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A cutaneous T-cell lymphoma that appears as one or a few red-purple nodules on the skin, often ulcerated, which may shrink on their own. Despite cells that look alarming under the microscope it stays in the skin in almost everybody and is treated with surgery or local radiotherapy rather than chemotherapy.
What it is. A lymphoma of CD30-positive T cells that arises in the skin and stays there. It usually appears as a single firm red or violet nodule or tumour, often several centimetres across and often breaking down into an ulcer, on a limb, the trunk, the head or the neck. Sometimes there are a few nodules in one area. Up to a quarter of lesions shrink partly or completely without any treatment, which is unusual for a lymphoma and is shared with its relative lymphomatoid papulosis.
How it differs from the lymphoma it is named after. The cells look the same as those of systemic anaplastic large cell lymphoma and carry the same CD30, but the disease is a different thing: WHO-HAEM5 files it among the primary cutaneous T-cell lymphomas rather than with the systemic anaplastic lymphomas, explicitly acknowledging its relationship to the skin lymphomas and its highly favourable outcome in contrast to systemic ALK-negative disease. It does not carry ALK, and a skin tumour with the same appearance that does carry ALK is usually systemic disease that has reached the skin.
How it sits beside lymphomatoid papulosis. The two are ends of one spectrum of CD30-positive skin disease: lymphomatoid papulosis is small papules that come and go in crops, and this is larger, more persistent nodules. The same person can have both, and the same clone can be found in both. The distinction is made on the clinical picture over time, not on the biopsy, which is why the dermatologist's photographs and history matter as much as the pathology.
What the outcome is. In the Stanford series of 56 patients with CD30-positive skin disease, disease-specific survival for this lymphoma was 85 per cent at both five and ten years. Localised and generalised skin disease behaved differently in that series, at 91 and 50 per cent five-year disease-specific survival, although with so few patients the difference was not statistically significant. Lesions recurred in 42 per cent, which is the usual course and is not a failure; three patients progressed beyond the skin.
How it is treated. Surgical excision or local radiotherapy for a single lesion or a few in one area, which is the great majority of patients. Low-dose methotrexate once a week for disease that keeps recurring in many places. Brentuximab vedotin for disease that is widespread or has spread beyond the skin: ALCANZA randomised patients with CD30-positive mycosis fungoides or primary cutaneous anaplastic large cell lymphoma to brentuximab vedotin or to the physician's choice of methotrexate or bexarotene, and the detail of that trial is on the cutaneous T-cell lymphoma page. Combination chemotherapy is reserved for disease outside the skin, because it produces short remissions and real harm in a disease that would otherwise be controlled for decades.
The European, American and international consensus group that writes the treatment recommendations describes the CD30-positive skin lymphomas as the second commonest form of cutaneous T-cell lymphoma after mycosis fungoides.
Averages across everyone diagnosed, often years ago. A median is the middle of a group: half the people counted lived longer than the figure shown, and some lived far longer. Your stage, subtype, age, fitness and the treatment you receive matter more than the average, and the numbers are improving quickly.
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, 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
No subtypes recorded beyond the ones named in the family strip above.
The diagnosis cannot be made on the biopsy alone, because the cells look identical to those of systemic anaplastic large cell lymphoma. It requires staging that shows no disease outside the skin, and a clinical history: how long the lesion has been there, whether others have come and gone, and whether the person has lymphomatoid papulosis or mycosis fungoides, which coexist with it. An ALK-positive skin tumour is usually systemic disease that has reached the skin, and is staged and treated as such.
Surgical excision or local radiotherapy. Both work; the choice is made on the site, the size and what will heal well. Lesions recur in a substantial proportion of patients, 42 per cent in the Stanford series, and recurrence in the skin is expected rather than a treatment failure: it is treated the same way again. Up to a quarter of lesions regress partly or completely without any treatment, so observing a lesion that is already shrinking is reasonable.
Low-dose weekly methotrexate for disease that keeps recurring in many places. Brentuximab vedotin for widespread or extracutaneous disease: ALCANZA randomised patients with CD30-positive mycosis fungoides or primary cutaneous anaplastic large cell lymphoma to brentuximab vedotin against the physician's choice of methotrexate or bexarotene, and the figures are on the cutaneous T-cell lymphoma page. Combination chemotherapy is reserved for disease outside the skin: it produces short remissions and real harm in a disease otherwise controlled for decades, and over-treatment is the commonest avoidable harm here.
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Query for this cancer: (TITLE:"Primary cutaneous anaplastic large cell lymphoma" OR ABSTRACT:"Primary cutaneous anaplastic large cell lymphoma" OR TITLE:"Primary cutaneous ALCL" OR ABSTRACT:"Primary cutaneous ALCL" OR TITLE:"pcALCL" OR ABSTRACT:"pcALCL" OR TITLE:"Primary cutaneous anaplastic large-cell lymphoma" OR ABSTRACT:"Primary cutaneous anaplastic large-cell lymphoma" OR TITLE:"Cutaneous anaplastic large cell lymphoma" OR ABSTRACT:"Cutaneous anaplastic large cell lymphoma" OR TITLE:"Primary cutaneous CD30-positive T-cell lymphoproliferative disorder: primary cutaneous anaplastic large cell lymphoma" OR ABSTRACT:"Primary cutaneous CD30-positive T-cell lymphoproliferative disorder: primary cutaneous anaplastic large cell lymphoma") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Primary cutaneous anaplastic large cell lymphoma, not a curated reading list.
Fifty-six patients with CD30-positive skin lymphoproliferative disorders: disease-specific survival for primary cutaneous anaplastic large cell lymphoma was 85 per cent at five and ten years, lesions recurred in 42 per cent, and three patients progressed beyond the skin.
A panel from the EORTC, the International Society for Cutaneous Lymphomas and the United States Cutaneous Lymphoma Consortium set out treatment recommendations for the CD30-positive skin lymphoproliferative disorders and noted that most published evidence is small retrospective series.
WHO-HAEM5 groups primary cutaneous anaplastic large cell lymphoma under the primary cutaneous T-cell lymphomas, acknowledging its relationship to them and its favourable outcome in contrast to systemic ALK-negative disease.
The targets of this cancer's medicines and the ones linked to it directly.
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Bleeding that does not stop by itself, bleeding from more than one site, or new bruising in several places or one large area.
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.
High-dose methotrexate requires normal renal function, hydration, urine alkalinisation and leucovorin rescue with level monitoring.
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.
See all on the product pages:Brentuximab vedotinMethotrexate·Printable cards in the navigator
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