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A lymphoma that starts in a lymph node is called nodal; one that starts in an organ is called extranodal. A substantial minority of non-Hodgkin lymphomas start outside the lymph nodes, and where it started often changes the cause, the treatment and the outlook more than the cell type does.
Lymphoid tissue is not confined to lymph nodes. It lines the gut, the airways, the salivary glands and the thyroid, and it accumulates wherever there is long-standing inflammation. A lymphoma arising in one of those places is a primary extranodal lymphoma, and the site is part of the diagnosis.
Four ways the site changes the disease:
It names the cause. Gastric marginal zone lymphoma follows Helicobacter pylori infection; lymphoma of the salivary gland follows Sjogren syndrome; lymphoma of the thyroid follows Hashimoto thyroiditis; enteropathy-associated T-cell lymphoma follows coeliac disease. Removing the cause can be the treatment: eradicating Helicobacter pylori puts most early gastric MALT lymphomas into lasting remission without any cancer treatment at all.
It changes the first treatment. Early extranodal marginal zone lymphoma of the orbit is treated with radiotherapy to a small field, or with an antibiotic; the same cells in a lymph node are not.
It creates sanctuaries. The brain, the vitreous and the retina, and the testis are immune-privileged: the immune system does not patrol them and most drugs do not reach them. WHO-HAEM5 grouped large B-cell lymphomas at those three sites into one entity in 2022 because they share a mutational profile and a habit of relapsing in each other. A lymphoma of the testis needs the other testis irradiated and the brain protected, or it comes back in one or the other.
It changes the emergencies. Lymphoma of the small bowel can perforate; lymphoma of the chest can obstruct the superior vena cava; lymphoma of the spine can compress the cord. Those are presentations of the site, not of the cell type.
The staging system counts extranodal disease twice over: the suffix E marks a single extranodal site reached by direct extension, stage IV marks diffuse involvement of an organ, and the number of extranodal sites is one of the five items in the International Prognostic Index.
Leukaemias, myeloma and MDS live in the marrow and blood; lymphomas grow in lymph nodes and spleen. The node stations are the disease map, not a route of spread, and staging counts them.
In lymphoma the node stations are the disease itself; staging (Ann Arbor / Lugano) counts how many regions and sides of the diaphragm are involved.
Same organ: High-grade B-cell lymphoma with MYC and BCL2 rearrangements (double-hit lymphoma), Mediastinal grey zone lymphoma, Primary effusion lymphoma, Plasmablastic lymphoma, T-cell/histiocyte-rich large B-cell lymphoma, EBV-positive diffuse large B-cell lymphoma, Primary large B-cell lymphoma of the testis, Gastric MALT lymphoma, Ocular adnexal MALT lymphoma, Extranodal NK/T-cell lymphoma, Adult T-cell leukaemia/lymphoma, ALK-positive anaplastic large cell lymphoma, ALK-negative anaplastic large cell lymphoma, Breast implant-associated anaplastic large cell lymphoma, Primary cutaneous anaplastic large cell lymphoma, Lymphomatoid papulosis, Mycosis fungoides, Enteropathy-associated T-cell lymphoma, Monomorphic epitheliotropic intestinal T-cell lymphoma, Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma), Splenic marginal zone lymphoma, Nodal marginal zone lymphoma, Primary cutaneous marginal zone lymphoma, Primary cutaneous follicle centre lymphoma, Sezary syndrome, Nodal T-follicular helper cell lymphoma, angioimmunoblastic type (angioimmunoblastic T-cell lymphoma), Hepatosplenic T-cell lymphoma, Intravascular large B-cell lymphoma, Lymphomatoid granulomatosis, T-cell prolymphocytic leukaemia, Splenic B-cell lymphoma/leukaemia with prominent nucleoli (formerly B-cell prolymphocytic leukaemia and hairy cell leukaemia variant), T-cell large granular lymphocytic leukaemia, Mixed-phenotype acute leukaemia, Myeloid leukaemia of Down syndrome, Burkitt leukaemia, Marginal zone lymphoma, Cutaneous T-cell lymphoma (mycosis fungoides and Sezary syndrome), Primary mediastinal (thymic) large B-cell lymphoma, Leukaemia (all types), Acute myeloid leukaemia, Acute lymphoblastic leukaemia, Chronic lymphocytic leukaemia, Chronic myeloid leukaemia (CML), Diffuse large B-cell lymphoma, Follicular lymphoma, Hodgkin lymphoma, Mantle cell lymphoma, Multiple myeloma, Myelodysplastic syndromes / neoplasms (MDS), Myeloproliferative neoplasms (PV, ET, myelofibrosis), Polycythaemia vera (PV), Essential thrombocythaemia (ET), Waldenström macroglobulinaemia, Hairy cell leukaemia, Peripheral T-cell lymphomas (including cutaneous T-cell lymphoma), Blastic plasmacytoid dendritic cell neoplasm (BPDCN), Burkitt lymphoma, HIV-associated (AIDS-related) lymphomas, Chronic myelomonocytic leukaemia and MDS/MPN overlap neoplasms, Systemic mastocytosis, Erdheim-Chester disease, Rosai-Dorfman disease and other histiocytic neoplasms, Langerhans cell histiocytosis (LCH), Post-transplant lymphoproliferative disorder (PTLD), FLT3-mutated acute myeloid leukaemia, IDH1- and IDH2-mutated acute myeloid leukaemia, NPM1-mutated and KMT2A-rearranged acute myeloid leukaemia, Secondary and therapy-related acute myeloid leukaemia, Acute promyelocytic leukaemia, Acute myeloid leukaemia in older or unfit patients, Smouldering multiple myeloma, Newly diagnosed multiple myeloma, transplant-eligible, Newly diagnosed multiple myeloma, transplant-ineligible, Relapsed or refractory multiple myeloma, Plasma cell leukaemia, Lower-risk myelodysplastic syndromes, Higher-risk myelodysplastic syndromes, Chronic lymphocytic leukaemia, first treatment, Relapsed or refractory chronic lymphocytic leukaemia, Richter transformation of chronic lymphocytic leukaemia, Chronic myeloid leukaemia, chronic phase, Chronic myeloid leukaemia, accelerated and blast phase, Primary myelofibrosis, Standard-risk B-cell acute lymphoblastic leukaemia in children, High-risk acute lymphoblastic leukaemia in children (high-risk B-ALL and T-ALL), Philadelphia chromosome-positive acute lymphoblastic leukaemia in children (Ph-positive ALL), Philadelphia chromosome-like acute lymphoblastic leukaemia (Ph-like or BCR::ABL1-like ALL), Infant acute lymphoblastic leukaemia (KMT2A-rearranged, under one year), Relapsed and refractory acute lymphoblastic leukaemia in children, Acute myeloid leukaemia in children, Erdheim-Chester disease, Rosai-Dorfman-Destombes disease, Single-system Langerhans cell histiocytosis (bone, skin or one other organ), Multisystem Langerhans cell histiocytosis (with or without risk-organ involvement), Indolent and smouldering systemic mastocytosis, Advanced systemic mastocytosis (aggressive SM, SM with an associated haematological neoplasm, mast cell leukaemia), Early-stage classical Hodgkin lymphoma (stage I to II), Advanced-stage classical Hodgkin lymphoma (stage III to IV), Nodular lymphocyte-predominant Hodgkin lymphoma (nodular lymphocyte-predominant B-cell lymphoma), Relapsed and refractory classical Hodgkin lymphoma
Showing the organ this term concerns: Non-Hodgkin lymphoma (all types).
Shares Breast implant-associated anaplastic large cell lymphoma, Lymphomatoid papulosis, Primary effusion lymphoma, Enteropathy-associated T-cell lymphoma and the tags heme, lymphoma.
Shares Lymphomatoid papulosis, Ocular adnexal MALT lymphoma, Primary cutaneous anaplastic large cell lymphoma, Gastric MALT lymphoma and the tags heme, lymphoma.
Shares Breast implant-associated anaplastic large cell lymphoma, Plasmablastic lymphoma, Lymphoma (tissue type), Extranodal NK/T-cell lymphoma and the tags heme, lymphoma.
Shares Enteropathy-associated T-cell lymphoma, Monomorphic epitheliotropic intestinal T-cell lymphoma, International Prognostic Index (IPI), Extranodal NK/T-cell lymphoma and the tags heme, lymphoma.
Shares Gastric MALT lymphoma, Mycosis fungoides, Marginal zone lymphoma, Non-Hodgkin lymphoma (all types) and the tags heme, lymphoma.
Shares Lymphoma (tissue type), Non-Hodgkin lymphoma (all types) and the tags heme, lymphoma.
Shares Breast implant-associated anaplastic large cell lymphoma, Primary cutaneous anaplastic large cell lymphoma, International Prognostic Index (IPI), Lugano classification / Ann Arbor staging and the tags heme, lymphoma.
Shares Breast implant-associated anaplastic large cell lymphoma, Primary cutaneous anaplastic large cell lymphoma, International Prognostic Index (IPI), Non-Hodgkin lymphoma (all types) and the tags heme, lymphoma.