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) * *See Summary Stage 2018 Manual, Appendix III for a detailed listing of primary site/histology combinations for this schema* 9591 and Schema Discriminator 1: 3, 9 (C000-C440, C442-C689, C691-C694, C698-C809) **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:** See the following schemas 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 3:** 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 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 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 4:** 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 5:** If there is peripheral blood or bone marrow involvement, code 7.

NAACCR Item

NAACCR #764
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 * OR nodes ABOVE the diaphragm involved 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