Primary cutaneous lymphomas: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up

Willemze, Rein; Hodak, Emmilia; Zinzani, Pier Luigi; Specht, Lena; Ladetto, Marco

doi:10.1093/annonc/mdy133

Cited by 193 publications

(149 citation statements)

References 57 publications

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“…Patients with SP TCL had the best outcome in our series, with a pOS at 5 years of 0.78, and this is in accordance to earlier reports [13,14]. Only one patient in our series was diagnosed with PCGD TCL, and that patient died from disease, in accordance to what is known from the literature [15].…”

Section: Discussionsupporting

confidence: 93%

Non-anaplastic peripheral T cell lymphoma in children and adolescents—an international review of 143 cases

et al. 2016

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Peripheral T cell lymphomas (PTCL) are rare in children and adolescents, and data about outcome and treatment results are scarce. The present study is a joint, international, retrospective analysis of 143 reported cases of non-anaplastic PTCL in patients <19 years of age, with a focus on treatment and outcome features. One hundred forty-three patients, between 0.3 and 18.7 years old, diagnosed between 2000 and 2015 were included in the study. PTCL not otherwise specified was the largest subgroup, followed by extranodal NK/T cell lymphoma, hepatosplenic T cell lymphoma (HS TCL), and subcutaneous panniculitis-like T cell lymphoma (SP TCL). Probability of overall survival (pOS) at 5 years for the whole group was 0.56 ± 0.05, and probability of event-free survival was (pEFS) 0.45 ± 0.05. Patients with SP TCL had a good outcome with 5-year pOS of 0.78 ± 0.1 while patients with HS TCL were reported with 5-year pOS of only 0.13 ± 0.12. Twenty-five percent of the patients were reported to have a pre-existing condition, and this group had a dismal outcome with 5-year pOS of 0.29 ± 0.09. The distribution of non-anaplastic PTCL subtypes in pediatric and adolescent patients differs from what is reported in adult patients. Overall outcome depends on the subtype with some doing better than others. Pre-existing conditions are frequent and associated with poor outcomes. There is a clear need for subtype-based treatment recommendations for children and adolescents with PTCL.

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Section: Discussionsupporting

confidence: 93%

Non-anaplastic peripheral T cell lymphoma in children and adolescents—an international review of 143 cases

et al. 2016

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“…Treatment alternatives identified in this study are congruent with clinical data and treatment information on MF patients reported by AEDV's primary cutaneous lymphomas patient registry, 20 and also concur with the treatments recommended for early-stage MF management in the main clinical practice guidelines. 11,[30][31][32] Additionally, the therapeutic options assessed in this study are consistent with the first results shown by the AEDV's primary cutaneous lymphomas. The SDTs assessed in this analysis represent 90.8% (topical corticosteroids), 86.1% (phototherapies), 8.3% (TSEBT) and 6.0% (topical chemotherapies) of the treatments used for MF patients recorded by the AEDV.…”

Section: Discussionsupporting

confidence: 83%

“…25 Treatment strategies for MF can be split into two categories: skin-directed treatments (SDT) and systemic treatments including biological response modifiers, single or multiple chemotherapies, epigenetic therapies, monoclonal antibodies and hematopoietic stem cell transplantation. 11,30,31 Despite the availability of multiple treatment options, none of these may be considered as standard. 32 Patients with limited-stage disease are usually treated with SDT, while patients with advanced stage MF require a broader multidisciplinary approach involving various combinations of SDT, biological response modifiers and systemic chemotherapy.…”

Section: Introductionmentioning

confidence: 99%

“…32 Patients with limited-stage disease are usually treated with SDT, while patients with advanced stage MF require a broader multidisciplinary approach involving various combinations of SDT, biological response modifiers and systemic chemotherapy. 11,[30][31][32] MF presents a significant clinical burden for patients with this rare disease. Patients not only feel overwhelmed by the physical symptoms of the disease but also suffer from sleep problems because of itching, 8,33 and experience a significant emotional impact caused by frustration and rejection of their disease.…”

Section: Introductionmentioning

confidence: 99%

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Cost of early-stage mycosis fungoides treatments in Spain

Ortiz‐Romero

Servitje

Estrach³

et al. 2018

European Journal of Cancer

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Aim: To identify the most common therapeutic options for the treatment of early-stage mycosis fungoides in Spain, quantify their associated healthcare resource use and costs. Methods: After reviewing the literature, a panel of 6 Spanish clinical dermatologists validated the treatments and healthcare resource use through a structured questionnaire. Individual responses were collected, analyzed and presented into a face-to-face meeting in order to reach a consensus. Cost categories considered were: drug acquisition and administration, photo/radiotherapy session and maintenance, clinical follow-up visits and laboratory tests. Costs were expressed in euros from 2018. The Spanish National Health System perspective was considered, taking into account direct health costs and time horizons of 1, 3 and 6 months. Results: Costs for the skin-directed treatments (SDT) assessed at 1, 3 and 6 months, were: Topical carmustine [€6,593.36, €19,780.09 and €27,592.78]; Phototherapy with psoralens and ultraviolet A light (PUVA) [€1,098.68, €2,999.99 and €3,187.60]; Narrow-band ultraviolet B phototherapy [€1,657.47, €4,842.10 and €4,842.10]; Total skin electron beam therapy (TSEBT) [€6,796.45, €7,913.34 and €7,913.34]. Cost for topical corticosteroids, being considered an adjuvant option, were €17.16, €51.49 and €102.97. Costs for the assessed systemic treatments alone or in combination with SDT at 1, 3 and 6 months, were: Systemic retinoids [€2,026.03, €5,206.63 and €7,426.42]; Systemic retinoids + PUVA phototherapy [€3,066.50, €8,271.26 and €10,046.58]; Interferon alfa + PUVA phototherapy [€1,541.09, €5,167.57 and €6,404.55].Conclusion: According to the Spanish clinical practice, phototherapies in monotherapy were the treatments with the lowest associated costs regardless of the time horizon considered. TSEBT turned out as the treatment with the highest associated costs when considering 1 month. However, while considering 3 and 6 months the treatment with the highest associated costs was topical carmustine. The results of this analysis may provide critical information to measure the disease burden, to detect unmet medical needs and to advocate towards better treatments for this rare disease. ClinicoEconomics and Outcomes Research Dovepressopen access to scientific and medical research Open Access Full Text Article submit your manuscript | www.dovepress.com ClinicoEconomics and Outcomes Research 2020:12 91-105

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“…For localized low-grade lymphomas, skin-directed therapies with surgery, intralesional corticosteroids or interferon-α, and radiotherapy are vivid treatment options (3,4). Despite high initial response rates, relapses are common.…”

Section: Introductionmentioning

confidence: 99%

Innovative Therapeutic Approaches in Primary Cutaneous B Cell Lymphoma

2020

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Background: Primary cutaneous B-cell lymphomas (pCBCL) include an infrequent group of non-Hodgkin lymphomas that are limited to skin sites at the time of diagnosis. They comprise roughly 20-25% of all cutaneous lymphomas and are subdivided into primary cutaneous marginal zone lymphoma (PCMZL), primary cutaneous follicle center lymphoma (PCFCL), and primary cutaneous diffuse large cell B cell lymphoma, leg type (PCDLCBCL, LT). The first two show a rather indolent course while PCDLCBCL, LT carries a worse prognosis. Intravascular large cell B-cell lymphoma is the most infrequent subtype, and its therapy is not covered in this review. Topical Therapy: For solitary, single-site PCMZL and PCFCL, several topical treatment options exist. They include, but are not limited to, excision, radiotherapy, and intralesional therapies, discussed in this review. However, in selected cases, even "watchful waiting" is reasonable. Systemic Therapy: Indolent types of pCBCL rarely require systemic treatment. However, in extended cases and more importantly DLCBCL, LT, systemic treatment is the first choice. Monoclonal anti-CD20-antibody rituximab is often used as monotherapy in PCMZL and PCFCL or combined with chemotherapy in PCDLBCL, LT. Newer options are monoclonal anti-CD40 antibody dacetuzumab, anti-PD-1 and anti-PD-L1 checkpoint inhibitors, and Bruton tyrosine kinase inhibitors. Conclusion: Indolent pCBCL are treated with a risk-adapted strategy using intralesional steroids, RT, and interferon-α as first-line treatments. Relapsing cases may profit from rituximab. In aggressive PCDLCBCL, LT, rituximab with polychemotherapy is recommended. Innovative therapies include intralesional oncolytic virotherapy, systemic monoclonal antibodies, and small molecules.

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Primary cutaneous lymphomas: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up

Cited by 193 publications

References 57 publications

Non-anaplastic peripheral T cell lymphoma in children and adolescents—an international review of 143 cases

Non-anaplastic peripheral T cell lymphoma in children and adolescents—an international review of 143 cases

Cost of early-stage mycosis fungoides treatments in Spain

Innovative Therapeutic Approaches in Primary Cutaneous B Cell Lymphoma

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