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Chinese Clinical Oncology Feb 2019Primary cutaneous lymphoma is the second most common type of extranodal lymphoma. The clinical behavior of this lymphoma differs from that of other extranodal lymphomas... (Review)
Review
Primary cutaneous lymphoma is the second most common type of extranodal lymphoma. The clinical behavior of this lymphoma differs from that of other extranodal lymphomas and thus requires a particular pretreatment evaluation and treatment strategy. Cutaneous T-cell lymphoma (CTCL) accounts for 80% of primary cutaneous lymphoma cases and includes several confirmed disease entities as well as provisional entities. Local radiation for CTCLs is applicable for both curative and palliative intents and is based on the involved-site radiotherapy (ISRT) concept. Primary cutaneous CD30-positive lymphoproliferative disorders include primary cutaneous anaplastic large-cell lymphoma (C-ALCL), lymphomatoid papulosis (LyP), and borderline lesions, all of which exhibit indolent behavior. One half of all LyP cases show spontaneous regression and do not require active treatment. Solitary or localized C-ALCL cases are treated with surgical excision or local radiation of 24-36 Gy. The most common relapse site after local treatment is other skin areas; however, skin relapse is not associated with worsened prognosis. Subcutaneous panniculitis-like T-cell lymphoma (SPTL) without hemophagocytic syndrome exhibits indolent behavior, and localized lesions can be successfully treated with local radiation of 40 Gy or more. Extranodal NK/T-cell lymphoma, nasal type has an aggressive clinical behavior even with intensive chemotherapy and a dose of 50 Gy or higher might be required for good tumor control. Palliative local radiation of 8 Gy in one or two fractions is effective in treating refractory or relapsed CTCLs. ISRT with curative intent should encompass the original suspicious lesions plus a 2-3 cm margin, while ISRT with palliative intent should encompass visible lesions plus a 1-2 cm margin. Appropriate electron beam energy and bolus thickness should be selected according to the skin surface dose and thickness of the patch and plaque lesions. All CTCL cases should be reviewed by a multidisciplinary team to ensure an appropriate treatment strategy.
Topics: Humans; Lymphoma, T-Cell, Cutaneous; Mycosis Fungoides; Sezary Syndrome
PubMed: 30180750
DOI: 10.21037/cco.2018.07.01 -
The Journal of Dermatology Dec 2019Mycosis fungoides (MF) is the most frequent type of cutaneous T-cell lymphoma. Folliculotropic MF (fMF) and erythrodermic MF (eMF) are two distinct variants of MF. Both... (Review)
Review
Mycosis fungoides (MF) is the most frequent type of cutaneous T-cell lymphoma. Folliculotropic MF (fMF) and erythrodermic MF (eMF) are two distinct variants of MF. Both variants have been considered aggressive and most cases are less responsive to standard skin-directed therapies than classical MF. We, however, experienced many cases with fMF or eMF who showed indolent clinical courses. In this article, we reviewed 10 cases with fMF and 13 cases with eMF who came to our department between 2005 and 2017. In patients with fMF, monotherapy with topical corticosteroid was effective in two cases (20%) and ultraviolet phototherapy with oral retinoid controlled disease activity in two cases (20%). Five patients with eMF (38%) responded well to ultraviolet phototherapy. In conclusion, patients with early fMF and a subgroup of eMF patients have an indolent disease course, as was proposed among the specialists. Skin-directed therapies are preferable rather than aggressive treatment in those cases.
Topics: Adult; Aged; Aged, 80 and over; Conservative Treatment; Female; Humans; Male; Middle Aged; Mycosis Fungoides; Retrospective Studies; Skin
PubMed: 31625194
DOI: 10.1111/1346-8138.15114 -
Frontiers in Immunology 2023Mycosis fungoides (MF) and Sézary syndrome (SS) are forms of cutaneous T cell lymphoma (CTCL) that pose significant challenges in their clinical management,... (Review)
Review
Mycosis fungoides (MF) and Sézary syndrome (SS) are forms of cutaneous T cell lymphoma (CTCL) that pose significant challenges in their clinical management, particularly in refractory and advanced-stage disease. With the emergence of novel therapeutic modalities however, there are increasing opportunities to exploit the current understanding of pathophysiologic mechanisms of MF/SS for treatment. This review summarizes recent advances in the treatment of MF/SS, with a focus on monoclonal antibodies, immunotherapies, and Janus kinase (JAK) inhibitors, including ongoing clinical trials.
Topics: Humans; Sezary Syndrome; Antibodies, Monoclonal; Janus Kinase Inhibitors; Skin Neoplasms; Mycosis Fungoides; Lymphoma, T-Cell, Cutaneous; Immunotherapy
PubMed: 38022633
DOI: 10.3389/fimmu.2023.1291259 -
Der Hautarzt; Zeitschrift Fur... Dec 2014Folliculotropic mycosis fungoides represents a rare variant of the CD4-positive cutaneous T-cell lymphoma mycosis fungoides. It is characterized by tropism of the...
BACKGROUND
Folliculotropic mycosis fungoides represents a rare variant of the CD4-positive cutaneous T-cell lymphoma mycosis fungoides. It is characterized by tropism of the lymphocytic infiltrate for hair follicle and other adnexal structures.
CASE PRESENTATION
Our patient presented with a 20-year history of pruritic skin lesions, which had been diagnosed as atopic dermatitis. The diagnosis of folliculotropic mycosis fungoides was confirmed by skin biopsy. Since topical therapy was ineffective, systemic therapy was initiated promptly. Treatment with pegylated interferon alpha-2b 25 µg/week led to significant improvement of the skin condition.
CONCLUSION
Due to its polymorphic clinical presentation, folliculotropic mycosis fungoides is diagnosed with delay and in a later stage than classical mycosis fungoides. Thus we recommend an early skin biopsy in patients with therapy-resistant pruritic skin lesions.
Topics: Antineoplastic Agents; Dermatitis, Atopic; False Positive Reactions; Humans; Interferon alpha-2; Interferon-alpha; Male; Middle Aged; Mycosis Fungoides; Polyethylene Glycols; Recombinant Proteins; Skin Neoplasms; Treatment Outcome
PubMed: 25355498
DOI: 10.1007/s00105-014-3539-z -
Annals of Diagnostic Pathology Aug 2020Mycosis Fungoides (MF) is known as 'the great mimicker' due to its capacity to emulate several dermatoses, both in the clinic and on histology. This often leads to the... (Review)
Review
Mycosis Fungoides (MF) is known as 'the great mimicker' due to its capacity to emulate several dermatoses, both in the clinic and on histology. This often leads to the diagnosis being missed or delayed, which consequently leads to poorer prognosis. For a timely diagnosis, it is crucial that the physician is aware of the various clinical and histological presentations of MF, as well as the proper diagnostic protocols. In the current review, we concisely encapsulate all the variants of MF as well has the conditions it mimics clinically and histologically. Through this, we aim to provide clinicians with a holistic picture of MF and help them determine when to suspect this disease and steps to take in order to nail the diagnosis.
Topics: Abscess; Biopsy; Dermatology; Diagnosis, Differential; Female; High Mobility Group Proteins; Humans; Lymphocytes; Lymphoma, T-Cell, Cutaneous; Male; Middle Aged; Mycosis Fungoides; Neoplasm Staging; Pathology; Practice Guidelines as Topic; Skin Diseases; Skin Neoplasms
PubMed: 32554312
DOI: 10.1016/j.anndiagpath.2020.151546 -
Journal of the American Academy of... Dec 2015Leonine facies (LF) is defined as displaying facial features similar to that of a lion with prominent convexities and furrowed creases. LF develops in a very small... (Review)
Review
BACKGROUND
Leonine facies (LF) is defined as displaying facial features similar to that of a lion with prominent convexities and furrowed creases. LF develops in a very small population of patients with cutaneous T-cell lymphoma.
OBJECTIVE
We aimed to study the clinicopathologic features and overall prognosis associated with LF in patients with mycosis fungoides and Sézary syndrome.
METHODS
We conducted a single-center retrospective study, reviewing 1338 patients with mycosis fungoides seen from 1987 to 2015 at a tertiary referral center for cutaneous T-cell lymphoma, and a systematic review of 14 patients in the literature.
RESULTS
We identified 10 patients with mycosis fungoides who developed LF. Folliculotropism was seen in all patients with LF who had facial biopsy specimens. Radiation was a beneficial therapy. Complete remission was achieved in 1 patient and overall 5-year survival was 26%. Systematic review of 10 additional patients showed that all patients with LF, including ours, had stage-IV disease and some degree of blood involvement, but not all met criteria for Sézary syndrome.
LIMITATIONS
This was a retrospective study with a small sample size.
CONCLUSION
LF is associated with stage-IV cutaneous T-cell lymphoma, is often accompanied by folliculotropism and blood involvement, and can be treated with local electron beam therapy.
Topics: Adult; Aged; Biopsy, Needle; Cancer Care Facilities; Cohort Studies; Combined Modality Therapy; Facies; Female; Follow-Up Studies; Humans; Immunohistochemistry; Lymphoma, T-Cell, Cutaneous; Male; Middle Aged; Mycosis Fungoides; Neoplasm Invasiveness; Neoplasm Staging; Positron-Emission Tomography; Retrospective Studies; Risk Assessment; Skin Neoplasms; Survival Rate; Tertiary Care Centers; Treatment Outcome
PubMed: 26476898
DOI: 10.1016/j.jaad.2015.09.017 -
Giornale Italiano Di Dermatologia E... Apr 2017Cutaneous T-cell lymphoma (CTCL) is a rare but increasing malignancy whose protean manifestations necessarily present in the integument, but can also spread to involve... (Review)
Review
Cutaneous T-cell lymphoma (CTCL) is a rare but increasing malignancy whose protean manifestations necessarily present in the integument, but can also spread to involve blood, lymph nodes and internal organs. We have developed efficacious and varied therapies to treat early and advanced stage disease, but there are still many who suffer tremendously from this illness. Although the pathogenesis of this cancer remains frustratingly elusive, over the last 200 years we have generated a robust body of evidence that points toward possible singular as well as multifactorial etiologies. Combining the historical hypotheses which have focused upon the concept of infectious causes, including carcinogenic genomic viral integration and bacterial superantigenic chronic stimulation as well as industrial/occupational exposure, along with the more recent revelations of both genetic and epigenetic alteration and immune dysregulation, we are closer than ever to understanding the etiology of CTCL. It is through this knowledge and continued research efforts that we will be able to better diagnose, treat, and potentially prevent or cure CTCL.
Topics: Epigenesis, Genetic; Humans; Lymphatic Metastasis; Lymphoma, T-Cell, Cutaneous; Mycosis Fungoides; Neoplasm Staging; Skin Neoplasms
PubMed: 27982549
DOI: 10.23736/S0392-0488.16.05527-9 -
International Journal of Molecular... Nov 2021Mycosis fungoides (MF) and Sézary syndrome (SS), the most common types of cutaneous T-cell lymphoma (CTCL), are characterized by proliferation of mature CD4+ T-helper...
Mycosis fungoides (MF) and Sézary syndrome (SS), the most common types of cutaneous T-cell lymphoma (CTCL), are characterized by proliferation of mature CD4+ T-helper cells. Patients with advanced-stage MF and SS have poor prognosis, with 5-year survival rates of 52%. Although a variety of systemic therapies are currently available, there are no curative options for such patients except for stem cell transplantation, and thus the treatment of advanced MF and SS still remains challenging. Therefore, elucidation of the pathophysiology of MF/SS and development of medical treatments are desired. In this study, we focused on a molecule called OX40. We examined OX40 and OX40L expression and function using clinical samples of MF and SS and CTCL cell lines. OX40 and OX40L were co-expressed on tumor cells of MF and SS. OX40 and OX40L expression was increased and correlated with disease severity markers in MF/SS patients. Anti-OX40 antibody and anti-OX40L antibody suppressed the proliferation of CTCL cell lines both in vitro and in vivo. These results suggest that OX40-OX40L interactions could contribute to the proliferation of MF/SS tumor cells and that the disruption of OX40-OX40L interactions could become a new therapeutic strategy for the treatment of MF/SS.
Topics: Antibodies, Anti-Idiotypic; Antigens, Differentiation; Cell Line, Tumor; Cell Proliferation; Gene Expression Regulation, Neoplastic; Humans; Lymphoma, T-Cell, Cutaneous; Mycosis Fungoides; OX40 Ligand; Sezary Syndrome
PubMed: 34830466
DOI: 10.3390/ijms222212576 -
Pediatric Dermatology Jan 2020Mycosis fungoides (MF) is the most common primary cutaneous lymphoma in pediatric patients. Given the indolent nature of MF, symptoms often present in childhood but may... (Review)
Review
Mycosis fungoides (MF) is the most common primary cutaneous lymphoma in pediatric patients. Given the indolent nature of MF, symptoms often present in childhood but may not be diagnosed as MF until adulthood. Delayed diagnosis is associated with poor long-term prognosis. Thus, increased clinician recognition and accurate diagnosis of early-stage MF in pediatric patients is critically important. In this review, we summarize the clinical features of the most common pediatric MF subtypes and highlight important differences between pediatric and adult MF. Moreover, we reviewed all pediatric MF case series published between 2008 and 2018 to analyze treatment modalities and identify emerging therapies. As treatment of pediatric MF is complex, selection of therapy varies significantly depending upon the specific clinical characteristics, disease severity, and patients' preferences.
Topics: Biopsy; Child; Diagnosis, Differential; Humans; Mycosis Fungoides; Neoplasm Staging; Prognosis; Skin Neoplasms
PubMed: 31630432
DOI: 10.1111/pde.14026 -
Indian Journal of Dermatology,...Background Follicular mycosis fungoides is a distinct variant of mycosis fungoides with a broad clinical spectrum. Recently, many studies have indicated that follicular...
Background Follicular mycosis fungoides is a distinct variant of mycosis fungoides with a broad clinical spectrum. Recently, many studies have indicated that follicular mycosis fungoides should be divided into different subtypes with disparate prognoses. Objective To define the clinicohistopathologic features and outcomes of follicular mycosis fungoides and to identify risk factors that may be related to the prognosis of Chinese patients with follicular mycosis fungoides. Materials and methods We conducted a single-centre retrospective study and reviewed the clinical, histopathologic and immunophenotypic data of 12 patients diagnosed with follicular mycosis fungoides between 2009 and 2020 in the Department of Dermatology of West China Hospital of Sichuan university. Results A total of 12 patients (seven males and five females) with a mean age of 30 ± 14 years (age range 16-55 years) were included. Scalp and face were the most common involved sites (100%). Follicular papules, acneiform lesions, plaques, and nodules, were the main clinical presentations. Histopathological findings were consistent with the classic manifestations of follicular mycosis fungoides, including folliculotropism, perifollicular and intrafollicular lymphocytic infiltrates and mucinous degeneration. Interferon α-1b was the most common treatment. Four patients died of follicular mycosis fungoides in three years. Notably, immunohistochemical analysis revealed a decreased number of CD20+ cells in the deceased patients. Limitations This is a retrospective evaluation with a small number of cases; further prospective studies are warranted to support our inferences. Conclusion Our patients were much younger than in previous studies. The observed difference in this cohort may be explained by race, in addition to the limited number of cases. A decreased number of B cells might be associated with a poor prognosis, and more studies are necessary to discover the role of B cells in follicular mycosis fungoides as well as in mycosis fungoides.
Topics: Male; Female; Humans; Adolescent; Young Adult; Adult; Middle Aged; Retrospective Studies; Skin Neoplasms; Mycosis Fungoides; Prognosis; China
PubMed: 37317772
DOI: 10.25259/IJDVL_1003_2021