{"entity":{"id":"primary-cutaneous-anaplastic-large-cell-lymphoma","kind":"cancer","name":"Primary cutaneous anaplastic large cell lymphoma","aka":["Primary cutaneous ALCL","pcALCL","Primary cutaneous anaplastic large-cell lymphoma","Cutaneous anaplastic large cell lymphoma","Primary cutaneous CD30-positive T-cell lymphoproliferative disorder: primary cutaneous anaplastic large cell lymphoma"],"tldr":"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.","summary":"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.\n\nHow 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.\n\nHow 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.\n\nWhat 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.\n\nHow 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.","asOf":"2026-09-29","wikipedia":"https://en.wikipedia.org/wiki/Anaplastic_large-cell_lymphoma","links":[{"label":"WHO Classification of Haematolymphoid Tumours, 5th edition: lymphoid neoplasms (Alaggio, Leukemia 2022)","url":"https://doi.org/10.1038/s41375-022-01620-2"},{"label":"International Consensus Classification of Mature Lymphoid Neoplasms (Campo, Blood 2022)","url":"https://doi.org/10.1182/blood.2022015851"},{"label":"EORTC, ISCL and USCLC consensus recommendations for the treatment of primary cutaneous CD30-positive lymphoproliferative disorders: lymphomatoid papulosis and primary cutaneous anaplastic large-cell lymphoma (Kempf, Blood 2011)","url":"https://doi.org/10.1182/blood-2011-05-351346"},{"label":"CD30-positive cutaneous lymphoproliferative disorders: the Stanford experience in lymphomatoid papulosis and primary cutaneous anaplastic large cell lymphoma, 56 patients (Liu, J Am Acad Dermatol 2003)","url":"https://doi.org/10.1016/S0190-9622(03)02484-8"},{"label":"Lymphoma incidence, survival and prevalence 2004 to 2014, subtype analyses from the UK Haematological Malignancy Research Network (Smith, Br J Cancer 2015)","url":"https://doi.org/10.1038/bjc.2015.94"}],"tags":["heme","lymphoma","subtype-page"],"related":["lymphomatoid-papulosis","cutaneous-t-cell-lymphoma","mycosis-fungoides","alk-negative-anaplastic-large-cell-lymphoma","sezary-syndrome"],"cancers":[],"sections":[],"technologies":["histopathology-ihc","palliative-radiotherapy"],"targets":["cd30"],"drugs":["brentuximab-vedotin","methotrexate"],"companies":[],"institutions":[],"pathways":[],"terms":["lymphoma-classification-2022","lymphoma-tx-skin-directed-therapy","lymphoma-tx-radiotherapy","lymphoma-nodal-versus-extranodal","lymphoma-indolent-versus-aggressive"],"trials":["alcanza"],"people":[],"bottlenecks":[],"keyPapers":[],"journals":[],"dependsOn":[],"notes":[],"group":"haematologic","burden":"In the United Kingdom population series that reports lymphoma by subtype, the primary cutaneous CD30-positive lymphoproliferative disorders, which group this disease with lymphomatoid papulosis, accounted for 37 of 5,796 lymphomas, a European age-standardised rate of 0.13 per 100,000 a year and a median age at diagnosis of 52.9 years, with five-year relative survival of 88.3 per cent. 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.","subtypes":[],"biomarkers":["CD30 on more than three-quarters of the large cells, which defines the group","Absence of ALK; an ALK-positive skin tumour is usually systemic disease that has reached the skin","Staging to confirm there is no disease outside the skin, which changes both the diagnosis and the treatment","A clonal T-cell receptor rearrangement, which may be shared with coexisting lymphomatoid papulosis","Whether the skin disease is localised or widespread, which the consensus recommendations use to choose treatment"],"standardOfCare":[{"setting":"Confirming it is confined to the skin","approach":"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.","refs":["histopathology-ihc","cd30","fdg-pet","lymphomatoid-papulosis","lugano-classification"],"guideline":{"version":"WHO Classification of Haematolymphoid Tumours, 5th edition (2022), with the International Consensus Classification (2022) where they differ","url":"https://doi.org/10.1038/s41375-022-01620-2"}},{"setting":"A single lesion or a few in one area, which is most patients","approach":"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.","refs":["palliative-radiotherapy","lymphoma-tx-skin-directed-therapy","lymphoma-tx-radiotherapy"],"guideline":{"version":"EORTC, ISCL and USCLC consensus recommendations for primary cutaneous CD30-positive lymphoproliferative disorders (Blood 2011)","url":"https://doi.org/10.1182/blood-2011-05-351346"}},{"setting":"Widespread skin disease, or disease beyond the skin","approach":"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.","refs":["methotrexate","brentuximab-vedotin","alcanza","cutaneous-t-cell-lymphoma","lymphoma-tx-skin-directed-therapy"],"guideline":{"version":"EORTC, ISCL and USCLC consensus recommendations (Blood 2011); NCCN Primary Cutaneous Lymphomas","url":"https://doi.org/10.1182/blood-2011-05-351346"}}],"stateOfArt":[],"history":[{"year":2003,"title":"The Stanford series","note":"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.","refs":[]},{"year":2011,"title":"International consensus recommendations","note":"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.","refs":["brentuximab-vedotin"]},{"year":2022,"title":"Filed with the skin lymphomas, not the systemic ones","note":"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.","refs":["lymphoma-classification-2022"]}],"pipeline":[],"openProblems":["The consensus recommendations that govern treatment state that most of the evidence behind them is small retrospective series and case reports, and that very few prospective or multicentre studies exist.","The line between this disease and lymphomatoid papulosis is drawn on the clinical course rather than on the biopsy, and in a person who has both there is no test that settles which lesion is which.","Over-treatment is the commonest harm: combination chemotherapy for skin-limited disease produces short remissions in a condition that is otherwise controlled for decades."],"parent":"cutaneous-t-cell-lymphoma"},"route":"/cancers/primary-cutaneous-anaplastic-large-cell-lymphoma/","neighbours":{"cancer":[{"id":"alk-negative-anaplastic-large-cell-lymphoma","kind":"cancer","name":"ALK-negative anaplastic large cell lymphoma","route":"/cancers/alk-negative-anaplastic-large-cell-lymphoma/"},{"id":"alk-positive-anaplastic-large-cell-lymphoma","kind":"cancer","name":"ALK-positive anaplastic large cell lymphoma","route":"/cancers/alk-positive-anaplastic-large-cell-lymphoma/"},{"id":"cutaneous-t-cell-lymphoma","kind":"cancer","name":"Cutaneous T-cell lymphoma (mycosis fungoides and Sezary syndrome)","route":"/cancers/cutaneous-t-cell-lymphoma/"},{"id":"lymphomatoid-papulosis","kind":"cancer","name":"Lymphomatoid papulosis","route":"/cancers/lymphomatoid-papulosis/"},{"id":"mycosis-fungoides","kind":"cancer","name":"Mycosis fungoides","route":"/cancers/mycosis-fungoides/"},{"id":"sezary-syndrome","kind":"cancer","name":"Sezary syndrome","route":"/cancers/sezary-syndrome/"}],"technology":[{"id":"fdg-pet","kind":"technology","name":"FDG PET","route":"/technologies/fdg-pet/"},{"id":"histopathology-ihc","kind":"technology","name":"Histopathology & immunohistochemistry","route":"/technologies/histopathology-ihc/"},{"id":"palliative-radiotherapy","kind":"technology","name":"Palliative radiotherapy","route":"/technologies/palliative-radiotherapy/"}],"target":[{"id":"cd30","kind":"target","name":"CD30","route":"/targets/cd30/"}],"drug":[{"id":"brentuximab-vedotin","kind":"drug","name":"Brentuximab vedotin","route":"/drugs/brentuximab-vedotin/"},{"id":"methotrexate","kind":"drug","name":"Methotrexate","route":"/drugs/methotrexate/"}],"term":[{"id":"lymphoma-indolent-versus-aggressive","kind":"term","name":"Indolent and aggressive lymphoma","route":"/terms/lymphoma-indolent-versus-aggressive/"},{"id":"lugano-classification","kind":"term","name":"Lugano classification / Ann Arbor staging","route":"/terms/lugano-classification/"},{"id":"lymphoma-nodal-versus-extranodal","kind":"term","name":"Nodal and extranodal lymphoma","route":"/terms/lymphoma-nodal-versus-extranodal/"},{"id":"lymphoma-tx-radiotherapy","kind":"term","name":"Radiotherapy in lymphoma: involved-site fields, 24 Gy, 4 Gy and total skin electron therapy","route":"/terms/lymphoma-tx-radiotherapy/"},{"id":"lymphoma-tx-skin-directed-therapy","kind":"term","name":"Skin-directed therapy in mycosis fungoides: creams, light and small radiotherapy fields","route":"/terms/lymphoma-tx-skin-directed-therapy/"},{"id":"lymphoma-classification-2022","kind":"term","name":"The two lymphoma classifications of 2022 (WHO-HAEM5 and ICC)","route":"/terms/lymphoma-classification-2022/"}],"trial":[{"id":"alcanza","kind":"trial","name":"ALCANZA","route":"/trials/alcanza/"}]}}