinous (8252) Bronchioloalveolar CA, mucinous (8253) Bronchioloalveolar CA, mixed mucinous & non mucinous (8254) Clear cell adenoca (8310) Mucinous cystadenoca (8470) Mucinous/colloid adenoca (8480) Mucin-producing adenocarcinoma (8481) Papillary adenoca (8260) Signet ring adenoca (8490) Solid AdenoCA (8230) Well differentiated fetal AdenoCA (8333) Large cell neuroendocrine CA (8013) Large cell CA with rhabdoid phenotype (8014) Lymphoepithelioma like CA (8082) Basaloid CA (8123) Clear cell CA (8310) Basaloid squamous cell CA (8083) Papillary squamous cell CA (8052) Squamous cell CA, clear cell type (8084) Squamous cell CA, keratinizing, NOS (8071) Squamous cell CA, large cell, nonkeratinizing, NOS (8072) Squamous cell CA, small cell, nonkeratinizing, (8073) 22

23 Malignant Neoplasm/Cells Neoplasm, NOS Malignant neoplasm, NOS Tumor cells, malignant, NOS Capability bl of invading or extending 23

24 Carcinoma, NOS Epithelial cells that have the capability of invading or extending Excludes sarcoma, lymphoma, and melanoma 24

25 Non-Small Cell Carcinoma Not small cell Default (inadequate tissue) 25

26 Sarcomatoid Carcinoma Sarcoma-like differentiation Contains spindle cells and/or giant cells 26

27 Pleomorphic Carcinoma Poorly differentiated Contains spindle cells and/or giant cells 27

28 Large Cell Carcinoma, NOS Undifferentiated Cannot identify squamous or glandular differentiation 28

29 Adenocarcinoma, NOS Carcinoma/epithelial with Glandular differentiation or Mucin production 29

30 Squamous Carcinoma, NOS Carcinoma, epithelial with Keratinization and/or Intercellular bridges 30

31 Most Common Histologies Squamous cell carcinoma Adenocarcinoma Large cell carcinoma 31

32 Neuroendocrine vs. Large Cell Neuroendocrine Large cell neuroendocrine Large size Neuroendocrine differentiation Neuroendocrine Small size 32

33 Histology Chart - Lung Chart Instructions: Use this chart with multiple primary rule M10 to identify types of non-small cell carcinoma. Use this chart with the histology rules to code the most specific histologic term. The tree is arranged in descending order. Each branch is a histology group, starting with the NOS or group terms and descending into the specific types for that group. As you follow the branch down, the terms become more specific. Neuroendocrine CA, NOS (8246) Malignant neoplasm, NOS and Malignant tumor cells (8000 and 8001) Carcinoma, NOS, Carcinoma, undifferentiated, NOS and Carcinoma, anaplastic, NOS (8010, 8020 and 8021) Non-Small Cell CA (8046) Sarcomatoid CA (8033) Carcinoid, NOS (8240) Combined Small Cell CA (8045) Small Cell CA, NOS (8041) Carcinosarcoma (8980) Giant cell CA (8031) Pleomorphic CA (8022) Pulmonary Blastoma (8972) Spindle cell CA (8032) Large Cell CA, Atypical carcinoid (8249) Fusiform cell CA (8043) NOS (8012) AdenoCA, NOS (8140) Adenoid cystic CA (8200) and Mucoepidermoid CA (8430) Adenosquamous (8560) Squamous Cell CA, NOS (8070) Acinar cell CA (8550) Adenocarcinoma, mixed subtypes (8255) Alveolar adenocarcinoma (8251) Bronchioloalveolar CA, NOS (8250) Bronchioloalveolar CA, non mucinous (8252) Bronchioloalveolar CA, mucinous (8253) Bronchioloalveolar CA, mixed mucinous & non mucinous (8254) Clear cell adenoca (8310) Mucinous cystadenoca (8470) Mucinous/colloid adenoca (8480) Mucin-producing adenocarcinoma (8481) Papillary adenoca (8260) Signet ring adenoca (8490) Solid AdenoCA (8230) Well differentiated fetal AdenoCA (8333) Large cell neuroendocrine CA (8013) Large cell CA with rhabdoid phenotype (8014) Lymphoepithelioma like CA (8082) Basaloid CA (8123) Clear cell CA (8310) Basaloid squamous cell CA (8083) Papillary squamous cell CA (8052) Squamous cell CA, clear cell type (8084) Squamous cell CA, keratinizing, NOS (8071) Squamous cell CA, large cell, nonkeratinizing, NOS (8072) Squamous cell CA, small cell, nonkeratinizing, (8073) 33

34 Equivalent Terms Low grade neuroendocrine carcinoma = carcinoid 34

35 Neuroendocrine CA, NOS Small cell (poor prognosis) Carcinoid (excellent prognosis) 35

36 Neuroendocrine CA, NOS Carcinoid, NOS (typical carcinoid) Atypical carcinoid Combined small cell Small cell ca, NOS Fusiform cell CA 36

37 Mixed/Combination Table

38 Table 1 Required Small cell ca AND one histology in Column 2 Note: Diagnosis must be small cell ca (NOS), not a subtype Additional Required Adenoca Large cell ca Squamous cell ca Term Combined small cell ca; Mixed small cell ca Code

39 Table 1 Required Squamous cell ca AND one histology Column 2 Additional Required Spindle cell carcinoma Sarcomatoid Term Squamous cell ca, spindle cell Squamous cell, sarcomatoid Code

40 Required Additional Required Term Code At least Acinar Adenoca 8255 two Bronchioloalveolar carcinoma /w mixed histologies subtypes in Column Bronchioloalveolar carcinoma 2 non mucinous Bronchioloalveolar carcinoma mucinous (goblet cell) Bronchioloalveolar ca mixed mucinous and non-mucinous Clear cell adenocarcinoma Papillary adenocarcinoma Solid adenocarcinoma Well-differentiated fetal adenocarcinoma 40

41 Colon 41

42 Colonoscopy Measurements* Transverse Ascending Descending Cecum 150 Rectum 4-16 Anus 0-4 Sigmoid Rectosigmoid * from anal verge Approximations only. Source: AJCC Cancer Staging Manual, fifth edition, page 85,

43 Frequency Distribution Transverse Hepatic Flex 6.4% Splenic Flex 3.9% 2.7% Ascending 11.1% Large Intestine, NOS 4.6% Descending 4.5% Cecum 16.3% Appendix 0.8% Rectum 18.6% Sigmoid 21.9% Rectosigmoid 9.2% Source: SEER Public Use File 18 registries, , November 2004 (N=439,000) 43

44 Colon MP Rules Many of historic rules still apply Tumor location is still key Most colon cancers are single tumors Do not expect change in # of cancers 44

45 Colon Histology Rules Most colon cancers are adenocarcinoma More focus on polyps More focus on amount of mucin present Fewer mucinous adenocarcinoma 45

46 Polyps It is important to know that the adenocarcinoma originated in a polyp in adenomatous polyp in adenomatous polyposis coli in multiple adenomatous polyps in villous adenoma in tubulovillous adenoma 46

47 Polyps Exception to Rules: Registrars can look for clinical or pathological evidence of polyps associated with a malignancy: Residual polyp Operative report Gross section of pathology report Microscopic i section of pathology report Other mention in record 47

48 Is it a Polyp? Exophytic or Polypoid do not a polyp make 48

49 Not Synonymous With Polyp Exophytic: growing outward Polypoid: polyp-shaped Nodular: knob-like growth 49

50 Exophytic nodular or polypoid pedunculated or sessile 50

51 Polypoid 51

52 Types of Polyps Pedunculated (on a stalk) Sessile (flat) Source: Abeloff et al: Clinical Oncology, third edition, Elsevier Churchill Livingstone,

53 Types of Polyps Tubular Tubulovillous Villous Head Stalk Muscularis mucosa Submucosa Muscularis propria Source: A.T. Skarin, Atlas of Diagnostic Oncology, 2nd ed., Mosby Wolfe,

54 Adenomatous Polyps AKA adenoma Benign neoplasms Precursor to cancer One or many polyps 54

55 CA in Adenomatous Polyposis Carcinoma in at least 2 polyps 55

56 CA in Adenomatous Polyposis Code 8221 (adenocarcinoma in multiple adenomatous polyps) when: <=100 polyps in specimen, or Multiple malignant polyps, number unknown or not stated and FAP not mentioned 56

57 Familial Adenomatous Polyposis Code 8220 (adenocarcinoma in adenomatous polyposis coli) when: >100 polyps in specimen, or Path diagnosis adenocarcinoma in adenomatous polyps and person has clinical history of familial polyposis Number of polyps unknown and diagnosis is familial polyposis 57

58 Familial Adenomatous Polyposis Inherited condition 100s to 1000s of colon polyps < 1% of colon cancers Colon cancer risk nearly 100% Most treated t with resection 58

59 Familial Adenomatous Polyposis 59

60 Multiple Primary Rules Adenocarcinoma in adenomatous polyposis coli (familial adenomatous polyposis) with one or more malignant polyps single primary. Tumors may be present in multiple segments of the colon or rectum or in a single segment of the colon. 60

61 Multiple Primary Rules Sites with topography codes that are different at the second (Cxxx), third (Cxxx) or fourth (C18x) character are multiple l primaries. i 61

62 Multiple Primary Rules Diagnosed more than one (1) year apart are multiple primaries. A frank adenocarcinoma and a tumor in a polyp (in situ or malignant) is a single primary. 62

63 Frank n Polyp 63

64 Multiple Primary Rules Multiple in situ and/or malignant polyps are a single primary. includes all combinations of adenomatous, tubular, villous, and tubulovillous adenomas or polyps. 64

65 Histology 95-98% are adenocarcinoma Only 10-15% 15% produce enough mucin to be classified as mucinous adenocarcinoma. 2-4% are of neuroendocrine origin 65

66 Histology Mixed/combination histology is rare Adenocarcinoid Composite carcinoid Combined mucinous and signet ring cell adenocarcinoma 66

67 Histology Neuroendocrine carcinoma is a group of carcinomas - includes carcinoid tumors Typical carcinoid tumors are low grade (grade I-II) neuroendocrine carcinomas Atypical carcinoid id Small cell carcinoma 67

68 Polyps Polyp rules come before mucin rules Descriptions in Definitions iti Exception to rules clinical/pathologic 68

69 Is it mucinous or signet ring? Mucinous adenocarcinoma Extra-cellular mucin > 50% of the tumor Not simply mucin-producing i Not simply mucin-secreting 69

70 Is it mucinous or signet ring? Signet ring cell adenocarcinoma Intra-cellular mucin > 50% of the tumor 70

71 with signet ring cells Does not mean Signet Ring Cell Carcinoma 71

72 For Colon ONLY Code 8480 (mucinous adenocarcinoma) only when the final diagnosis is mucinous adenocarcinoma, or Documentation on path clearly states >50% mucinous (may use microscopic) 72

73 For Colon ONLY Code 8490 (signet ring adenocarcinoma) only when the final diagnosis is signet ring cell adenocarcinoma, or Documentation on path clearly states >50% signet ring cell (may use micro) 73

74 What about mucin-producing? When the final diagnosis states mucin-producing then code it. Do not code mucinous or mucin- producing unless meets specific criteria. 74

75 Most Invasive Code the most invasive when: Frank adenocarcinoma and carcinoma in a polyp, or In situ and invasive, or Multiple invasive 75

76 Urinary 76

77 Synonyms Flat transitional cell Flat urothelial In situ transitional cell In situ urothelial l 77

78 78

79 79

80 Cross-section of Ureter Muscular layer Connective Tissue Epithelium 80

81 Hierarchical Order M5 Bladder tumors with any combination of the following histologies: papillary carcinoma (8050), transitional cell carcinoma ( ), 8124), or papillary transitional cell carcinoma ( ), are a single primary. M6 Tumors diagnosed more than three years apart are multiple primaries 81

82 Rule M8 Urothelial tumors in two or more of the following sites are a single primary Renal pelvis (C659) Ureter (C669) Bladder (C670-C679) Urethra/prostatic urethra (C680) 82

83 CIS: Mucosal Spread Confined to epithelium Focal, multifocal, diffuse Flat-non-papillary (default to Tis) High-grade carcinoma 83

84 Diffuse CIS Prostatic urethra Prostatic ducts Seminal vesicles Distal ureters 84

85 Field Effect Change in urothelium Multifocal Multicentric Multiple centers of origin Multiple l urinary sites 85

86 Implantation Tumor cells in urine implant Head to toe direction Discontinuous 86

87 H3 Code 8120 TCC Pure TCC or flat TCC or TCC with Squamous differentiation OR Glandular differentiation OR Trophoblastic differentiation dff OR Nested TCC or microcystic TCC 87

88 Transitional Cell Carcinoma >90% are TCC Gross appearance varies Some are flat Some are papillary Under microscope most look papillary 88

89 Transitional Cell Carcinoma Classified by growth pattern Papillary tumors Wart-like lesion Attached to a stalk Non-papillary tumors (flat) Not common More invasive 89

90 Transitional Cell Carcinoma Only information is CIS of bladder, then code 8120 transitional cell Multi-focal with both flat TCC and papillary TCC, then code 8130 but in CS code flat (06) Working with CS Task Force 90

91 Breast 91

92 Overview Mucinous/colloid carcinoma Duct carcinoma and DCIS Lobular carcinoma and LCIS Tables 1, 2, and 3 92

93 When To Code Mucinous Mucinous, mucin producing = colloid Not same rules as colon % mucin not important 93

94 Duct Carcinoma, NOS Largest group of breast cancers Glandular (adeno)carcinoma that originates in the breast ducts Does not meet criteria for subtyping Lacks specific features 94

95 DCIS Multi-focal Multi-centric Diffuse 95

96 Duct carcinoma Lobular carcinoma 96

97 Lobular Carcinoma, NOS Specific type Tubulo-lobular = tubular and lobular DO use code for lobular + other (not duct) 97

98 LCIS Usually found by chance Mammograms usually negative Originates in lobules (milk production) Often bl bilaterall 98

99 Table 1 - Intraductal Code Type 8201 Cribriform 8230 Solid 8401 Apocrine 8500 Intraductal, NOS 8501 Comedo 8503 Papillary 8504 Intracystic carcinoma 8507 Micropapillary/Clinging 99

100 Table 2 - Duct Code Type 8022 Pleomorphic carcinoma 8035 Carcinoma with osteoclast-like giant cells 8500 Duct, NOS 8501 Comedocarcinoma 8502 Secretory carcinoma of breast 8503 Intraductal papillary adenocarcinoma with invasion 8508 Cystic hypersecratory carcinoma 100

101 Table 3 Required Any combination excluding lobular and duct histologies from Tables 1 and 2 Combined with Other than duct and lobular Term Adenoca with mixed subtypes Code 8255/3 101

102 Table 3 Required Intraductal and two or more of the histologies in Column 2 Combined with Cribriform Solid Apocrine Papillary Micropapillary Clinging Term Intraductal mixed with other types of carcinoma Code 8523/2 102

103 Biggest Concern for MP Multiple intraductal and/or duct carcinomas are a single primary Note: Use Table 1 and Table 2 to identify intraductal and duct carcinomas 103

104 Biggest Concern Histology Code 8530 (inflammatory ca) only when the final dx of the path report specifically states inflammatory ca Note: Record dermal lymphatic invasion in Collaborative Staging 104

105 105

106 Combination Codes H15 Two or more specific ducts H16 Lobular and duct 8522 H17 Duct and other 8523 No more duct histology after H17 106

107 Lobular with other carcinoma Code 8524 (lobular mixed with other types of carcinoma) when the tumor is lobular (8520) and any other carcinoma. 107

108 H19 The Others Code 8255 (adenocarcinoma with mixed subtypes) for multiple histologies that do not include duct or lobular (8520). 108

109 109

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