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A rare lymphoma that grows in the scar capsule the body forms around a breast implant, usually many years after the operation, and usually shows itself as sudden swelling of the breast from fluid around the implant. It is linked to textured implants, and when it is confined to the capsule it is usually cured by removing the implant and the capsule whole.
What it is. A T-cell lymphoma that arises in the fibrous capsule the body builds around a breast implant. In most women it stays inside that capsule and in the fluid between the capsule and the implant, and does not invade. WHO-HAEM5 describes it as an entity distinct from other ALK-negative anaplastic large cell lymphomas, usually non-invasive, arising in association with textured-surface implants, and associated with an excellent outcome, and adds that invasion of adjacent structures worsens the outlook.
How it shows itself, and the one thing that must not be missed. The usual presentation is a late seroma: the reconstructed or augmented breast swells, often suddenly, more than a year after the operation and typically many years after it. The rule that follows is simple and is the single most useful sentence on this page. A late seroma around a breast implant is aspirated, and the fluid is sent for cytology and for CD30 immunohistochemistry, rather than simply drained. Less often the disease presents as a mass in the capsule or as contracture of the capsule, and those carry a worse outlook because the disease has left the fluid.
How it differs from the lymphoma it is named after. It carries CD30 and lacks ALK, like systemic ALK-negative anaplastic large cell lymphoma, and it behaves nothing like it. The systemic disease is treated with combination chemotherapy and is cured in a minority; this one, confined to the capsule, is treated surgically and is cured in almost everybody.
Why the implant matters. The cases are almost exclusively in women with textured implants. In the Dutch series, 23 of the 28 implants of known type in the lymphoma cases were macrotextured, 82 per cent, against 45 per cent of implants sold in the same country over the same years. The meta-analysis found the same risk whether the implant was placed for reconstruction after cancer or for cosmetic reasons. WHO-HAEM5 describes the biology as involving an allergic inflammatory response, escape from the immune system through amplification at 9p24.1 and overexpression of PD-L1 in more than half of cases, and constant activation of the JAK-STAT pathway through mutations of STAT3, STAT5B, JAK1 and JAK2 and loss-of-function mutations of SOCS1 and SOCS3.
What the treatment is. Complete removal of the implant together with the whole capsule, intact where possible, and removal of any mass, which for disease confined to the capsule is usually the whole of the treatment. Disease that has spread beyond the capsule or formed a mass is staged and treated systemically, as a CD30-positive T-cell lymphoma. Women with implants and no symptoms are not advised to have them removed; the advice rests on recognising a late seroma and investigating it properly.
Rare in absolute terms, and strongly concentrated in women with textured implants. In the Dutch nationwide pathology registry, which identified every primary breast lymphoma between 1990 and 2016, 32 of 43 women with anaplastic large cell lymphoma of the breast had an implant on the same side, against 1 of 146 women with other primary breast lymphomas. The cumulative risk in women with implants was 29 per million at age 50 and 82 per million at 70, and the authors calculated that 6,920 women would need an implant to cause one case before the age of 75. A later meta-analysis across 525,475 patients with implants and 254 cases put the median time from implant to diagnosis at 13.16 years. The glossary entry written for the breast cancer pages carries the full set of figures.
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, 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, Non-Hodgkin lymphoma (all types), 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
A seroma appearing around a breast implant more than a year after the operation is aspirated and the fluid sent for cytology and for CD30 immunohistochemistry, rather than simply drained. That single step is what makes the diagnosis, and draining without testing is how it is missed. The same applies to a new mass in the capsule or to capsular contracture appearing years after surgery. Imaging, usually ultrasound in the first instance, maps the fluid and any mass.
Complete removal of the implant together with the whole capsule, intact where it can be done, and removal of any associated mass. For disease that has not left the capsule this is usually the whole of the treatment, and WHO-HAEM5 describes the entity as usually non-invasive and associated with an excellent outcome. Removal of an implant on the other side is discussed case by case. Women with implants and no symptoms are not advised to have them removed.
Staged and treated as a CD30-positive T-cell lymphoma, on the pathway used for systemic anaplastic large cell lymphoma, because WHO-HAEM5 records that invasion of adjacent structures worsens the outlook. Radiotherapy to the chest wall is used in some series for disease that cannot be removed completely. The evidence is case series; there are no trials and there will not be, because the great majority of patients are cured by surgery.
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Query for this cancer: (TITLE:"Breast implant-associated anaplastic large cell lymphoma" OR ABSTRACT:"Breast implant-associated anaplastic large cell lymphoma" OR TITLE:"BIA-ALCL" OR ABSTRACT:"BIA-ALCL" OR TITLE:"Breast implant-associated ALCL" OR ABSTRACT:"Breast implant-associated ALCL" OR TITLE:"Breast implant-associated anaplastic large-cell lymphoma" OR ABSTRACT:"Breast implant-associated anaplastic large-cell lymphoma" OR TITLE:"Implant-associated ALCL" OR ABSTRACT:"Implant-associated ALCL" OR TITLE:"Breast implant lymphoma" OR ABSTRACT:"Breast implant lymphoma") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Breast implant-associated anaplastic large cell lymphoma, not a curated reading list.
The revised fourth edition of the WHO classification named breast implant-associated anaplastic large cell lymphoma as a provisional entity separate from systemic ALK-negative disease.
The Dutch pathology registry found an implant on the same side in 32 of 43 women with breast anaplastic large cell lymphoma against 1 of 146 with other primary breast lymphomas, and put the cumulative risk in women with implants at 29 per million by age 50 and 82 per million by 70.
WHO-HAEM5 lists it as one of the three anaplastic large cell lymphomas, describes it as usually non-invasive and associated with an excellent outcome, and records that invasion of adjacent structures worsens the outlook.
The targets of this cancer's medicines and the ones linked to it directly.
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Any new neurological symptom that is not explained. Brentuximab vedotin carries a boxed warning for progressive multifocal leukoencephalopathy, a brain infection; the label says to hold the drug and investigate at the first suspicion.
Temperature of 38 C or higher, or feeling shivery and unwell even without a fever. Antibody-drug conjugates suppress the bone marrow, and several carry a boxed warning for severe neutropenia.
Tingling, numbness or weakness that affects walking or using the hands; peripheral neuropathy is common with the vedotin (MMAE) payload and doses are reduced or stopped at grade 2 to 3.
Nerve damage from chemotherapy that causes numbness, tingling and pain in the hands and feet, and sometimes weakness or hearing loss. It builds up with each dose, can be permanent, and is the main reason oxaliplatin, taxanes and vincristine have to be stopped or reduced.
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