Summary Stage 2018: Lymphoma Ocular Adnexa

Summary Stage 2018

Notes

**Lymphoma Ocular Adnexa** 9590-9699, 9702-9719, 9725-9726, 9734-9738, 9823, 9826-9827, 9930 (C441, C690, C695-C696) **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 71 *Ocular Adnexal Lymphoma*, 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:** **Not applicable codes** * Code 0 is not applicable for this chapter. **Note 3:** **Ocular adnexal lymphomas** * Ocular adnexal lymphomas (OAL) originate in conjunctiva, eyelids, lacrimal gland, lacrimal drainage apparatus, and other orbital tissues surrounding the eye. * This chapter should not be used for secondary lymphomatous involvement of ocular adnexa or for intraocular lymphomas. **Note 4:** **Peripheral blood or bone marrow involvement** * If there is peripheral blood or bone marrow involvement, code 7. **Note 5:** **The preferred histology terms in this schema are based on the 2024 WHO Classification of Tumours, Haematolymphoid Tumours, 5th edition.** * 9590: Malignant lymphoma, NOS * 9591: Malignant lymphoma, non-Hodgkin, NOS * 9596: Mediastinal grey zone lymphoma (MGZL) * 9597: Primary cutaneous follicle centre lymphoma (PCFCL) * 9650: Classic Hodgkin lymphoma (CHL), NOS * 9651: Classic Hodgkin lymphoma, lymphocyte-rich (LR-cHL) * 9652: Classic Hodgkin lymphoma, mixed cellularity (MC-cHL) * 9653: Classic Hodgkin lymphoma, lymphocyte-depleted, NOS (LD-cHL) * 9659: Nodular lymphocyte predominant Hodgkin lymphoma (NLPHL) * 9663: Classic Hodgkin lymphoma, nodular sclerosis, NOS (NSCHL) * 9673: Mantle cell lymphoma (MCL) * 9678: Primary effusion lymphoma (PEL) * 9679: Primary mediastinal large B-cell lymphoma (PBML/PMBCL) * 9680: Diffuse large B-cell lymphoma (DLBCL) * 9687: Burkitt lymphoma (BL), NOS * 9688: T-cell/histiocyte-rich large B-cell lymphoma (THRLBCL) * 9689: Splenic marginal zone lymphoma (SMZL) * 9690 Follicular lymphoma (FL), NOS (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: Nodal T follicular helper cell lymphoma, angioimmunoblastic type * 9708: Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) * 9709: Primary cutaneous peripheral T-cell lymphomas (pcCTCL) * 9712: Intravascular large B-cell lymphoma (IVLBCL) * 9714: Anaplastic large cell lymphoma, ALK-positive (ALCL) * 9715: Anaplastic large cell lymphoma, ALK-negative (ALK-ALCL) (2021+ only) * 9716: Hepatosplenic T-cell lymphoma (HSTCL) * 9717: Intestinal T-cell lymphoma, NOS (ITCL-NOS) * 9718: Primary cutaneous anaplastic large cell lymphoma (C-ALCL) * 9719: Extranodal NK/T-cell lymphoma (ENKTL) (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 (PCGD-TCL) (2018-2020 only, see code 9687/3 for 2021+) * 9734: Extramedullary plasmacytoma (EMP) * 9735: Plasmablastic lymphoma (PBL) * 9737: ALK-positive large B-cell lymphoma (ALK+LBCL) * 9738: KSHV/HHV8-positive diffuse large B-cell lymphoma * 9823: Chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) * 9826: Burkitt cell leukemia (2018-2020 only, see code 9687/3 for 2021+) * 9827: Adult T-cell leukemia/lymphoma (ATLL) * 9930: Myeloid Sarcoma

NAACCR Item

NAACCR #764
SS2018 Description
1 **Localized only (localized, NOS)** - Lymphoma involving the conjunctiva alone WITHOUT or UNKNOWN eyelid or orbital involvement - Lymphoma with orbital involvement WITH or WITHOUT conjunctival involvement - Lymphoma with preseptal eyelid involvement WITH or WITHOUT orbital or conjunctival involvement
2 **Regional by direct extension only** - Orbital adnexal lymphoma AND extraorbital lymphoma extending beyond the orbit to adjacent structures + Bone + Brain + Maxillofacial sinuses
3 **Regional lymph node(s) involved only** - Cervical - Parotid - Preauricular - Regions draining the ocular adnexal structures - Submandibular - Regional lymph node(s), NOS + Lymph node(s), NOS
4 **Regional by BOTH direct extension AND regional lymph node(s) involved** Codes (2) + (3)
7 **Distant site(s)/lymph node(s) involved** - Distant sites + Non-contiguous involvement of other extranodal sites including, but not limited to * Breast * Gastrointestinal tract * Kidney * Liver * Lung * Parotid gland * Salivary gland * Spleen * Submandibular gland - Distant lymph node(s), NOS + Diffuse OR disseminated involvement of peripheral and central lymph node regions + Mediastinum - Distant metastasis, NOS + Blood/peripheral blood + Bone marrow + Distant metastasis WITH or WITHOUT distant lymph node(s)
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) Heegaard, S., Finger, P.T., et al. **Ocular Adnexal Lymphoma**. In: Amin, M.B., Edge, S.B., Greene, F.L., et al. (Eds.) AJCC Cancer Staging Manual. 8th Ed. New York: Springer; 2017