1. CSv2 for the Hematopoietic Neoplasms 1

2. 2 This includes five schemas …. Hematopoietic, Reticuloendothelial, Immunopro-liferative and Myeloproliferative neoplasms Myeloma and Plasma Cell Disorders Lymphoma Lymphoma, Ocular Adnexa Mycosis fungoides and Sézary syndrome 2

3. 3 CS Data Items new in 2010 are NA Data ItemCodeExplanation Grade Path ValueBlankNot applicable Grade Path SystemBlankNot applicable Lymph vascular invasion (LVI) 8Not applicable Mets at Dx-Bone8Not applicable Mets at Dx-Lung8Not applicable Mets at Dx-Brain8Not applicable Mets at Dx-Liver8Not applicable 3

4. Hematopoietic / Reticuloendothelial Neoplasms

5. Based primarily on histology 9733, 9740-9742, 9750-9758, 9760-9762, 9764-9769, 9800-9801, 9805-9809, 9811-9818, 9820, 9823 [C420, C421, or C424 ONLY], 9826, 9827 [C420, C421, or C424 ONLY], 9831-9837, 9840, 9860-9861, 9863, 9865-9867, 9869-9876, 9891, 9895-9898, 9910, 9920, 9930-9931, 9940, 9945-9946, 9948, 9950, 9960-9967, 9970, 9971, 9975, 9980, 9982-9987, 9989, 9991-9992 Note: AJCC does not define TNM staging for this site. 5

6. Heme Retic Schema 6

7. CS Extension Description 100Localized disease: (single/solitary/unifocal/isolated) may be coded for: Mast cell sarcoma (9740) Malignant histiocytosis (9750) Langerhans cell histiocytosis (9751) Histiocytic sarcoma (9755) Langerhans cell sarcoma (9756) Dendritic cell sarcoma (9757, 9758) Myeloid sarcoma (9930) 800Systemic disease All histologies including those in 100 999Unknown, cannot be assessed, not documented in record 7

8. 8 Remaining CS Data Elements All these fields are “not applicable” ▫ Tumor size=988 ▫ CS Lymph nodes=988 ▫ Reg LNs positive and Reg LNs examined=99 ▫ CS Mets at Dx=98 ▫ CS Eval fields (TS/Ext, Reg Nodes, Mets)=9 8

9. CS Site Specific Factor 1 - JAK-2 CodeDescription 000JAK-2 test result stated as negative 010JAK2 test performed, positive for mutation V617F in exon 14 020JAK2 test performed, positive for mutation of exon 12 800JAK2 test performed, positive for other specified mutation 810JAK2 test performed, positive for more than one mutation 850JAK2 test performed, positive NOS; specific mutation(s) not stated 888 OBSOLETE DATA CONVERTED V0200 See code 988: Not applicable for this schema 988 Not applicable: Information not collected for this case (May include cases converted from code 888 used in CSv1. If this item is required to derive T, N, M, or any stage, use of code 988 may result in an error.) 997Test ordered, results not in chart 998Test not done 999Unknown, Insufficient information, Not documented in record 9

10. Myeloma / Plasma Cell Disorders

11. 11

12. MyelomaPlasmaCell - Extension 12

13. Myeloma – Lymph Nodes 13

14. Myeloma – CS Mets at Dx – use 98 14

15. Other CS fields Not applicable Tumor size/Extent Eval = 9 CS Lymph Nodes Eval = 9 CS Mets Eval = 9 Regional Nodes Positive = 99 Regional Nodes Examined = 99 15

16. Myeloma – SSF1 JAK2 –Obsolete Use only code 988 16

17. Myeloma SSF2- Durie-Salmon Stage Note 1: Durie-Salmon staging applies to multiple myeloma (9732) only. For plasmacytomas, (9731 and 9734), use code 987 for not applicable. Note 2: The A and B subclassifications reflect the serum creatinine levels at diagnosis. If the A or B subclassification is not noted, use the NOS codes. Note 3: Only code the Durie-Salmon stage when the stage is documented in the medical record. Note 4: Code the stage as Durie-Salmon when the record states the stage, but does not identify the staging system. 17

18. New: Myeloma – SSF2 18

19. Myeloma SSF 3- MM Terminology Note 1: Descriptors for multiple myeloma (9732) only are collected. Code 987, not applicable, for histologies 9731 and 9734. Note 2: A number of terms are used to characterize early multiple myeloma at time of diagnosis. All these terms are reportable according to the new Hematopoietic and Lymphoid Neoplasms rules effective for cases diagnosed January 1, 2010 and later. Note 3: Code the terminology used by a physician at the time of diagnosis. The terminology may be taken from sections of the record other than a pathology report. Do not change the code in this Site Specific Factor if a term used at a later time indicates progression to a more aggressive disease course. 19

20. New: Myeloma – SSF 3 20

21. Hodgkin and Non-Hodgkin Lymphomas

22. Lymphatic vs Extralymphatic sites LymphaticExtralymphatic Lymph nodes (C77._) Thymus (C37.9) Spleen (C42.2) Peyer’s patches (C17.2) Nodules in the Appendix (C18.1) Waldeyer’s Ring (C14.2) ▫Pharyngeal tonsil ▫Tubal tonsils ▫Palatine tonsils ▫Lingual tonsils Stomach Small Intestine Gastrointestinal tract Brain Lung Any organ can be involved by lymphoma 22

23. 23 The Importance of the Diaphragm The diaphragm’s role in staging: ▫ The muscle that separates the chest (thoracic) cavity from the abdomen. ▫ Is the dividing point for staging in lymphomas (below and above, on both sides) The diaphragm is used in defining stage: ▫ Same side of the diaphragm ▫ Opposite side of the diaphragm ▫ Both sides of the diaphragm 23

24. 24 CS Extension Summary table Nodal/ Lymphatic Extranodal/ Extralymphatic Spleen (involvement) 100110120* 200210220, 230 300310320, 330 800 999 24 *Primary site=Spleen (C42.2), no other organs or lymph nodes involved Side of Diaphragm Same Both Both, metastatic Unknown

25. 25 CS Extension-Lymphatic 100 (Stage I) ▫ Primary site: Lymphatic ▫ Involvement of single lymph node region 200 (Stage II) ▫ Primary site: Lymphatic ▫ Two or more lymph node regions involved on same side of diaphragm 300 (Stage III) ▫ Primary site: Lymphatic ▫ Lymph node regions involvement on both sides of the diaphragm 25

26. 26 CS Extension-Extralymphatic 110 (Stage IE) ▫ Primary site: Extralymphatic organ ▫ Involvement of extralymphatic organ only 210 (Stage IIE) ▫ Primary site: Extralymphatic organ ▫ Lymph node involvement on same side of diaphragm 310 (Stage IIIE) ▫ Primary site: Extralymphatic organ ▫ Lymph node involvement on both sides of diaphragm 26

27. 27 CS Extension-Spleen 120 (Stage IS) ▫ Primary site: Spleen ONLY 220 (Stage IIS) ▫ Primary site: Spleen or lymphatic structure ▫ Spleen involved ▫ Lymph nodes below the diaphragm involved 230 (Stage IIES) ▫ Primary site: Extralymphatic organ ▫ Spleen involved ▫ Extralymphatic organ below the diaphragm involved ▫ Lymph nodes below the diaphragm involved/not involved 27

28. CS Extension-Spleen 320 (Stage IIIS) ▫ Primary site: Spleen or lymphatic structure ▫ Spleen involved ▫ Lymph nodes above diaphragm or on both sides of diaphragm involved 330 (Stage IIIES) ▫ Primary site: Extralymphatic organ ▫ Spleen involved ▫ Lymph nodes involved on opposite or both sides of the diaphragm 28

29. 29 CS Extension 800 (Stage IV) ▫ Lymphatic sites (including Spleen)  Metastatic sites (Bone marrow, Liver, Lung) ▫ Extralymphatic sites  Diffuse or disseminated (multifocal) involvement of one or more extralymphatic sites with or without lymph node involvement  Involvement of isolated extralymphatic organ in absence of involvement of adjacent lymph nodes, but in conjunction with disease in distant sites  Metastatic sites (Bone marrow, Liver, Lung) 999 (Unknown)

30. 30 CS TS/Ext Eval 30

31. 31 Remaining CS Data Elements All these fields are “not applicable” ▫ Tumor size=988 ▫ CS Eval fields (Reg Nodes, Mets)=9 ▫ CS Lymph nodes=988 ▫ Reg LNs positive and Reg LNs examined=99 ▫ CS Mets at Dx=98 31

32. 32 New Data Items Data ItemCodeExplanation Grade Path ValueBlankNot applicable Grade Path SystemBlankNot applicable Lymph vascular invasion (LVI) 8Not applicable Mets at Dx-Bone8Not applicable Mets at Dx-Lung8Not applicable Mets at Dx-Brain8Not applicable Mets at Dx-Liver8Not applicable 32

33. 33 SSF 1 and SSF 2 SSF 1 Associated with HIV/AIDS ▫ If there is no mention of AIDS in the chart and no evidence of HIV testing, code 999 for unknown SSF 2 Systemic symptoms at diagnosis ▫ Coding “none” if H&P, progress notes and consults make no mention of B symptoms ▫ B symptoms  Drenching Night sweats  Unexplained fever (above 38 degrees C)  Unexplained weight loss (greater than 10% loss of body weight in last six months before admission) ▫ Pruritis (alone it does not mean B symptoms) 33

34. 34 SSF 3-5 SSF 3 International Prognostic Index (IPI) ▫This SSF applies to B-cell lymphomas ▫Additional categories added (stated as “low” risk, stated as “intermediate” risk, etc.) ▫Criteria have been listed, DO NOT figure out index, only enter what has been documented SSF 4 Follicular Lymphoma International Prognostic Index (FLIPI) SSF 5 International Prognostic Score (IPS) ▫Used with Hodgkin Lymphoma 34

35. Lymphoma, Ocular Adnexa 35

36. 36

37. Mycosis fungoides Sézary syndrome

38. 38 Histology Mycosis Fungoides (9700) is the most common form of Cutaneous T cell lymphoma (CTCL) Sezary Syndrome (9701) is an aggressive leukemic form of CTCL Different staging system from other CTCL due to the prognosis of these diseases 38

39. 39 CS Extension 100 OBSOLETE (see codes 110-130) 110 Patches only with less than 10% of skin surface involved 120 Plaques, with or without patches, with less than 10% of skin surface involved 130 Plaques, papules, or eythematous patches (“plaque stage”); Limited plaques/patches; MFCG Stage I; Stated as T1 39

40. 40 CS Extension 200 OBSOLETE (see codes 210-240) 210 Patches only with more than 10% of skin surface involved 220 Plaques, with or without patches, with more than 10% of skin surface involved 230 Plaques, papules, or erythematous patches (“plaque stage”); Generalized plaques/patches; MFCG Stage II; Stated as T2 40

41. 41 CS Extension 250 Plaques, papules, or erythematous patches (“plaque stage”); body surface not stated 300 Skin involvement, NOS 500 OBSOLETE 550 One or more tumors (tumor stage) less than 1 cm, cutaneous tumors, size not stated 600 One or more tumors (tumor stage) greater than 1 cm 650 Stated as T3 41

42. 42 CS Extension 700 OBSOLETE 730 Generalized erythroderma (greater than 50% and less than 80%) 750 Generalized erythroderma (greater than or equal to 80%) 800 Stated as T4 950 No evidence of primary tumor 999 Unknown

43. 43 CS Lymph Nodes Lymph node staging is based on clinical and pathologic factors Lymph node biopsies only recommended on clinically abnormal nodes greater than 1.5 cm Clonality (negative, positive or unknown) will determine a, b or NOS Dutch grade system and NCI LN grade system are also included in lymph node definitions 43

44. 44 CS Mets at Dx Referred to as “Visceral Organ Involvement” instead of mets 00 No visceral organ involvement 05 Clinical confirmation only of Visceral (non cutaneous, extra nodal) involvement (except for Liver and Spleen imaging, see code 10) 10 Imaging confirmation of Spleen or Liver metastases 40 OBSOLETE 44

45. 45 CS Mets at Dx 45 Visceral (non cutaneous, extra nodal) involvement, pathologically confirmed, involvement by at least one organ outside the skin, nodes or blood or bone marrow 60 Carcinomatosis; Distant mets, NOS; MFCG Stage IV; Unknown if clinical or pathologic confirmation 70 Stated as M1 99 Unknown 45

46. 46 New Data Items Data ItemCodeExplanation Grade Path ValueBlankNot applicable Grade Path SystemBlankNot applicable Lymph vascular invasion (LVI)8Not applicable Mets at Dx-Bone0,1,9Applicable Mets at Dx-Lung0,1,9Applicable Mets at Dx-Brain0,1,9Applicable Mets at Dx-Liver0,1,9Applicable 46

47. 47 SSF1 Peripheral blood involvement A peripheral blood smear must be done to detect peripheral blood involvement The categories for peripheral blood involvement are: ▫B0 – no significant blood involvement ▫B1 – Low blood tumor burden ▫B2 – High blood tumor burden Clonality may also be assessed on blood samples. Record the B rating as stated by the physician. If counts or percentages of neoplastic cells, flow cytometry test results, and/or clonality test results are performed but a B rating is not stated by the physician, use code 090. 47

48. 48 Summary remarks for this schema Mycosis fungoides/ Sézary syndrome ▫Extension divides T1, T2 into ‘a’ and ‘b’ categories based on whether you have patches or plaques ▫Lymph nodes are divided into “a” and “b” categories for N1, N2 based on clonality (negative vs positive) ▫Peripheral blood involvement (SSF1) now a part of TNM stage group and is further divided into “a” and “b” categories for B0 and B1 based on clonality (negative vs positive) 48