Summary Stage 2018: Lymphoma
Summary Stage 2018
Notes
**Lymphoma**
9590, 9596-9663, 9673-9699, 9702-9719, 9725-9726, 9735, 9737-9738, 9823, 9826-9827 (varying primary sites and histologies)
* C700-C729, C751-C753: 9690, 9719 (2018-2022 only) (See Note 2)
C000-C440, C442-C689, C691-C694, C698-C809: 9591 and Schema Discriminator 1: 3, 9
*See Summary Stage 2018 Manual, Appendix III for a detailed listing of primary site/histology combinations for this schema*
**Note 1:** **Sources used in the development of this chapter**
* SEER Extent of Disease 1988: Codes and Coding Instructions (3rd Edition, 1998) (https://seer.cancer.gov/archive/manuals/EOD10Dig.3rd.pdf)
* SEER Summary Staging Manual-2000: Codes and Coding Instructions (https://seer.cancer.gov/tools/ssm/ssm2000/)
* Collaborative Stage Data Collection System, version 02.05: https://cancerstaging.org/cstage/Pages/default.aspx
* Chapter 79 *Hodgkin and Non-Hodgkin Lymphomas*, in the AJCC Cancer Staging Manual, Eighth Edition (2017) published by Springer International Publishing. Used with permission of the American College of Surgeons, Chicago, Illinois.
* Chapter 80 *Pediatric Hodgkin and Non-Hodgkin Lymphomas*, in the AJCC Cancer Staging Manual, Eighth Edition (2017) published by Springer International Publishing. Used with permission of the American College of Surgeons, Chicago, Illinois.
**Note 2:** **Histologies moved to Brain, CNS, Intracranial Gland**
* For the histologies listed below, these have moved to the Brain, CNS Other and Intracranial Gland Summary Stage chapters starting with 2023 diagnoses.
* If you have one of these cases for 2018-2022, then this is the appropriate chapter. If you have one of these cases diagnosed on January 1, 2023, forward, see the appropriate chapter
* 9690, 9719
* C700, C710-C719: *Brain*
* C701, C709, C720-C725, C728-C729: *CNS Other*
* C751-C753: *Intracranial Gland*
**Note 3:** **Other Summary Stage chapters with the listed histologies**
* **Brain**: C700, C710-C719 (9680, 9699, 9702-9715)
* **CNS Other**: C701, C709, C720-C725, C728-C729 (9680, 9699, 9702-9715)
* **Heme Retic**: 9591 and Schema Discriminator 1: 1, 2 (C000-C440, C442-C689, C691-C694, C698-C809)
* **Intracranial Gland**: C751-C753 (9680, 9699, 9702-9715)
* **Lymphoma Ocular Adnexa**: C441, C690, C695-C696 (9590-9699, 9673-9699, 9702-9719, 9725-9726, 9734-9738, 9823, 9826-9827, 9930)
* **Primary Cutaneous Lymphoma**: C440, C442-C449, C510, C609, C632 (9597, 9680, 9708-9709, 9712, 9718-9719, 9726): *Primary Cutaneous Lymphomas*
**Note 4:** **Chapter includes the preferred terms based on the *2017 WHO Classification of Haematopoietic and Lymphoid Tissues***
9590 Malignant lymphoma, NOS
9591 Non-Hodgkin lymphoma, NOS
9596 B-cell lymphoma, unclassifiable, with features intermediate between DLBCL and classic Hodgkin lymphoma
9597 Primary cutaneous follicle centre lymphoma
9650 Classical Hodgkin lymphoma
9651 Lymphocyte-rich classic Hodgkin lymphoma
9652 Mixed cellularity classic Hodgkin lymphoma
9653 Lymphocyte-depleted classic Hodgkin lymphoma
9659 Nodular lymphocyte predominant Hodgkin lymphoma
9663 Nodular sclerosis classic Hodgkin lymphoma
9673 Mantle cell lymphoma
9678 Primary effusion lymphoma
9679 Primary mediastinal (thymic) large B-cell lymphoma
9680 Diffuse large B-cell lymphoma (DLBCL)
9687 Burkitt lymphoma
9688 T-cell/histiocyte-rich large B-cell lymphoma
9689 Splenic marginal zone lymphoma
9690 Follicular lymphoma (except C700-C729, C751-C753 for 1/1/2023+)
9691 Follicular lymphoma, grade 2
9695 Follicular lymphoma, grade 1
9698 Follicular lymphoma, grade 3
9699 Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma)
9702 Peripheral T-cell lymphoma, NOS
9705 Angioimmunoblastic T-cell lymphoma
9708 Subcutaneous panniculitis-like T-cell lymphoma
9709 Primary cutaneous peripheral T-cell lymphomas
9712 Intravascular large B-cell lymphoma
9714 Anaplastic large cell lymphoma, ALK-positive
9715 Anaplastic large cell lymphoma, ALK-negative (2021+ only)
9716 Hepatosplenic T-cell lymphoma
9717 Enteropathy-associated T-cell lymphoma
9718 Primary cutaneous anaplastic large cell lymphoma
9719 Extranodal NK/T-cell lymphoma, nasal type (except C700-C729, C751-C753 for 1/1/2023+)
9725 Hydroa vacciniforme-like lymphoma (2018-2020 only, nonreportable as of 2021)
9726 Primary cutaneous gamma-delta T-cell lymphoma (2018-2020 only, see code 9687/3 for 2021+)
9735 Plasmablastic lymphoma
9737 ALK-positive large B-cell lymphoma
9738 HHV8-positive DLBCL, NOS
9766 Lymphomatoid granulomatosis grade 3 (2021+ only)
9823 Chronic lymphocytic leukemia (CLL)/small lymphocytic lymphoma
9826 Burkitt cell leukemia (2018-2020 only, see code 9687/3 for 2021+)
9827 Adult T-cell leukemia/lymphoma
**Note 5:** **Lymph node involvement**
* Any mention of the terms including fixed, matted, mass in the hilum, mediastinum, retroperitoneum, and/or mesentery, palpable, enlarged, shotty, lymphadenopathy are all regarded as involvement for lymphomas when determining appropriate code.
**Note 6:** **Peripheral Blood or Bone Marrow**
* If there is peripheral blood or bone marrow involvement, code 7.
**Note 7:** **Splenic involvement**
* Splenic involvement is based on splenomegaly and FDG-PET or CT scans that state diffuse uptake, solitary mass, miliary lesions, or enlargement of greater than 13 cm
* A physician’s statement of splenomegaly may be used
* FDG uptake in the spleen that is not diffuse is not enough to code as splenic involvement
**Note 8:** **Bilateral sites** Code 7 for involvement of bilateral sites (i.e., breast, eye, kidney, etc.).
* *Example*: Patient with extranodal non-Hodgkin's Lymphoma, involving bilateral choroids, (single focus both sites), and no lymph node involvement.
* Code 7 for bilateral involvement of choroids (eye)
**Note 9:** **Not applicable codes**
* Codes 0, 3, and 4 are not applicable for this chapter.
| SS2018 |
Description |
| 1 |
**Localized only**
Nodal Lymphomas
- Single lymph node region involved
- Involvement of multiple nodal chains in the SAME lymph node region
Extranodal Lymphomas
- Single extralymphatic site
+ WITHOUT nodal involvement (see code 2 for WITH nodal involvement)
- Multifocal involvement of one extralymphatic organ/site (EXCEPT multifocal lung involvement or any liver involvement, see code 7)
+ WITHOUT nodal involvement (see code 7 for WITH nodal involvement) |
| 2 |
**Regional by direct extension only**
Bulky disease present
Nodal Lymphomas
- Two or more lymph node regions involved SAME side of diaphragm
- Contiguous extension between extralymphatic sites and regional nodes
+ WITH or WITHOUT involvement of other nodal regions on SAME side of diaphragm
Extranodal Lymphomas
- Localized involvement of a single extralymphatic organ/site
+ WITH involvement of its regional lymph node(s) OR
+ WITH involvement of other lymph node(s) on the SAME side of the diaphragm |
| 7 |
**Distant site(s)/lymph node(s) involved**
- Distant involvement
+ Diffuse or disseminated involvement of ONE OR MORE extralymphatic organ(s)/site(s) WITH or WITHOUT nodal involvement
+ Involvement of isolated extralymphatic organ in absence of involvement of adjacent lymph nodes, but in conjunction with disease in distant sites
+ Involvement of lymph node regions on BOTH sides of the diaphragm WITH or WITHOUT spleen involvement
+ Involvement of lymph node regions ABOVE the diaphragm WITH spleen involvement
+ Multifocal involvement of one extralymphatic organ/site WITH nodal involvement
+ Noncontiguous extralymphatic organ involvement in conjunction with nodal disease (two or more sites involved)
- Distant metastasis, NOS
+ Blood/peripheral blood
+ Bone marrow
+ Cerebrospinal fluid (CSF)
+ Liver
+ Lung (other than by direct extension in code 2) |
| 9 |
Unknown if extension or metastasis |
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(2) Young JL Jr, Roffers SD, Ries LAG, Fritz AG, Hurlbut AA (eds.). **SEER Summary Staging Manual-2000: Codes and Coding Instructions**, National Cancer Institute, NIH Pub. No. 01-4969, Bethesda, MD, 2001.
(3) Collaborative Stage Work Group of the American Joint Committee on Cancer. **Collaborative Stage Data Collection System User Documentation and Coding Instructions, version 02.05**. American Joint Committee on Cancer (Chicago, IL)
(4) Gress, D.M., Edge, S.B., Gershenwald, J.E., et al. **Principles of Cancer Staging**. In: Amin, M.B., Edge, S.B., Greene, F.L., et al. (Eds.) AJCC Cancer Staging Manual. 8th Ed. New York: Springer; 2017
(5) **Introduction to Hematologic Malignancies**. In: Amin, M.B., Edge, S.B., Greene, F.L., et al. (Eds.) AJCC Cancer Staging Manual. 8th Ed. New York: Springer; 2017
(6) Zelenetz, A.D., Jaffe, E.S., Leonard, J.P., et al. **Hodgkin and Non-Hodgkin Lymphomas**. In: Amin, M.B., Edge, S.B., Greene, F.L., et al. (Eds.) AJCC Cancer Staging Manual. 8th Ed. New York: Springer; 2017
(7) Link, M.P., Jaffe, E.S., Leonard, J.P. **Pediatric Hodgkin and Non-Hodgkin Lymphomas**. In: Amin, M.B., Edge, S.B., Greene, F.L., et al. (Eds.) AJCC Cancer Staging Manual. 8th Ed. New York: Springer; 2017
