Summary Stage 2018: Lymphoma
Summary Stage 2018
Notes
**Lymphoma**
9590, 9596-9663, 9673-9699, 9702-9719, 9725-9726, 9735, 9737-9738, 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:** The following sources were 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:** 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:** See the following chapters for the listed histologies
* C441, C690, C695-C696 (9590-9699, 9702-9719, 9725-9726, 9734-9738, 9823, 9826-9827, 9930): *Lymphoma Ocular Adnexa*
* C440, C442-C449, C510, C609, C632 (9597, 9680, 9708-9709, 9712, 9718-9719, 9726): *Primary Cutaneous Lymphomas*
* C700, C710-C719 (9680, 9699, 9700-9715): *Brain*
* C701, C709, C720-C725, C728-C729 (9680, 9699, 9700-9715): *CNS Other*
* C751-C753 (9680, 9699, 9700-9715): *Intracranial Gland*
* 9591 and Schema Discriminator 1: 1, 2 (C000-C440, C442-C689, C691-C694, C698-C809): *HemeRetic*
**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:** 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:** If there is peripheral blood or bone marrow involvement, code 7.
| 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 |
(1) Fritz AG, Ries LAG (eds). **SEER Extent of Disease 1988: Codes and Coding Instructions (3rd Edition, 1998)**, National Cancer Institute, NIH Pub. No. 98-2313, Bethesda, MD, 1998
(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
