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Frontiers in Oncology 2023Mycosis fungoides (MF) and Sézary syndrome (SS) are cutaneous T-cell lymphomas. MF is the most common cutaneous lymphoma, and it is classified into classic... (Review)
Review
Mycosis fungoides (MF) and Sézary syndrome (SS) are cutaneous T-cell lymphomas. MF is the most common cutaneous lymphoma, and it is classified into classic Alibert-Bazin MF, folliculotropic MF, pagetoid reticulosis, and granulomatous slack skin, each with characteristic clinical presentation, histopathological findings, and distinct clinical behaviors. SS is an aggressive leukemic variant of cutaneous lymphoma, and it is characterized by erythroderma, lymphadenopathy, and peripheral blood involvement by malignant cells. There is a wide range of dermatological manifestations of MF/SS, and prompt recognition is essential for early diagnosis. Skin biopsy for histopathology and immunohistochemical analysis is imperative to confirm the diagnosis of MF/SS. Histopathology may also provide information that may influence prognosis and treatment. Staging follows the TNMB system. Besides advanced stage, other factors associated with poorer prognosis are advanced age, male gender, folliculotropism in histopathology of patients with infiltrated plaques and tumors in the head and neck region, large cell transformation, and elevated lactate dehydrogenase. Treatment is divided into skin-directed therapies (topical treatments, phototherapy, radiotherapy), and systemic therapies (biological response modifiers, targeted therapies, chemotherapy). Allogeneic bone marrow transplantation and extracorporeal photopheresis are other treatment modalities used in selected cases. This review discusses the main clinical characteristics, the histopathological/immunohistochemical findings, the staging system, and the therapeutic management of MF/SS.
PubMed: 37124514
DOI: 10.3389/fonc.2023.1141108 -
Healthcare (Basel, Switzerland) Feb 2023Mycosis fungoides is the most common primary cutaneous T-cell lymphoma, characterized by skin-homing CD4+ T cells derivation, indolent course, and low-grade of... (Review)
Review
Mycosis fungoides is the most common primary cutaneous T-cell lymphoma, characterized by skin-homing CD4+ T cells derivation, indolent course, and low-grade of malignancy. Mycosis fungoides's classic type typically onsets with cutaneous erythematous patches, plaque, and tumor. In WHO-EORTC classification, folliculotropic mycosis fungoides, pagetoid reticulosis, and granulomatous slack skin are recognized as distinct variants of mycosis fungoides, because of their clinical and histological features, behavior, and /or prognosis. Mycosis fungoides often shows diagnostic difficulties, due to its absence of specific features and lesional polymorphism. A patient's treatment requires staging. In about 10% of cases, mycosis fungoides can progress to lymph nodes and internal organs. Prognosis is poor at advanced stage and management needs a multidisciplinary team approach. Advanced stage disease including tumors, erythroderma, and nodal, visceral, or blood involvement needs skin directed therapy associated with systemic drugs. Skin directed therapy includes steroids, nitrogen mustard, bexarotene gel, phototherapy UVB, and photochemiotherapy, i.e., total skin electron radiotherapy. Systemic therapies include retinoids, bexarotene, interferon, histone deacetylase inhibitors, photopheresis, targeted immunotherapy, and cytotoxic chemotherapy. Complexity of mycosis fungoides associated with long-term chronic evolution and multiple therapy based on disease stage need a multidisciplinary team approach to be treated.
PubMed: 36833148
DOI: 10.3390/healthcare11040614 -
Anais Brasileiros de Dermatologia 2013Ketron-Goodman disease was formerly considered a disseminated type of pagetoid reticulosis. However, according to the new classification consensus, it should be regarded...
Ketron-Goodman disease was formerly considered a disseminated type of pagetoid reticulosis. However, according to the new classification consensus, it should be regarded as aggressive epidermotropic CD8 T-cutaneous lymphoma, cutaneous gamma/delta T-lymphoma, or tumor-stage mycosis fungoides, depending on the clinical-histological picture. This case highlights a rare and challenging presentation of Ketron-Goodman disease with an indolent presentation and evolution and good response to a low-grade treatment regimen, not fitting well into the new classification criteria.
Topics: Biopsy; Humans; Lymphoma, T-Cell, Cutaneous; Male; Middle Aged; Pagetoid Reticulosis; Skin Neoplasms; Time Factors; Treatment Outcome
PubMed: 24173195
DOI: 10.1590/abd1806-4841.20132258 -
Diagnostic Histopathology (Oxford,... Apr 2016Conventional presentations of mycosis fungoides may be diagnostically challenging, particularly in light of the controversial boundaries defining the disease. Variant...
Conventional presentations of mycosis fungoides may be diagnostically challenging, particularly in light of the controversial boundaries defining the disease. Variant presentations of this cutaneous T-cell lymphoma add a further layer of complexity, requiring a sophisticated and informed perspective when evaluating lymphoid infiltrates in the skin. Herein we discuss well-defined (WHO-EORTC) variants pagetoid reticulosis, granulomatous slack skin and folliculotropic mycosis fungoides as well as less well-defined morphologic/architectural variants, and divergent immunohistochemical presentations of this typically indolent T-cell lymphoproliferative disease.
PubMed: 29225700
DOI: 10.1016/j.mpdhp.2016.04.004 -
Romanian Journal of Morphology and... 2021Neoplasms with pagetoid features are a category of rare lesions defined by the presence of atypical cells at different levels of the epidermis. The most important...
INTRODUCTION
Neoplasms with pagetoid features are a category of rare lesions defined by the presence of atypical cells at different levels of the epidermis. The most important diseases within this category are mammary Paget disease (MPD), extramammary Paget disease (EMPD), Bowen's disease, in situ melanoma, and pagetoid reticulosis.
AIM
The aim of this analysis was to describe the importance of the cytokeratin 8∕18 (CK 8∕18) immunostaining in diagnosing MPD and EMPD and differentiating them from other lesions.
MATERIALS AND METHODS
A retrospective study was employed, based on the histopathological and immunohistochemical (IHC) characteristics of 30 cases that presented pagetoid features. The cases were processed and analyzed at the Department of Pathology, Mureş Clinical County Hospital, Târgu Mureş, Romania, from 2017 to 2020.
RESULTS
Five MPD cases, one EMPD case, one pagetoid reticulosis case, 10 Bowen's disease cases, and 13 in situ melanoma cases were collected. Under Hematoxylin-Eosin staining, cells presented pale cytoplasm in MPD, EMPD, and in 25% of the melanoma cases. Hyperchromasia with nuclear enlargement was seen in all cases. Immunostaining with CK 8∕18 was positive in all MPD and EMPD cases. Tests for CK7, p63, and CK AE1∕AE3 were positive in MPD, EMPD, and Bowen's cases. Tests for S100, SRY-box transcription factor 10 (SOX10), human melanoma black 45 (HMB45), and Melan A were positive in melanoma cases, while cluster of differentiation (CD)3, CD4, and CD8 tests were positive in the pagetoid reticulosis case.
CONCLUSIONS
CK 8∕18 is an IHC marker that can help establish the diagnosis of MPD and EMPD and differentiate them from other pagetoid neoplasms, ensuring the proper diagnosis and prognosis are provided.
Topics: Biomarkers; Biomarkers, Tumor; Bowen's Disease; Diagnosis, Differential; Humans; Keratin-18; Keratin-8; Retrospective Studies; Skin Neoplasms
PubMed: 35263404
DOI: 10.47162/RJME.62.3.13 -
Cureus Oct 2021Pagetoid reticulosis is a rare form of cutaneous T cell lymphoma, a malignancy of T lymphocytes, that invades the skin leading to a multitude of dermatologic...
Pagetoid reticulosis is a rare form of cutaneous T cell lymphoma, a malignancy of T lymphocytes, that invades the skin leading to a multitude of dermatologic manifestations. Pagetoid reticulosis commonly presents as a localized slow-growing or indolently manifesting hyperkeratotic patch/plaque on the extremities that is confined to the epidermis. Diagnosis is confirmed via a skin biopsy of the affected area followed by a cytologic examination. Treatment typically entails topical corticosteroids, alkylating agents, and retinoids. With disseminated disease, a multidisciplinary approach involving chemotherapy and radiation is necessary. To raise awareness about pagetoid reticulosis, its diagnosis, and management, we report a case localized to the left forehead and earlobe of a 57-year-old female.
PubMed: 34754679
DOI: 10.7759/cureus.18524 -
Dermatology Online Journal Jan 2008Pagetoid reticulosis of Woringer-Kolopp is a rare form of cutaneous T-cell lymphoma that primarily affects middle-aged males. It is characterized by the presence of one...
Pagetoid reticulosis of Woringer-Kolopp is a rare form of cutaneous T-cell lymphoma that primarily affects middle-aged males. It is characterized by the presence of one or several scaly patches and plaques with an acral distribution. We present a case of a 58-year-old woman, otherwise healthy, with a 5-month history of asymptomatic, hyperkeratotic plaques on the hands and feet. Histological and immunohistochemical analysis confirmed the diagnosis. Nearly complete spontaneous regression was noted 7 months after the initial examination. After 5 years no evidence of the disease remained.
Topics: Female; Foot Diseases; Hand; Humans; Immunohistochemistry; Lymphoma, T-Cell, Cutaneous; Middle Aged; Skin Neoplasms
PubMed: 18319035
DOI: No ID Found -
Dermatopathology (Basel, Switzerland) 2016Pagetoid reticulosis is a rare variant of mycosis fungoides that presents with a large, usually single, erythematous, slowly growing scaly plaque containing an...
Pagetoid reticulosis is a rare variant of mycosis fungoides that presents with a large, usually single, erythematous, slowly growing scaly plaque containing an intraepidermal proliferation of neoplastic T lymphocytes. Histopathologically, this disease has distinctive attributes. In this report, we present two cases of pagetoid reticulosis, compare its microscopic features to those of 'classical' mycosis fungoides, and provide a brief review of the pertinent literature.
PubMed: 27195265
DOI: 10.1159/000444660 -
Cureus Jan 2022Background Mycosis fungoides (MF) is one of the primary cutaneous T-cell lymphomas and is considered to be the most common extranodal non-Hodgkin lymphomas. MF is...
Background Mycosis fungoides (MF) is one of the primary cutaneous T-cell lymphomas and is considered to be the most common extranodal non-Hodgkin lymphomas. MF is characterized by different subtypes based on clinical presentation and immunophenotyping studies. We aimed to study the clinical patterns and treatment response in cases of MF among the patients attending a tertiary referral hospital in Saudi Arabia. Methodology A retrospective study, case record-based study was done to review all the patients diagnosed with MF from January 2011 to May 2016. All cases with histopathological confirmation and immunophenotyping were included in the study. Treatment follow-up was reviewed for 9 months in all cases. Treatment response was graded based on a global physician assessment-complete response, good response, moderate response, and minimal or no response. Results Out of 34 cases of MF included in the study, 11 were hyperpigmented MF, 21 were hypopigmented MF, and there was one case each of poikilodermatous MF and pagetoid reticulosis. Of the total, fourteen (66.7%) of hypopigmented MF patients showed a complete response to phototherapy Narrowband UVB (NB-UVB) in combination with topical corticosteroids. Nine (81.8%) of hyperpigmented MF patients showed partial to the phototherapy NB-UVB in combination with the topical corticosteroid. Among the other types; one case of poikilodermatous MF (2.9%) showed a moderate response to phototherapy NB-UVB with topical corticosteroid and systemic acitretin. Conclusions The most common type of MF seen in our study was the hypopigmented type, affecting a younger age group, and the same showed a good response to phototherapy NB-UVB combined with topical corticosteroids.
PubMed: 35174031
DOI: 10.7759/cureus.21231