LYMPHOMA, MADE EASIER

1
LYMPHOMA, MADE EASIER

• Cynthia Carlisle, RHIT, CTR

2
General Rule of Thumb

• Code to the Site
• Nodal Lymphomas
• C77._
• Extra-Nodal Lymphomas
• Code to the site (i.e., C16._, C18._, C50._)
• Stage to the Disease
• Nodal or Extra Nodal
• Use staging scheme for Lymphoma
• Not paired Site!
• Even if bilateral lymph node involvement
  documented.

3
Malignant Lymphoma

• Malignant Lymphoma (ML) - an umbrella term
  covering both Hodgkin lymphoma and non-Hodgkin
  lymphoma
• Hodgkin Lymphoma (HL)
• Nodular Sclerosing (NS)
• Lymphocyte Predominant (LP)
• Lymphocyte Depletion (LD)
• Mixed Cellularity (MC)
• Non-Hodgkin Lymphoma (NHL)
• About 3 times more common than HL
• Cell types nodular or diffuse
• Classifications
• Lymphocytic (well-differentiated and poorly
  differentiated)
• Histiocytic
• Mixed lymphocytic and histiocytic
• Undifferentiated

4
Malignant Lymphoma

• Microglioma an intracranial tumor of microglial
  cell origin similar to reticulum cell sarcoma.
• Mycosis fungoides malignant lymphoma arising in
  the skin T-cell origin spreads to viscera.
• Sezarys syndrome mycosis fungoides with
  malignant lymphocytes in the peripheral blood.
• Brill Symmers Disease an infrequently used term
  for giant follicular lymphoma (a
  well-differentiated small lymphocytic lymphoma).

5
Site Codes for Lymphoma

• Code as an extra-nodal site when
• There is no nodal involvement of any kind
• OR
• It is stated in the medical record that the
  origin was an extra-nodal site.

6
Malignant LymphomaPrimary Sites

• Nodal Lymphoma
• Lymph nodes
• Spleen
• Thymus
• Lymphoid nodules in the appendix
• Peyers Patches
• Waldeyers Ring

• Extra-nodal Lymphoma
• Stomach
• Small Intestine
• Uterus
• Bone
• Brain
• Breast
• Large intestine

Note Extra-nodal lymphomas may have a better
prognosis. Extra-nodal Hodgkin Lymphoma is
uncommon.
7
Regional Lymph Nodes

• Above the Diaphragm
• Head, Face, Neck (C77.0)
• Intra-thoracic (C77.1)
• Axilla or Arm (C77.3)
• Below the Diaphragm
• Intra-abdominal (C77.2)
• Inguinal Region (C77.4)
• Pelvis (C77.5)
• Multiple Regions (C77.8)
• Lymph Node, NOS (C77.9)

8
Lymph Node Chains

• Above the Diaphragm
• Waldeyers ring
• Tonsils, adenoids (nasopharynx), lingual tonsils
• Cervical (neck)
• Occipital
• Preauricular
• Submental
• Submandibular
• Internal Jugular
• Intraclavicular
• Supraclavicular (scalene)
• Axillary, pectoral
• Mediastinal
• Peritracheal
• Thymic region
• Hilar
• Epitrochlear, brachial

• Below the Diaphragm
• Upper abdomen
• Splenic hilar
• Celiac
• Porta hepatis
• Lower abdomen
• Iliac
• Para-aortic
• Retroperitoneal
• Mesenteric
• Abdominal, NOS
• Iliac
• Inguinal
• Femoral
• Popliteal
• Spleen

9
Always Document the Primary Site

• Physician statement or final diagnosis
• Especially when there is involvement of both an
  extra-nodal site and lymph nodes
• Is there ever a time to use C80.9 (for
  Lymphomas)?!
• The primary site is not necessarily the site of
  the biopsy.

10
Site CodesExtra-Nodal vs. Stage IV

• Site of Origin
• Stomach, Colon, Brain, Uterus
• Most likely extra-nodal
• Bone, Lung
• Most likely Stage IV
• Liver, Bone Marrow
• Always Stage IV

• Number of Sites
• One Extra-nodal
• Many or Diffuse
• Stage IV

11
Extra Nodal Lymphoma
Usually

• Localized, solitary involvement of an
  extra-lymphatic site

12
Distinguishing Extra-Nodal Disease from Stage IV
Nodal Disease

• Determine

Site of Origin
Number of Sites Involved
13
Histology of Malignant Lymphoma
Non-Hodgkin Lymphoma
Hodgkin Lymphoma

• Staging the same for both Extra-Nodal and Nodal
  Lymphomas

14
Rye Classification for Hodgkin Lymphoma

• This system found that the different cellular
  subtypes had different prognosis
• Reed-Sternberg cells are diagnostic of Hodgkin
  Lymphoma because of their specific morphologic
  characteristics.

• Most Favorable
• Lymphocyte predominant NOS, Diffuse, Nodular
• Favorable
• Nodular sclerosis NOS, Cellular Phase, HL NS,
  Lymphocyte Predominance, HL NS Mixed Cellularity,
  HL NS Lymphocytic Depletion
• Guarded
• Mixed Cellularity NOS
• Least Favorable
• Lymphocyte Depletion NOS, Diffuse Fibrosis,
  Reticular

15
Reed-Sternberg Cells

• The nodules contain numerous cells (mostly
  lymphocytes) and two distinct types of malignant
  cells.
• One of these malignant cells, the Reed-Sternberg
  cell, is large, frequently binuclear or
  multinuclear.
• Reed-Sternberg cells are infrequent but increase
  in as the disease progresses.
• Its presence is necessary for the diagnosis of
  Hodgkin's disease but is not specific as similar
  cells can be seen in other conditions such as
  infectious mononucleosis. Other features
  therefore have to be present in addition to the
  Reed Sternberg cell for the diagnosis of
  Hodgkin's disease

16
Major Cellular Classifications for NHL

• Nodular/follicular
• Diffuse
• Histiocytic
• Lymphocytic (poorly differentiated and well
  differentiated)
• Mixed

17
Working Formulation for NHL

• Developed by NCR in 1982 as a method to translate
  among recognized classification systems for NHL
• i.e., Rappaport, Dorfman, Lukes and Collins, Kiel
  British.
• Major groups are identified by letters (A-J) and
  grouped according to prognosis.
• Cell types categorized as unclassified by the
  Working Formulation include the non-specific
  terms malignant lymphoma, non-hodgkin lymphoma,
  diffuse malignant lymphoma, nodular or follicular
  malignant lymphoma, and cutaneous lymphoma.

18
Working Formulation for NHL

• Low Grade (most favorable)
• Small Lymphocytic
• Follicular, small cleaved cell
• Follicular, mixed small cleaved and large cell
• Intermediate Grade
• Follicular, large cell
• Diffuse, small cleaved cell
• Diffuse, mixed small and large cell
• Diffuse, large cell
• High Grade (least favorable)
• Large cell immunoblastic
• Lymphoblastic
• Small noncleaved cell

19
Guidelines

• Differences in histology refer to differences in
  the first 3 digits of the ICD-O morphology code.
• A simultaneous diagnosis of malignant lymphocytic
  lymphoma (small cell type) and chronic
  lymphocytic leukemia (CLL) is coded to CLL.
• For lymphomas, information on T-cell or B-cell
  has precedence over information on grading or
  differentiation.

20
Guidelines, cont.

• The term phenotype, as in B-cell phenotype,
  indicates the type or origin of the tumor. This
  information can be used to assign the 6th digit
  B- or T- cell code.
• For lymphomas, do not code the descriptions high
  grade, low grade, or intermediate grade in
  the 6th digit grade or differentiation field.
  These terms refer to categories in the Working
  Formulation and NOT to histologic grade.
• Information on T-cell, B-cell, or null cell for
  lymphomas and leukemias has precedence over the
  information on grade/differentiation. If the
  grade/differentiation is stated (moderately
  differentiated, poorly differentiated, well
  differentiated) and the cell type is not, use the
  grade/differentiation information.

21
Guidelines, cont.

• Most lymphoblastic lymphomas are T-cell. With
  the exception of the immunoblastic large cell
  lymphomas and the mixed small and large cell
  diffuse lymphoma, most of the rest are B-cell.
• Lymphomas may be classified by the Rappaport or
  Working Formulation. If both are used, the
  Working Formulation takes precedence.

22
Lymphoma/Hematopoietic

• Histology codes M-9590-9989
• Code to the more specific term
• Use the term that does not include NOS or
  synonyms
• Consult a medical advisor or pathologist (or in
  our case a medical oncologist)
• Cross-reference

23
Cross Reference Coding

• 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)

24
Cross Reference Coding

• B-cell chronic lymphocytic leukemia/ small
  lymphocytic lymphoma
• Malignant lymphoma, small lymphocytic
• 9670/3 cross-referenced to 9823/3
• IF
• Dx in blood or bone marrow code 9823/3
• Dx in tissue code 9670/3
• Dx in all the above code 9670/3

25
Cross Referenced Diseases

• Cross-referenced morphology codes should be
  aggregated for analysis unless you are requested
  to do otherwise.

26
Equivalent Lymphoma Terms

• Follicular
• Mantle cell
• Anaplastic large B-cell
• Mature T-cell, NOS

• Follicle center cell
• Mantle zone
• Diffuse large cell
• Peripheral T-cell

27
NHL Prognostic Factors
Better
Worse

• Follicular
• Small lymphocytic
• Asymptomatic
• Young
• Not extra-nodal

• Diffuse
• Large Cell
• B-symptoms
• Elderly
• Bulky mass
• GI tract or marrow involved

28
Coding Histology for NHL

• Do not code the descriptions
• -- Grade 1
• -- Grade 2
• -- Grade 3
• Grade for lymphomas refers to type or
  category from the pathologist not the grade
  of the tumor.

29
Coding Differentiation for NHL

• Terms like anaplastic or poorly
  differentiated are double coded
• Examples
• Lymphoma, large cell, anaplastic, NOS
• Code 9714/34
• Lymphoma, nodular, poorly differentiated
• Code 9591/33

30
Immunophenotype

• Code Description
• 5 T-Cell
• 6 B-Cell
• 7 Null Cell
• 8 NK Cell
• 9 Cell type not determined, not stated or not
  applicable

31
Coding Grade for NHL

• T-Cell and B-Cell
• Priority over grading or differentiation.
• Code any statement of T-cell or B-cell.
• Phenotype (B-Cell phenotype) use to assign
  B-Cell or T-Cell code.

32
Staging

• Guided by Histology Code

33
Common Metastatic Sites

• Lymphatic Spread
• Hodgkin Lymphoma
• Adjacent lymph node chains
• Non-Hodgkin Lymphoma
• Spreads irregularly

• Hematogenous Spread
• Bone marrow
• Lung parenchyma
• Pleura
• Liver
• Bone
• Skin
• Kidneys
• GI Tract

34
Extent of Disease Evaluation

• History, including presence and duration of
• Fevers unexplained fever with temperature above
  38C.
• Night sweats drenching sweats that require
  change of bedclothes.
• Weight loss unexplained weight loss of more
  than 10 of the usual body weight in the 6 months
  prior to diagnosis.
• NOTE Pruritus alone does not qualify a for a B
  classification, nor does alcohol intolerance,
  fatigue, or a short, febrile illness associated
  with suspected infections.

35
Extent of Disease Eval., cont.

• Physical Exam
• Involvement of lymph nodes matted nodes, fixed
  vs mobile, lymphadenopathy, enlarged, shotty
  nodes, palpable, enlarged, visible swelling
• A palpable mass anywhere in the body
• Enlarged abdominal organs distention,
  organomegaly, hepatomegaly, splenomegaly,
  hepatosplenomegaly/HSM
• Pharyngeal examination for tonsil or oropharynx
  involvement
• Neurologic exam

36
Extent of Disease Eval., cont.

• Imaging
• Key Information size and location of primary
  tumor, involvement of additional lymph node
  chains, involvement of distant sites or visceral
  organs.
• CXR Imaging, Lung, Bone, Brain, Liver or Spleen.
  Abdomen or Pelvis Whole Lung Tomogram Gallium
  Scan, Ultrasound, Lymphangiogram.

37
Extent of Disease Eval., cont.

• Tumor Markers
• DNA Studies
• C-myc DNA Amplifications juxtaposition of this
  chromosome with a heavy chain immunoglobulin
  occurs frequently in Burkitts lymphoma and other
  B-cell lymphomas, as well as breast cancer and
  acute lymphoblastic leukemia.
• bcl-2 Oncogene Analysis diagnostic method to
  differentiate B-cell and follicular types of
  lymphomas.
• Beta-2M (ß-2 Microglobulin) elevated levels
  present in lymphoproliferative disorders.
• TDT (Terminal-Deoxynucleotidal Transferase)
  differentiates lymphoblastic lymphomas from other
  non-Hodgkins lymphomas.
• Ferritin elevated levels present in
  lymphoproliferative disease may indicate
  Hodgkins lymphoma or leukemia.

38
Extent of Disease Eval., cont.

• Endoscopies
• Mediastinoscopy
• Other Endoscopy

39
Extent of Disease Eval., cont.

• Pathology
• Key Information Cell type, size of lesion/mass,
  presence of multiple involved nodes/areas,
  extension to adjacent tissues (organs, muscles,
  fascia), results of biopsies of distant sites.
• Cytology Reports
• Excision or Needle Biopsy
• Thoracentesis

40
Extent of Disease Eval., cont.

• Staging Laparotomy evaluation of the contents
  of the abdomen to determine extent of disease.
• Includes abdominal exploration, wedge and needle
  biopsies of the liver, multiple lymph node
  biopsies, bone marrow biopsy, and splenectomy.
• Diagnostic not surgical treatment!
• Splenectomy surgical removal of the spleen
• May occur as part of a full staging laparotomy or
  occasionally as a separate procedure.
• Unsuspected HL found in approx 25 of splenectomy
  specimens.
• Abdominal Washings
• Bone Marrow Biopsy/Aspiration aspiration of
  bone marrow cells to determine involvement.
• Optional in low stage cases.
• Bilateral bone marrow biopsies should be
  performed for higher stage and symptomatic cases.

41
Stage of Disease

• AJCC
• Stage I
• Stage II
• Stage III
• Stage IV

• Summary Stage
• Local (1)
• Regional (5)
• Distant (7)
• Distant (7)
• Do not use regional extension (Code 2) or
  regional lymph nodes (Code 3)

42
Criteria for TNM Clinical Stage

• Includes information from
• Medical history and physical examination
• Imaging of chest, abdomen, pelvis
• Blood chemistry panels
• Complete blood count
• Bone marrow biopsy/aspiration

43
Criteria for TNM Path. Stage

• Only patients who undergo a staging laparotomy
  with an explicit intent to assess the presence of
  abdominal disease or to define histologic
  microscopic disease extent in the abdomen.

44
AJCC Staging Stage I

• Involvement of a single lymph node region (I)
• Localized involvement of a single extralymphatic
  organ or site in the absence of any lymph node
  involvement (IE)
• rare in Hodgkin lymphoma

45
AJCC Staging Stage II

• Involvement of two or more lymph node regions on
  the same side of the diaphragm (II)
• Localized involvement of a single extralymphatic
  organ or site in association with regional lymph
  node involvement with or without involvement of
  other lymph node regions on the same side of the
  diaphragm (IIE)
• NOTE The of regions involved may be
  indicated by a subscript, i.e. II3.

46
AJCC Staging Stage III

• Involvement of lymph node regions on both sides
  of the diaphragm (III)
• May also be accompanied by extralymphatic
  extension in association with adjacent lymph node
  involvement (IIIE)
• Involvement of the spleen (IIIS)
• Involvement of spleen extralymphatic and spleen
  (IIIES)

47
AJCC Staging Stage IV

• Diffuse or disseminated involvement of one or
  more extralymphatic organs, with our without
  associated lymph node involvement
• Isolated extralymphatic organ involvement in the
  absence of adjacent regional lymph node
  involvement, but in conjunction with disease in
  distant site(s).
• Any involvement of the liver or bone marrow, or
  nodular involvement of the lung(s).

48
Differences in Stage

• Stage IIE NHL involving mediastinal lymph nodes
  with lung extension
• Stage IV NHL involving m mediastinal, hilar
  and axillary lymph nodes with multiple pulmonary
  nodules

49
Stage of Disease

• Special Note for Mycosis Fungoides
• AJCC use Non-Hodgkin Lymphoma schema
• Summary Stage 2000 use Mycosis Fungoides, page
  176

50
Staging Lymphoma

• Any mention of lymph nodes is indicative of
  involvement
• Palpable
• Rubbery
• Enlarged
• Shotty/matted
• Caution rock hard may indicate a carcinoma
  instead of a lymphoma

51
Staging Lymphomas
Definition of C77.8
Definition of Local Disease

• Lymph nodes of multiple regions

• Lymph nodes of
• one region

52
Staging Lymphomas

• Local OR Stage I
• NEVER
• Coded to C77.8

53
Spleen Involvement

• Can be considered clinically involved if
• Palpable splenomegaly
• AND
• Confirmed by imaging

54
Staging Lymphomas

• Bilateral nodal involvement
• Each side is considered a lymph node region.
• Example Bilateral axillary lymph node
  involvement is considered two regions
  (Regional, NOS Code 5)

Lf Axillary Lymph Nodes
Rt Axillary Lymph Nodes
55
Staging Lymphomas

• Bilateral paired organ involvement
• With NO other involvement
• AJCC Stage 1E
• Summary Stage 2000 Stage Local
• Example Bilateral lymphoma of the eye
• AJCC 1E SS2000 Local (1)

56
Staging Lymphomas

• Involvement of soft tissue adjacent to a lymphoma
  site does not alter the staging
• Example Lymphoma of the 3rd lumbar vertebra
  with extension into the soft tissues surrounding
  the bone but without LN involvement.
• AJCC IE SS2000 Local

57
Lymphomas and Collaborative Staging

• Hodgkin and Non-Hodgkin Lymphomas of All Sites
  (excl. Mycosis Fungoides and Sezary Disease)
• (ICD-O-3 M-959-972 EXCEPT 9700/3 and 9701/3)
• CS Tumor Size 888CS ExtensionCS
  TS/Ext-EvalCS Lymph Nodes 88CS Reg Nodes Eval
  9Reg LN Pos 99Reg LN Exam 99CS Mets at
  DX 88CS Mets Eval 9CS Site-Specific Factor
  1Associated with HIV/AIDSCS Site-Specific
  Factor 2Systemic Symptoms at DiagnosisCS
  Site-Specific Factor 3IPI ScoreCS Site-Specific
  Factor 4 888CS Site-Specific Factor 5 888CS
  Site-Specific Factor 6 888Histologies for
  Which AJCC Staging Is Not Generated NAAJCC
  Stage NASEER Summary StageExtension Stage
  Table

58
Lymphoma and Collaborative Staging

• Mycosis Fungoides and Sezary Disease of Skin,
  Vulva, Penis, Scrotum
• CS Tumor SizeCS ExtensionCS TS/Ext-EvalCS
  Lymph NodesCS Reg Nodes EvalReg LN PosReg LN
  ExamCS Mets at DXCS Mets EvalCS Site-Specific
  Factor 1Peripheral Blood InvolvementCS
  Site-Specific Factor 2 888CS Site-Specific
  Factor 3 888CS Site-Specific Factor 4 888CS
  Site-Specific Factor 5 888CS Site-Specific
  Factor 6 888Histology Exclusion TableAJCC
  StageSEER Summary Stage

59
Lymphoma Surgery Codes(C77._)

• Surgery Field
• Surgical Approach
• Surgery of Primary Site
• Surgical Margins

• Code to
• Usually 5 (open, not assisted)
• 10 Most frequently used for biopsy of less than
  a full chain
• 7 usually not evaluable

60
Lymphoma Surgery Codes(C77._)

• Surgery Field
• Scope of Reg. Lymph Nodes
• of Regional Lymph Nodes
• Surgery Other
• Reconstruction

• Code to
• Always code 9
• Always code 99
• Code as appropriate
• Always code 9

61
Multiple Primaries Table

• Definitions of Single and Subsequent Primaries
  for Hematologic Malignancies
• Based on ICD-O-3 reportable malignancies.
• Effective with cases diagnosed beginning January
  1, 2001.

62
Determining Multiple Hematopoietic Primaries

• Two diagnoses prior to 01/01/2001
• USE ICD-O-2 table
• ROADS Appendix B
• TCR Cancer Reporting Handbook
• Two diagnoses after 01/01/2001
• Use ICD-O-3 table
• Hematopoietic Primaries Table

63
Determining Multiple Hematopoietic Primaries

• When was diagnosis made?
• To determine which rules to follow.
• Find appropriate ICD-O-3 Morphology codes for
  diagnoses in question.
• Using rules, determine if case is considered
  subsequent primary or progression of disease.

64
(No Transcript)
65
(No Transcript)
66
(No Transcript)
67
(No Transcript)
68
(No Transcript)