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8 slides generated from the cancer page, with a quiz from the open benchmark and speaker notes that cite the sources. Arrow keys move between slides; Print gives one slide per page.
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.
| Setting | Approach | Guideline |
|---|---|---|
| Confirming it is confined to the skin | 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. | not mapped |
| A single lesion or a few in one area, which is most patients | 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. | not mapped |
| Widespread skin disease, or disease beyond the skin | 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. | not mapped |