Cutaneous Lymphoma

A special issue of Cancers (ISSN 2072-6694). This special issue belongs to the section "Molecular Cancer Biology".

Deadline for manuscript submissions: 1 December 2024 | Viewed by 6817

Special Issue Editor


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Guest Editor
Department of Dermatology, University of Washington, Seattle, WA 98195, USA
Interests: cutaneous lymphoma; cutaneous T-cell lymphoma; mycosis fungoides; quality of life

Special Issue Information

Dear Colleagues,

Primary cutaneous lymphomas (PCLs) are a heterogeneous group of non-Hodgkin lymphomas and include the more common primary cutaneous T-cell lymphomas (CTCLs) as well as primary cutaneous B-cell lymphomas (CBCLs). Despite their unifying T-cell origin, the most common CTCL subtypes (mycosis fungoides, Sézary syndrome, and CD30 lymphoproliferative disorders) exhibit a wide variety of clinical and histologic characteristics.

For this Special Issue, we invite authors to submit high-quality original research and review articles that advance our understanding of PCL. Topics may include (but are not limited to) the pathophysiology of disease, molecular and biological markers for cutaneous lymphoma, novel therapies, and supportive care and quality of life.

Dr. Michi M. Shinohara
Guest Editor

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Keywords

  • primary cutaneous lymphoma
  • cutaneous T-cell lymphoma
  • mycosis fungoides
  • Sézary syndrome
  • health-related quality of life

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Published Papers (6 papers)

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Research

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15 pages, 12589 KiB  
Article
Non-Invasive Imaging Including Line-Field Confocal Optical Coherence Tomography (LC-OCT) for Diagnosis of Cutaneous Lymphomas
by Martina D’Onghia, Maria Mendonça-Sanches, Maria Erasti, Alessandra Cartocci, Laura Calabrese, Azzurra Sirchio, Linda Tognetti, Anastasia Batsikosta, Stefano Lazzi, Mariano Suppa, Simone Soglia, Josep Malvehy, Javiera Perez-Anker, Emanuele Cencini, Alberto Fabbri, Pietro Rubegni and Elisa Cinotti
Cancers 2024, 16(21), 3608; https://doi.org/10.3390/cancers16213608 - 25 Oct 2024
Viewed by 1032
Abstract
Background/Objectives: Primary cutaneous lymphomas (PCL) are a heterogeneous group of non-Hodgkin lymphomas arising from malignant T (CTCL) or B (CBCL) cells, often mimicking other skin conditions. Recently, non-invasive diagnostic imaging modalities, including dermoscopy, Reflectance Confocal Microscopy (RCM), and Line-field Optical Coherence Tomography (LC-OCT), [...] Read more.
Background/Objectives: Primary cutaneous lymphomas (PCL) are a heterogeneous group of non-Hodgkin lymphomas arising from malignant T (CTCL) or B (CBCL) cells, often mimicking other skin conditions. Recently, non-invasive diagnostic imaging modalities, including dermoscopy, Reflectance Confocal Microscopy (RCM), and Line-field Optical Coherence Tomography (LC-OCT), have become increasingly important, supporting clinicians in clinical practice. Hence, our study aimed to describe dermoscopic, RCM, and LC-OCT features of PCL and to explore their role in PCL management. Methods: Between December 2022 and January 2024, 40 lesions of 25 patients with PCL were retrospectively analyzed at the Dermatologic Unit of the University of Siena, Italy. Predefined dermoscopic, LC-OCT, and RCM criteria were assessed and their frequencies were calculated. Results: At dermoscopy, CTCL lesions were characterized by pinkish structureless areas (58,6%) and homogeneous distributed dotted vessels (35,7%), whereas 57.1% of CBCL presented with orange-yellow structureless areas. Considering CTCL, lymphocytes in the epidermis, dermal-epidermal junction, and dermis were detected by LC-OCT in 73.1%, 66.7%, and 51.9% and by RCM in 72.2%, 55.6%, and 61.1% of cases, respectively. The detection of lymphocytes was more precise using RCM than LC-OCT in CTCL (p < 0.001). Dermal infiltration of medium-reflective cells was visible in 80% and 40% of CBCL cases by LC-OCT and RCM, respectively. Conclusions: Non-invasive imaging techniques may support clinicians in managing PCL; however, further studies are mandatory in this field. Full article
(This article belongs to the Special Issue Cutaneous Lymphoma)
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10 pages, 234 KiB  
Article
Evaluation of Quality of Life and Treatment Satisfaction in Newly Diagnosed Cutaneous T-Cell Lymphoma Patients
by Rosanne Ottevanger, Sylvia van Beugen, Juliette M. Kersten, Andrea W. M. Evers, Maarten H. Vermeer, Rein Willemze and Koen D. Quint
Cancers 2024, 16(5), 937; https://doi.org/10.3390/cancers16050937 - 26 Feb 2024
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Abstract
Background: Little is known about the impact of MF on quality of life (QoL) in newly diagnosed patients. Objectives: To describe the impact of the MF diagnosis on QoL, patient expectations, and treatment satisfaction over the first 6 months after diagnosis. Methods: Outcomes [...] Read more.
Background: Little is known about the impact of MF on quality of life (QoL) in newly diagnosed patients. Objectives: To describe the impact of the MF diagnosis on QoL, patient expectations, and treatment satisfaction over the first 6 months after diagnosis. Methods: Outcomes of this prospective cohort study of newly diagnosed MF patients conducted between 2020 and 2022 at the Leiden University Medical Center included the Skindex-29, RAND-12 Health Survey, degree of itch, pain, and fatigue (Visual Analogue Scale (VAS)), patient expectations, and Client Satisfaction Questionnaire-8 (CSQ-8), measured at baseline and after six months. Results: A total of 28 patients with MF were included. At baseline, 66% (n = 18) “strongly-totally” expected positive effects of the treatment. At the time of diagnosis, 28% of the patients (n = 8) were moderately to severely affected. There was no statistical change in the Skindex-29 score sum score (20 [10–34] vs. 20 [9–36]; p = 0.81) or in the other three subdomains, the RAND-12 scores, and the VAS itch, pain, and fatigue over time. Treatment satisfaction was high overall. Conclusion: Despite that the newly diagnosed MF patients anticipate a positive treatment effect, few improvements in QoL and symptom reduction were found. These data can be used for adequate expectation management and provide a rationale for further evaluation of treatment regimens in these patients. Full article
(This article belongs to the Special Issue Cutaneous Lymphoma)
14 pages, 997 KiB  
Article
A Retrospective Chart Review of Treatment Patterns and Overall Survival among a Cohort of Patients with Relapsed/Refractory Mycosis Fungoides in France, Germany, Italy, Spain and the United Kingdom
by Chalid Assaf, Timothy M. Illidge, Nathalie Waser, Mary He, Tina Li, Athanasios Zomas, Nawal Bent-Ennakhil, Meredith Little, Pablo L. Ortiz-Romero, Nicola Pimpinelli, Mehul Dalal and Martine Bagot
Cancers 2023, 15(23), 5669; https://doi.org/10.3390/cancers15235669 - 30 Nov 2023
Cited by 1 | Viewed by 1450
Abstract
(1) Background: Most patients with mycosis fungoides (MF), a form of cutaneous T-cell lymphoma (CTCL), develop relapsed/refractory (R/R) disease following front-line systemic therapy. This report describes treatment patterns and outcomes from the subpopulation with R/R MF. (2) Methods: This observational, retrospective, cohort study [...] Read more.
(1) Background: Most patients with mycosis fungoides (MF), a form of cutaneous T-cell lymphoma (CTCL), develop relapsed/refractory (R/R) disease following front-line systemic therapy. This report describes treatment patterns and outcomes from the subpopulation with R/R MF. (2) Methods: This observational, retrospective, cohort study analyzed patient records (1984–2016) from 27 clinical sites in Europe. Outcomes included treatments received, response to first-, second- and third-line treatment, overall survival (OS) and progression-free survival (PFS). (3) Results: Of 104 patients with MF, 100 received second-line and 61 received third-line therapy. The median (range) times from the start of first-line therapy to the first R/R MF and from the first to the second R/R MF were 11.2 (0.3–166.5) and 13.5 (0.0–174.6) months, respectively. Second-and third-line treatment options varied and comprised systemic therapies (85% and 79% of patients, respectively), radiotherapy (32% and 34%, respectively) and topical therapies (48% and 36%, respectively). The median (95% confidence interval [CI]) OS from the diagnosis of the first R/R MF was 11.5 (6.5–not reached [NR]) years and was higher with non-chemotherapy (NR) versus chemotherapy (6.5 years); the estimated median PFS (95% CI) from the time of the first R/R MF was 1.3 (1.0–2.1) years. (4) Conclusions: High rates of R/R disease were observed after second- and third-line treatments in this real-world cohort, with longer median OS in patients receiving non-chemotherapy treatment versus chemotherapy. Following the standard management of MF and using recently approved targeted therapies can help improve patient outcomes in advanced-stage MF. Full article
(This article belongs to the Special Issue Cutaneous Lymphoma)
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Review

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11 pages, 1908 KiB  
Review
The Landscape of Cutaneous T-Cell Lymphoma (CTCL) in the Middle East and North Africa (MENA) and the Establishment of the MENA CTCL Working Group
by Rasha Abdel Tawab, Jihan Rajy, Salah Abdallat, Muna Abdula Almurrawi, Khalil Al Farsi, Jehad Alassaf, Hussni Alhateeti, Atlal Al Lafi, Reem El Bahtimi, Abdulhadi Jfri and Chalid Assaf
Cancers 2024, 16(19), 3380; https://doi.org/10.3390/cancers16193380 - 2 Oct 2024
Viewed by 902
Abstract
The high cancer burden in the Middle East and North Africa (MENA region) is coupled with an increasing cancer incidence. While the MENA region constitutes 6% of the world’s population, it remains underrepresented in clinical trials. Cutaneous T-cell lymphomas (CTCLs) represent a heterogeneous [...] Read more.
The high cancer burden in the Middle East and North Africa (MENA region) is coupled with an increasing cancer incidence. While the MENA region constitutes 6% of the world’s population, it remains underrepresented in clinical trials. Cutaneous T-cell lymphomas (CTCLs) represent a heterogeneous group of rare extranodal non-Hodgkin lymphomas with variable clinical presentation. In the MENA region, where darker skin colors are more common than in the West, CTCL generally presents at a younger age and with distinct clinical features that necessitate special expertise and management across disciplines: rare forms of CTCL are more common (hypo- and hyperpigmented MF) and a higher prevalence of pediatric MF is noticed. The multidisciplinary approach to cancer management is growing worldwide and is necessary for the comprehensive management of CTCL. The MENA CTCL group was established with the aim of creating a collaborative environment for the diagnosis and treatment of CTCL in the region. Its first meeting was held in May 2023. The group plans to increase the global representation of the MENA region and establish CTCL registries and patient advocacy groups in the region. Full article
(This article belongs to the Special Issue Cutaneous Lymphoma)
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10 pages, 1172 KiB  
Review
Clinical and Histologic Variants of CD8+ Cutaneous T-Cell Lymphomas
by Madisen A. Swallow, Goran Micevic, Amanda Zhou, Kacie R. Carlson, Francine M. Foss and Michael Girardi
Cancers 2024, 16(17), 3087; https://doi.org/10.3390/cancers16173087 - 5 Sep 2024
Viewed by 926
Abstract
Although the vast majority of CTCL subtypes are of the CD4+ T-helper cell differentiation phenotype, there is a spectrum of CD8+ variants that manifest wide-ranging clinical, histologic, and phenotypic features that inform the classification of the disease. CD8, like CD4, and cytotoxic molecules [...] Read more.
Although the vast majority of CTCL subtypes are of the CD4+ T-helper cell differentiation phenotype, there is a spectrum of CD8+ variants that manifest wide-ranging clinical, histologic, and phenotypic features that inform the classification of the disease. CD8, like CD4, and cytotoxic molecules (including TIA and granzyme) are readily detectable via IHC staining of tissue and, when expressed on the phenotypically abnormal T-cell population, can help distinguish specific CTCL subtypes. Nonetheless, given that the histopathologic differential for CD8+ lymphoproliferative disorders and lymphomas may range from very indolent lymphomatoid papulosis (LyP) to aggressive entities like CD8+ aggressive epidermotropic cytotoxic T-cell lymphoma (AECTCL), CD8 and/or cytotoxic molecule expression alone is insufficient for diagnosis and is not in itself an indicator of prognosis. We present a review of CTCL subtypes that can demonstrate CD8 positivity: CD8+ mycosis fungoides (MF), LyP type D, subcutaneous panniculitis-like T-cell lymphoma (SPTCL), primary cutaneous gamma/delta T-cell lymphoma (PCGDTL), CD8+ AECTCL, and acral CD8+ T-cell lymphoproliferative disorder (acral CD8+ TCLPD). These diseases may have different clinical manifestations and distinctive treatment algorithms. Due to the rare nature of these diseases, it is imperative to integrate clinical, histologic, and immunohistochemical findings to determine an accurate diagnosis and an appropriate treatment plan. Full article
(This article belongs to the Special Issue Cutaneous Lymphoma)
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10 pages, 253 KiB  
Review
Assessing Health-Related Quality of Life in Mycosis Fungoides and Sézary Syndrome: Unmet Needs
by Danielle Brazel, Cecilia Larocca and Michi M. Shinohara
Cancers 2024, 16(15), 2757; https://doi.org/10.3390/cancers16152757 - 3 Aug 2024
Viewed by 862
Abstract
Mycosis fungoides (MF) and Sézary syndrome (SS) can impair multiple dimensions of health-related quality of life (HRQoL). Currently, there is no standardized assessment tool for measuring HRQoL in patients with MF/SS. Here, we describe the existing literature on multiple dimensions of HRQoL in [...] Read more.
Mycosis fungoides (MF) and Sézary syndrome (SS) can impair multiple dimensions of health-related quality of life (HRQoL). Currently, there is no standardized assessment tool for measuring HRQoL in patients with MF/SS. Here, we describe the existing literature on multiple dimensions of HRQoL in MF/SS with a special focus on the gaps in the current knowledge and identify future directions necessary to assess the HRQoL of patients with this disease. Full article
(This article belongs to the Special Issue Cutaneous Lymphoma)
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