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4 standard-of-care settings across 4 lines and 1 biomarker subgroup. Rows come from the cancer page's standard of care; the grid places each on its line and subgroup.
| Line | All comers |
|---|---|
| Screening, prevention and diagnosis | 1 |
| Early / localised | 1 |
| Advanced, first line | 1 |
| Other settings | 1 |
| Subgroup | Setting | Approach | Products and trials | Guideline | Evidence |
|---|---|---|---|---|---|
| All comers | Mycosis fungoides: staging first, because stage decides everything | Mycosis fungoides is staged by the ISCL/EORTC TNMB system, which classifies skin (patches or plaques covering under or over 10 per cent of the body surface, tumours, erythroderma), nodes, viscera and blood. The staging revision of 2007 remains the basis of treatment choice and trial eligibility, and a validation study in a large cohort confirmed that the resulting stages separate survival. Why it matters more here than in most cancers: early-stage mycosis fungoides (stage IA to IIA, patches and plaques without tumours or blood involvement) has a life expectancy close to that of the general population and is treated with creams and light, while advanced disease (tumour stage, erythroderma, nodal or blood involvement) needs systemic treatment. Treating early disease with chemotherapy shortens neither the disease nor anything else and causes harm, which is the single most important thing to get right. The diagnosis itself is often slow, because early mycosis fungoides looks like eczema or psoriasis for years and the biopsy is frequently non-diagnostic until the disease is established. Repeated biopsies over time, read by a dermatopathologist, are normal and are not a failure. | NCCN Primary Cutaneous Lymphomas; ESMO; ISCL/EORTC staging | 0 |
| Subgroup | Setting | Approach | Products and trials | Guideline | Evidence |
|---|---|---|---|---|---|
| All comers | Early-stage mycosis fungoides (IA to IIA): skin-directed treatment only | The aim is control of the skin with the least treatment that achieves it, over decades. Topical corticosteroids, potent or very potent, are first line for patches and plaques and clear a large proportion. Topical mechlorethamine (chlormethine) gel, approved in the United States on 23 August 2013 for stage IA and IB mycosis fungoides-type cutaneous T-cell lymphoma after prior skin-directed therapy, cleared index lesions in its pivotal randomised trial in 58.5 per cent of patients against 47.7 per cent with the traditional compounded ointment, meeting the non-inferiority bar; contact dermatitis is the main problem and is managed by reducing frequency rather than stopping. Topical bexarotene gel and topical carmustine are alternatives. Imiquimod is used for a small number of resistant plaques. Phototherapy is the mainstay for widespread patch and plaque disease: narrowband UVB for patches and thin plaques, PUVA (psoralen with UVA) for thicker plaques and follicular disease, given two or three times a week at a dermatology unit and then tapered to a maintenance schedule. Cumulative UV dose carries its own long-term skin cancer risk, which is the reason for tapering. Localised radiotherapy at low dose, often 8 Gy in two fractions, clears an individual tumour or a resistant plaque quickly and can be repeated. Total skin electron beam therapy treats the whole skin surface and is reserved for extensive disease; low-dose schedules of 10 to 12 Gy are now preferred to the historic 30 to 36 Gy because they can be repeated. | Mechlorethamine (chlormethine)BexaroteneCarmustineImiquimodTotal skin electron beam therapyPalliative radiotherapySkin-directed therapy in mycosis fungoides: creams, light and small radiotherapy fieldsRadiotherapy in lymphoma: involved-site fields, 24 Gy, 4 Gy and total skin electron therapyTopical chemotherapy in cutaneous T-cell lymphoma: positive results of a randomized, controlled, multicenter trial testing the efficacy and safety of a novel mechlorethamine, 0.02%, gel in mycosis fungoides | NCCN Primary Cutaneous Lymphomas; ESMO; BAD/BSH primary cutaneous lymphoma guideline | 84 |
| Subgroup | Setting | Approach | Products and trials | Guideline | Evidence |
|---|---|---|---|---|---|
| All comers | Advanced mycosis fungoides (IIB to IVB): systemic treatment, and what to reach for first | Systemic treatment is indicated for tumour-stage disease, erythroderma, nodal or visceral involvement, blood involvement, or skin disease that has failed skin-directed therapy. Single agents are preferred to combination chemotherapy, which produces fast responses that do not last and damages the immune system in a patient already prone to skin infection and sepsis. Retinoids and interferon. Oral bexarotene and interferon alfa are long-standing options, often combined with phototherapy; both are slow-acting and require monitoring of lipids and thyroid function in the case of bexarotene. Methotrexate at low weekly oral dose, which is inexpensive, familiar and effective in a proportion. Mogamulizumab, an anti-CCR4 antibody approved in the United States on 8 August 2018. MAVORIC randomised 372 patients with previously treated mycosis fungoides or Sezary syndrome to mogamulizumab or vorinostat: median progression-free survival 7.7 against 3.1 months (hazard ratio 0.53) and overall response 28 against 5 per cent, with a response in the blood compartment of 68 per cent. It is therefore the drug of choice where the blood is involved. Brentuximab vedotin for CD30-expressing disease. ALCANZA randomised patients with CD30-positive mycosis fungoides or primary cutaneous anaplastic large cell lymphoma to brentuximab vedotin or to physician's choice of methotrexate or bexarotene: objective response lasting four months or more was 56.3 against 12.5 per cent and median progression-free survival 16.7 against 3.5 months. Peripheral neuropathy is the limiting toxicity. Other options: romidepsin, denileukin diftitox, which returned in 2024 as Lymphir for relapsed or refractory stage I to III disease after at least one systemic therapy, gemcitabine, pegylated liposomal doxorubicin, and allogeneic transplant with reduced-intensity conditioning, which is the only treatment that produces long remissions in advanced disease and is offered to younger fit patients. | MAVORIC: mogamulizumab versus vorinostat in previously treated cutaneous T-cell lymphomaALCANZA: brentuximab vedotin versus physician's choice in CD30-positive cutaneous T-cell lymphomaMogamulizumabBrentuximab vedotinBexaroteneInterferon alfa-2a/2bMethotrexateRomidepsinVorinostatDenileukin diftitoxGemcitabinePegylated liposomal doxorubicinAllogeneic stem cell transplantationSkin-directed therapy in mycosis fungoides: creams, light and small radiotherapy fields | NCCN Primary Cutaneous Lymphomas; ESMO; MAVORIC, ALCANZA | 96 |
| Subgroup | Setting | Approach | Products and trials | Guideline | Evidence |
|---|---|---|---|---|---|
| All comers | Living with mycosis fungoides: itch, infection and the things that make the difference day to day | Itch is the symptom patients rank as the worst and it is undertreated. Emollients applied several times a day, potent topical steroids, sedating antihistamines at night, gabapentin or pregabalin for neuropathic itch, and aprepitant or mirtazapine in resistant cases. Controlling the disease is the most effective anti-itch treatment, which is an argument for treating skin disease that is not otherwise dangerous. Infection. Staphylococcus aureus colonises broken skin and is a common cause of both flares and sepsis; swabs, topical antiseptics, bleach baths and prompt antibiotics for cellulitis are part of routine care, and a patient with advanced disease and a fever is treated as an emergency. Skin care. Soap substitutes, lukewarm baths, cotton clothing, and avoidance of overheating. Photoprotection after phototherapy or radiotherapy. The long view. Early-stage mycosis fungoides is a chronic skin disease to be managed rather than an emergency, and most people with it die of something else. That is worth saying out loud at diagnosis, because the word lymphoma does not sound like that. | NCCN Primary Cutaneous Lymphomas; BAD/BSH primary cutaneous lymphoma guideline | 87 |
Lines and subgroups are parsed from the setting text of each standard-of-care row and can misclassify an unusual phrasing; the row’s own setting is always shown. Guideline chips reflect the NCCN category and ESMO-MCBS grade recorded on the cancer page, checked on its stated date. Not medical advice.