RESUMO
BACKGROUND: Narrowband ultraviolet B (NB-UVB) phototherapy is a popular and relatively contemporary treatment option. However, only a few studies to date have explored the potential risk of skin cancer following NB-UVB treatment. OBJECTIVE: This study aimed to investigate the potential long-term risk of skin cancer in patients treated with NB-UVB. METHODS: This cohort study included patients with psoriasis, vitiligo, and mycosis fungoides treated with NB-UVB at two university hospitals in Israel in 2000-2005. Patients were followed up for skin cancer for at least 10 years. Data were extracted from the hospital and community medical records. RESULTS: A total of 767 patients were included in this study: 509 with psoriasis, 122 with vitiligo, and 136 with mycosis fungoides. The mean follow-up duration was 13 years. Among these patients, 4.43% developed skin cancer during the follow-up (3.93% had psoriasis, 2.46% had vitiligo, and 8.09% had mycosis fungoides). Old age and fair skin type were the only significant independent risk factors for skin cancer. There was no significant difference in the mean number of NB-UVB treatments among patients who developed skin cancer and those who did not (99.09 vs. 94.79, respectively). CONCLUSION: No association was observed between the number of NB-UVB treatments and carcinogenesis in any study group. Age is a significant risk factor, and older patients treated with NB-UVB should be followed up carefully.
Assuntos
Micose Fungoide , Psoríase , Neoplasias Cutâneas , Terapia Ultravioleta , Vitiligo , Humanos , Vitiligo/epidemiologia , Vitiligo/terapia , Estudos de Coortes , Terapia Ultravioleta/efeitos adversos , Psoríase/epidemiologia , Psoríase/radioterapia , Psoríase/complicações , Neoplasias Cutâneas/etiologia , Micose Fungoide/epidemiologia , Micose Fungoide/radioterapia , Fototerapia/efeitos adversos , Resultado do TratamentoRESUMO
Mycosis fungoides is a rare cutaneous lymphoma in the paediatric population. The aim of this study was to examine the epidemiological, clinical, and histological characteristics, as well as the treatment modalities and response to therapy of paediatric patients with mycosis fungoides. This retrospective cohort study reviewed the records of 37 paediatric patients treated at Rambam Medical Center, Israel, between 2013 and 2021. Extracted data included epidemiology, clinical presentation, histological reports, infiltrate clonality status, treatment modalities and response to therapy. The mean follow-up period was 60 months. All patients were diagnosed with stage IA or IB disease. Folliculotropic mycosis fungoides was the most prevalent variant (49%). Most patients were treated with phototherapy (90%), with a response rate of 85%, and a complete response rate of 55% after the first course. There were no significant differences in response to phototherapy between the folliculotropic or other variants (p = 0.072). Similarly, delayed diagnosis, atopic diathesis, clonality, phototherapy type or number of treatments, were not associated with response to therapy, while protracted phototherapy was associated with prolonged remission. In conclusion, mycosis fungoides in the paediatric population is an indolent disease with a favourable prognosis and potentially prolonged response to phototherapy.
Assuntos
Linfoma Cutâneo de Células T , Micose Fungoide , Neoplasias Cutâneas , Humanos , Criança , Estudos Retrospectivos , Resultado do Tratamento , Micose Fungoide/epidemiologia , Micose Fungoide/terapia , Micose Fungoide/diagnóstico , Neoplasias Cutâneas/epidemiologia , Neoplasias Cutâneas/terapia , Neoplasias Cutâneas/diagnóstico , Linfoma Cutâneo de Células T/patologiaRESUMO
BACKGROUND: Mycosis fungoides (MF) is the most common cutaneous T-cell lymphoma. Skin-directed therapies, including phototherapy, are the first-line treatment modalities. Psoralen plus ultraviolet A light photochemotherapy (PUVA) is quite effective in controlling the disease; however, long-term adverse effects, particularly carcinogenesis, are the cons of this treatment. OBJECTIVE: There are various studies on the negative impact of PUVA on skin cancer in patients with autoimmune skin diseases. The data on the long-term effects of phototherapy on MF patients are scarce. METHODS: All MF cases that received PUVA alone or combined with other treatments at a single tertiary center were analyzed. This study compared the development of non-melanoma skin cancers, melanoma, and solid organ tumors in MF patients with at least 5-year follow-up data with age- and sex-matched controls. RESULTS: A total of 104 patients were included in the study. Ninety-two malignancies were detected in 16 (15.4%) patients, and six developed multiple malignancies. Skin cancers consisted of 56 basal cell carcinomas, 16 Bowen's disease, four squamous cell carcinomas, three melanomas, two basosquamous cell carcinomas, one Kaposi sarcoma, and one keratoacanthoma were found in nine (8.7%) patients. Eight patients developed three solid cancers and six lymphomas. The risk of developing skin cancer was associated with the total number of PUVA sessions (<250 vs ≥250 sessions; hazard ratio (HR) 4.44, 95% confidence interval (CI) 1.033-19.068; p = .045). 9 (13.2%) of 68 patients who had follow-ups for at least 5 years developed skin cancer. Compared to an age- and sex-matched cohort, the prevalence of new skin cancer was considerably greater (p = .009). CONCLUSIONS: Patients with MF are predisposed to develop secondary malignancies, and continual exposure to PUVA may potentiate this risk. Annual digital dermoscopic follow-up in MF patients treated with UVA is advised for early diagnosis and treatment of secondary cutaneous malignancies.
Assuntos
Micose Fungoide , Fotoquimioterapia , Neoplasias Cutâneas , Humanos , Terapia PUVA/efeitos adversos , Micose Fungoide/tratamento farmacológico , Micose Fungoide/epidemiologia , Neoplasias Cutâneas/epidemiologia , Neoplasias Cutâneas/etiologia , Neoplasias Cutâneas/diagnóstico , FototerapiaRESUMO
Mycosis fungoides (MF) is the most common type of cutaneous T-cell lymphoma. However, it is rare in pediatric population. Most of the cases of pediatric MF present with hypopigmented patches and/or various other forms, which may often mimic common childhood dermatoses, thereby causing a delay in the diagnosis. There are no established treatment guidelines for pediatric MF. As the progression of childhood MF is extremely rare and it has an indolent course, it is usually diagnosed at an early stage (IA, IB, IIA), and hence phototherapy with a response rate of >80% is a well-established effective treatment in children. However, as recurrences are frequently seen on stopping the therapies, a maintenance regimen and long-term follow-up is equally important. This article reviews the epidemiological factors, clinical presentations, diagnosis, and various treatment modalities used in pediatric MF. We analyzed and compared the data of almost 616 childhood MF cases from various studies undertaken from 1988 to 2021.
Assuntos
Hipopigmentação , Linfoma Cutâneo de Células T , Micose Fungoide , Neoplasias Cutâneas , Criança , Humanos , Neoplasias Cutâneas/diagnóstico , Neoplasias Cutâneas/epidemiologia , Neoplasias Cutâneas/terapia , Micose Fungoide/diagnóstico , Micose Fungoide/epidemiologia , Micose Fungoide/terapia , Linfoma Cutâneo de Células T/patologia , FototerapiaRESUMO
BACKGROUND AND OBJECTIVES: Primary cutaneous lymphomas (PCL) often strongly differ in clinical behavior and prognosis from systemic lymphomas of the same histopathologic type. The aim of the study was to investigate the distribution of PCL subtypes, the average time from disease manifestation to diagnosis, the importance of diagnostic procedures, the occurrence of secondary malignancies and the different treatment modalities. PATIENTS AND METHODS: Retrospective analysis of 152 patients with PCL examined at the Department of Dermatology of the University Hospital Tübingen from 2010-2012. RESULTS: 105 patients with CTCL (69.1 %) and 47 patients with CBCL (30.9 %) were included. The average time from disease manifestation to diagnosis was four years. The most common diagnosed lymphoma was mycosis fungoides (MF) (47.4 %). First-line therapies here include phototherapy only (psoralen-UV-A [PUVA], n = 48; UVB 311 nm, n = 7) or combination therapies primarily phototherapy with systemic retinoids (n = 18). Most frequent second-line therapy was interferon (INF)-α plus PUVA (n = 15). The outcome was favorable (45.2 % remission, 28.6 % stable disease, 22.6 % progressive disease). Malignant comorbidities were observed more frequently compared to a healthy control group. CONCLUSIONS: The diagnosis of lymphoma often takes several years. The value of staging procedures is still low and the treatment modalities for MF in earlier stages are mainly based on phototherapy.
Assuntos
Micose Fungoide , Neoplasias Cutâneas , Humanos , Micose Fungoide/diagnóstico , Micose Fungoide/epidemiologia , Micose Fungoide/terapia , Terapia PUVA , Fototerapia , Estudos Retrospectivos , Neoplasias Cutâneas/diagnóstico , Neoplasias Cutâneas/epidemiologia , Neoplasias Cutâneas/terapiaRESUMO
OBJECTIVE: To describe the patient population with mycosis fungoides (MF) in Puerto Rico in terms of demographics, disease course, and management. METHODS: We performed a retrospective chart review of patients with MF who were being followed at the University of Puerto Rico dermatology clinics from 1999 through 2016. Collected data included demographics, age at diagnosis, stage at diagnosis, follow-up time, treatment, and stage at the time of the study. RESULTS: A total of 53 patients were diagnosed with MF from 1999 through 2016, with a mean follow-up period of 89 months. Of those, 45% were male. At the time of diagnosis, 40% were at stage 1A, 53% were at stage 1B, and 7% were at stages 2 to 4. During data collection, 74% of the patients remained stable, 18% improved clinically, and 8% progressed in disease stage. The treatment modalities used included phototherapy, topical steroids, topical and systemic retinoids, methotrexate, topical and systemic chemotherapy, and interferon. CONCLUSION: Our study reflects the chronic and indolent course of MF, which has an overall good prognosis if diagnosed at an early stage, as has been demonstrated in the recent literature. The information contained within this manuscript should contribute to the understanding and characterization of MF in patients in Puerto Rico.
Assuntos
Micose Fungoide/epidemiologia , Neoplasias Cutâneas/epidemiologia , Adulto , Idoso , Progressão da Doença , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Micose Fungoide/diagnóstico , Micose Fungoide/terapia , Estadiamento de Neoplasias , Prognóstico , Porto Rico/epidemiologia , Estudos Retrospectivos , Neoplasias Cutâneas/diagnóstico , Neoplasias Cutâneas/terapia , Adulto JovemRESUMO
Patients with mycosis fungoides (MF) are thought to be at increased risk of melanoma. However, studies addressing surveillance-bias and treatments as a possible confounder are lacking. This retrospective study compared the prevalence and risk of melanoma between 982 patients with MF, and 3,165 patients with psoriasis attending tertiary cutaneous-lymphoma/psoriasis clinics during 2009 to 2018. Melanoma was diagnosed in 47 patients with MF (4.8%; 43 early-stage) and in 23 patients with psoriasis (0.7%) (odds ratio 6.6, p < 0.0001). In 60% of patients, MF/psoriasis preceded melanoma diagnosis. Hazard ratio (HR) for a subsequent melanoma in MF vs psoriasis was 6.3 (95% confidence interval (95% CI) 3.4-11.7, p < 0.0001). Compared with the general population, melanoma standardized incidence ratios were 17.5 in patients with MF (95% CI 11.0-23.9, p < 0.0001), and 2.2 (95% CI 0.6-3.8, p = 0.148) in patients with psoriasis. Narrow-band ultraviolet B was not a contributory factor (HR 1.15, 95% CI 0.62-2.14, p = 0.66). These findings add evidence that patients with MF have a significantly higher risk of melanoma, not only compared with the general population, but also compared with patients with psoriasis. This comorbidity may be inherent to MF.
Assuntos
Melanoma , Micose Fungoide , Psoríase , Neoplasias Cutâneas , Humanos , Melanoma/diagnóstico , Melanoma/epidemiologia , Micose Fungoide/diagnóstico , Micose Fungoide/epidemiologia , Psoríase/diagnóstico , Psoríase/epidemiologia , Estudos Retrospectivos , Neoplasias Cutâneas/diagnóstico , Neoplasias Cutâneas/epidemiologia , Neoplasias Cutâneas/terapiaAssuntos
Micose Fungoide/epidemiologia , Micose Fungoide/patologia , Neoplasias Cutâneas/epidemiologia , Neoplasias Cutâneas/patologia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Antimetabólitos Antineoplásicos/uso terapêutico , Eosinofilia/epidemiologia , Feminino , Glucocorticoides/uso terapêutico , Humanos , L-Lactato Desidrogenase/sangue , Masculino , Metotrexato/uso terapêutico , Pessoa de Meia-Idade , Marrocos/epidemiologia , Micose Fungoide/terapia , Fototerapia , Indução de Remissão , Estudos Retrospectivos , Neoplasias Cutâneas/terapia , Adulto JovemRESUMO
BACKGROUND: Hypopigmented mycosis fungoides (hMF) is a rare subtype of mycosis fungoides. The aim of this study was to identify the clinical-epidemiological profile of our patient group and also to provide additional information about treatment responses and prognosis. METHODS: This is a cross-sectional retrospective observational study, with exploratory analysis. The outcome variables were disease progression and related death. RESULTS: Twenty patients with hMF were selected from a group of 102 patients diagnosed with MF. There was no gender difference (10 females and 10 males). Mean age at diagnosis was 43.85 years, and most patients had mixed or black skin color. The mean time between the onset of the lesions and the diagnosis was 66.75 months. Patients were equally distributed in stages IA (50%) and IB (50%). Photochemotherapy (psoralen and ultraviolet A) was the predominant therapeutic modality. The mean follow-up time was 7.25 years. In 10%, disease progression was observed. Death related to the disease occurred in one patient. CONCLUSIONS: The clinical and epidemiological profile of patients with hypopigmented MF found in our sample is in agreement with what is described in the literature, with the exception of the age at diagnosis, higher than expected. Diagnostic delay time, despite long, is also consistent with the medical literature; however, in this sample, we had two cases of disease progression, with death of one patient, despite the treatment, which is extremely important since hypopigmented MF is usually associated with good prognosis.
Assuntos
Hipopigmentação/diagnóstico , Hipopigmentação/tratamento farmacológico , Micose Fungoide/diagnóstico , Micose Fungoide/tratamento farmacológico , Adolescente , Adulto , Idade de Início , Idoso , Brasil/epidemiologia , Criança , Estudos Transversais , Diagnóstico Tardio , Progressão da Doença , Feminino , Seguimentos , Humanos , Hipopigmentação/complicações , Hipopigmentação/epidemiologia , Masculino , Pessoa de Meia-Idade , Micose Fungoide/complicações , Micose Fungoide/epidemiologia , Terapia PUVA , Estudos Retrospectivos , Taxa de Sobrevida , Adulto JovemRESUMO
La micosis fungoide (MF) es el linfoma cutáneo primario de células T más frecuente. Su aparición en la infancia es excepcional. Objetivos: Describir las características epidemiológicas, clínicas, histopatológicas e inmunofenotípicas de los pacientes con MF. Describir los tratamientos utilizados y la evolución. Material y método: Se incluyeron todos los pacientes admitidos en el Hospital de Pediatría Dr. J. P. Garrahan (Argentina) en el período comprendido entre agosto de 1988 y julio de 2014 con diagnóstico clínico e histopatológico de MF. Resultados: Se diagnosticaron 14 pacientes con MF. La distribución por sexo fue M/F: 1:1,33. La edad media al diagnóstico fue de 11,23 años (rango: 8 a 15 años). El tiempo promedio de evolución hasta el momento del diagnóstico fue de 3 años y 6 meses (rango: 4 meses a 7 años). Todos los pacientes presentaron la forma clínica hipopigmentada y en el 42% se asoció la forma clásica. El 50% (n = 7) exhibió un inmunofenotipo CD8 positivo de forma exclusiva. El 78% presentó estadio IB. La fototerapia fue el tratamiento de elección. Cuatro pacientes tuvieron por lo menos una recaída y 3 demostraron progresión de su enfermedad a nivel cutáneo. La evolución fue favorable en todos los casos. Conclusiones: La MF es una entidad infrecuente en la infancia, siendo la forma hipopigmentada la más frecuente. Su diagnóstico es tardío debido a la similitud con otras enfermedades hipopigmentadas frecuentes en la niñez. A pesar de tener un buen pronóstico, presenta alta tasa de recidivas y requiere un seguimiento a largo plazo (AU)
Mycosis fungoides (MF), the most common primary cutaneous T-cell lymphoma, is unusual in children. Objectives: We aimed to describe the epidemiologic, clinical, histopathologic, and immunophenotypic characteristics of MF as well as treatments and course of disease in a pediatric case series. Material and method: Data for all patients admitted to our pediatric hospital (Hospital Dr. J. P. Garrahan) in Argentina with a clinical and histopathologic diagnosis of MF between August 1988 and July 2014 were included. Results: A total of 14 patients were diagnosed with MF. The ratio of boys to girls was 1:1.33. The mean age at diagnosis was 11.23 years (range, 8-15 years). The mean time between onset and diagnosis was 3.5 years (range, 4 months-7 years). All patients had hypopigmented MF and 42% also presented the features of classic MF. Seven (50%) had the CD8+ immunophenotype exclusively. Seventy-eight percent were in stage IB at presentation. Phototherapy was the treatment of choice. Four patients relapsed at least once and skin lesions progressed in 3 patients. All patients improved. Conclusions: MF is unusual in children. The hypopigmented form is the most common. Diagnosis is delayed because the condition is similar to other hypopigmented diseases seen more often in childhood. Although prognosis is good, the rate of recurrence is high, so long-term follow-up is necessary (AU)
Assuntos
Humanos , Masculino , Feminino , Criança , Adolescente , Micose Fungoide/epidemiologia , Linfoma Cutâneo de Células T/epidemiologia , Neoplasias Cutâneas/patologia , Distribuição por Idade e Sexo , Micose Fungoide/patologia , Linfoma Cutâneo de Células T/patologia , Progressão da Doença , Estudos Retrospectivos , Pitiríase Liquenoide/patologiaRESUMO
Mycosis fungoides (MF), the most common primary cutaneous T-cell lymphoma, is unusual in children. OBJECTIVES: We aimed to describe the epidemiologic, clinical, histopathologic, and immunophenotypic characteristics of MF as well as treatments and course of disease in a pediatric case series. MATERIAL AND METHOD: Data for all patients admitted to our pediatric hospital (Hospital Dr. J. P. Garrahan) in Argentina with a clinical and histopathologic diagnosis of MF between August 1988 and July 2014 were included. RESULTS: A total of 14 patients were diagnosed with MF. The ratio of boys to girls was 1:1.33. The mean age at diagnosis was 11.23 years (range, 8-15 years). The mean time between onset and diagnosis was 3.5 years (range, 4 months-7 years). All patients had hypopigmented MF and 42% also presented the features of classic MF. Seven (50%) had the CD8+ immunophenotype exclusively. Seventy-eight percent were in stage IB at presentation. Phototherapy was the treatment of choice. Four patients relapsed at least once and skin lesions progressed in 3 patients. All patients improved. CONCLUSIONS: MF is unusual in children. The hypopigmented form is the most common. Diagnosis is delayed because the condition is similar to other hypopigmented diseases seen more often in childhood. Although prognosis is good, the rate of recurrence is high, so long-term follow-up is necessary.
Assuntos
Hospitais Pediátricos , Micose Fungoide/epidemiologia , Neoplasias Cutâneas/epidemiologia , Adolescente , Corticosteroides/uso terapêutico , Idade de Início , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Argentina/epidemiologia , Criança , Estudos Transversais , Diagnóstico Tardio , Erros de Diagnóstico , Feminino , Hospitais Pediátricos/estatística & dados numéricos , Humanos , Hipopigmentação/etiologia , Masculino , Micose Fungoide/tratamento farmacológico , Micose Fungoide/patologia , Micose Fungoide/radioterapia , Terapia PUVA , Recidiva , Estudos Retrospectivos , Dermatopatias/diagnóstico , Neoplasias Cutâneas/tratamento farmacológico , Neoplasias Cutâneas/patologia , Neoplasias Cutâneas/radioterapia , Terapia UltravioletaRESUMO
BACKGROUND: Folliculotropic mycosis fungoides (FMF) is an aggressive variant of mycosis fungoides (MF) and generally less responsive to standard skin-directed therapies (SDTs). Recent studies distinguished indolent (early-stage FMF) and more aggressive (advanced-stage FMF) subgroups. The optimal treatment for both subgroups remains to be defined. OBJECTIVES: To evaluate initial treatment results in patients with early- and advanced-stage FMF. METHODS: A study was undertaken of 203 patients (84 early-stage, 102 advanced-stage, 17 extracutaneous FMF) included in the Dutch Cutaneous Lymphoma Registry between 1985 and 2014. Type and results of initial treatment were retrieved from the Dutch Registry. Main outcomes were complete remission (CR); sustained complete remission; partial remission (PR), > 50% improvement; and overall response (OR; CR + PR). RESULTS: Patients with early-stage FMF were treated with nonaggressive SDTs in 67 of 84 cases resulting, respectively, in CR and OR of 28% and 83% for monotherapy topical steroids, 0% and 83% for ultraviolet B (UVB), and 30% and 88% for psoralen plus ultraviolet A (PUVA). In patients with advanced-stage FMF these SDTs were less effective (combined CR and OR 10% and 52%, respectively). In patients with advanced-stage FMF local radiotherapy (CR 63%; OR 100%), total skin electron beam irradiation (CR 59%; OR 100%) and PUVA combined with local radiotherapy (CR 5%, OR 75%) were most effective. CONCLUSIONS: The results of the present study demonstrate that not all patients with FMF should be treated aggressively. Patients with early-stage FMF may benefit very well from standard SDTs also used in early-stage classic MF and have an excellent prognosis.
Assuntos
Micose Fungoide/terapia , Neoplasias Cutâneas/terapia , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Humanos , Micose Fungoide/epidemiologia , Países Baixos/epidemiologia , Terapia PUVA/estatística & dados numéricos , Sistema de Registros , Neoplasias Cutâneas/epidemiologiaRESUMO
Hypopigmented mycosis fungoides (HMF) is uncommon clinical variant that was commonly observed in dark-skinned individuals. We described the clinical characteristics, pathological features, immunohistochemical profile and prognosis of HMF in Egyptian patients. During the period from January 2004 to December 2011, we were able to diagnose and follow up 27 patients with HMF. The study included 18 males (66.7%) and 9 females (33.3%) with a mean age of 35.39 ±13.13 years. The duration ranged from 1 to 6 years with a mean of 3.26 ±1.7 years. The majority of patients were skin type IV (63%) and presented with multiple (88.9%), asymptomatic (74.1%), ill-defined (70.4%) and non-scaly (77.8%) lesions distributed on the trunk (81.5%). Histologically, epidermotropic lymphocytes were observed in 100%, basal alignment of lymphocytes in 81.5%, Pautrier's microabscesses in 29% and folliculotropism in 18.5%. Immunostaining showed predominance of epidermal CD8+ cells in 51.9% while in 29.6% CD4+ cells were predominant. Phototherapy was effective in 86.7% of patients with success rate 66.7% of narrow band (NB) ultraviolet-B and 80% of psoralen ultraviolet-A. HMF among Egyptians could be classified as non-aggressive epidermotropic cytotoxic CD8+ variant. It is common among middle age males with skin type IV and mostly well respond to phototherapy.
Assuntos
Micose Fungoide/patologia , Transtornos da Pigmentação/patologia , Neoplasias Cutâneas/patologia , Adulto , Comorbidade , Egito/epidemiologia , Feminino , Humanos , Masculino , Micose Fungoide/epidemiologia , Micose Fungoide/terapia , Fototerapia , Transtornos da Pigmentação/epidemiologia , Transtornos da Pigmentação/terapia , Neoplasias Cutâneas/epidemiologia , Neoplasias Cutâneas/terapia , Pigmentação da Pele , Resultado do TratamentoAssuntos
Linfoma de Células B , Linfoma Cutâneo de Células T , Neoplasias Cutâneas , Corticosteroides/uso terapêutico , Diagnóstico Diferencial , Toxidermias/diagnóstico , Humanos , Mordeduras e Picadas de Insetos/diagnóstico , Interferons/uso terapêutico , Linfoma de Células B/diagnóstico , Linfoma de Células B/epidemiologia , Linfoma de Células B/patologia , Linfoma Cutâneo de Células T/diagnóstico , Linfoma Cutâneo de Células T/epidemiologia , Linfoma Cutâneo de Células T/patologia , Micose Fungoide/diagnóstico , Micose Fungoide/epidemiologia , Micose Fungoide/patologia , Micose Fungoide/terapia , Fototerapia , Pseudolinfoma/diagnóstico , Dermatopatias/diagnóstico , Neoplasias Cutâneas/diagnóstico , Neoplasias Cutâneas/epidemiologia , Neoplasias Cutâneas/patologiaRESUMO
Primary cutaneous lymphomas are distinguished by singular clinical, histological and prognostic features that are not shared by other lymphomas originating from the lymphoid tissues. This review is an update of the current concepts about the biology, diagnosis and treatment of mycosis fungoides, the most frequent entity among the primary cutaneous T cell lymphomas. It is currently established that mycosis fungoides originates from T lymphocytes normally homing the dermis. Such a situation implies that treatments at least in the initial stages of the disease, should primarily target the skin without searching for an impact on internal lymphoid tissues.
Assuntos
Micose Fungoide/patologia , Neoplasias Cutâneas/patologia , Administração Cutânea , Bélgica/epidemiologia , Diagnóstico Diferencial , Glucocorticoides/administração & dosagem , Glucocorticoides/uso terapêutico , Humanos , Incidência , Micose Fungoide/epidemiologia , Micose Fungoide/terapia , Estadiamento de Neoplasias , Fototerapia , Prevalência , Prognóstico , Neoplasias Cutâneas/epidemiologia , Neoplasias Cutâneas/terapia , Taxa de Sobrevida , Linfócitos T/patologiaRESUMO
BACKGROUND/OBJECTIVES: There are limited data on the clinical presentation and progression of pediatric cutaneous lymphoma. This study focuses on the clinical characteristics of pediatric patients with mycosis fungoides (MF). MATERIALS AND METHODS: This descriptive study presents clinical characteristics of 22 pediatric patients with MF, enrolled in the international Childhood Registry for Cutaneous Lymphomas (CRCL). RESULTS: The mean ages at onset and at diagnosis were 7.5 (SD 3.8 years) years and 9.9 (SD 3.4) years, respectively. The most common MF presentation was patch stage (68%), followed by hypopigmentation (59%) and plaque stage disease (50%). Epidermotropism and lymphocytic atypia were the most common pathologic features, found in 89% and 85%, respectively. Cerebriform nuclei were noted in 42%, and Pautrier microabscesses were seen in 16% of cases. A cytotoxic pattern was more commonly seen (67% vs 33%), and clonality was detected in 21% (3 of 14) of patients. All patients presented with early-stage disease and received skin-directed therapy (topical steroids, 73%; light therapy, 54%; or combination therapy, 35%). CONCLUSIONS: Pediatric patients with MF present in the first decade of life, with early-stage disease and unusual forms such as hypopigmented variant. Further patient enrollment will provide information regarding natural history, treatment response, and overall prognosis of pediatric cutaneous T-cell lymphoma (CTCL).
Assuntos
Linfoma Cutâneo de Células T/epidemiologia , Micose Fungoide/epidemiologia , Neoplasias Cutâneas/epidemiologia , Austrália/epidemiologia , Canadá/epidemiologia , Criança , Progressão da Doença , Feminino , Humanos , Linfoma Cutâneo de Células T/patologia , Linfoma Cutâneo de Células T/terapia , Masculino , Micose Fungoide/patologia , Micose Fungoide/terapia , Estadiamento de Neoplasias , Fenótipo , Sistema de Registros , Neoplasias Cutâneas/patologia , Neoplasias Cutâneas/terapia , Estados Unidos/epidemiologiaRESUMO
Se realizó un estudio retrospectivo para evaluar cuáles eran las características clínicas y anatomo patológicas para el diagnóstico de los pacientes con micosis fungoides (MF) tratados en el servicio de dermatología del CES Sabaneta entre abril de 1997 y mayo de 2004. Además se buscaba describir las características sociodemográficas locales y contrastarlas con el perfil epidemiológico descrito en la literatura. Materiales y métodos: la muestra estuvo conformada por 43 casos con diagnóstico de MF a los que se les revisó la historia clínica y las placas histológicas fueron evaluadas por dos patólogos separadamente. Resultados: la edad media de los pacientes fue de 57,7 años, la mayoría fueron mujeres de fototipo II. El 58,1% presentó compromiso de la superficie corporal mayor al 11% y la lesión semiológica mas frecuente fue la placa. Las áreas mas afectadas fueron las extremidades inferiores luego la espalda, extremidades superiores y por último abdomen. Sólo 12 pacientes tenían un diagnóstico previo, el más frecuente fue la parapsoriasis de grandes placas. El 71% de los casos se diagnosticó en la primera biopsia. Los hallazgos histológicos más frecuentes entre los dos patólogos fueron: la presencia de infiltrado inflamatorio a nivel papilar y perivascular; epidermotropismo lineal y pagetoide; las microabscesos de Pautrier y la fibrosis de la dermis papilar. Al comparar los diagnósticos entre los dos patólogos, sólo los microabscesos de Pautrier mostraron concordancia.
A retrospective study was made to evaluate clinical characteristics and anatomic pathological for the diagnosis of patients with mycosis fungoides (MF) treated in the dermatology service of the CES Sabaneta from April 1997 until May 2004. In addition, a description of the local socio demographics characteristics were looked for and contrast them with the epidemiologist profile described in literature. Materials and methods: The sample was conformed by 43 cases with diagnose of MF, in which were review their clinical history and the histological plates were evaluated by two pathologists separately. Results: The average age of the patients was of 57, 7 years; the majority was women of phototype II. 58,1% had affected a greater corporal surface above 11% and the frequents semiologic injury was the plate. The more affected areas were the inferior extremities then the back, superior extremities and finally the abdomen. Just 12 patients had previous diagnosis; the most frequent was the parapsoriasis of great plates. In this study 71% of the cases were diagnosed in the first biopsy, the most frequent histologists findings made by the two pathologists were: the presence of infiltrated inflammatory at papillary level and perivascular, linear and pagetoid epidermotropism, the microabscesses of Pautrier and the fibrosis of the papillar dermis. When those findings were compared by the two pathologists through the index of Kappa with a confidence level of 95%, just the microabscesses of Pautrier showed agreement.
Assuntos
Humanos , Diagnóstico Clínico/diagnóstico , Micose Fungoide/diagnóstico , Micose Fungoide/epidemiologia , Patologia/métodos , Patologia/tendênciasRESUMO
BACKGROUND: Cutaneous T-cell lymphoma (CTCL) represents a spectrum of diseases composed of malignant T lymphocytes. The most common type is mycosis fungoides (MF). An accurate diagnosis of early MF may be difficult because of the varied clinical and histologic expressions of the disease. METHODS: The authors review the epidemiology, possible risk factors, clinical manifestations, diagnostic techniques, staging, prognosis, and treatment options for MF. RESULTS: The varied and often nonspecific clinical and histologic presentations of MF may delay diagnosis and staging, thus necessitating further studies such as immunophenotyping and T-cell receptor gene rearrangement analysis. CONCLUSIONS: A multidisciplinary approach to the diagnosis, staging, and treatment of MF assists in optimizing outcomes from management of patients with this disease.
Assuntos
Micose Fungoide/diagnóstico , Neoplasias Cutâneas/diagnóstico , Antineoplásicos/uso terapêutico , Humanos , Micose Fungoide/epidemiologia , Micose Fungoide/terapia , Estadiamento de Neoplasias , Fototerapia , Prognóstico , Fatores de Risco , Neoplasias Cutâneas/tratamento farmacológicoRESUMO
Parapsoriasis en plaque has been suggested to be an early manifestation of mycosis fungoides (cutaneous T-cell lymphoma). We explored the disease course of patients with small plaque or large plaque parapsoriasis in a 26-year retrospective cohort analysis of 105 parapsoriasis patients, who were clinically and histopathologically followed up in Helsinki and Tampere University Hospitals. Eventual later cancers of these patients were verified from the Finnish Cancer Registry. In the small plaque parapsoriasis group, 7 patients (10%) and in the large plaque parapsoriasis group 12 patients (35%), developed histologically confirmed mycosis fungoides during a median of 10 and 6 years, respectively. No significant differences were found regarding the risk of developing mycosis fungoides or the tendency to remission in patients treated with or without phototherapy. Our results show that not only large plaque parapsoriasis, but also small plaque parapsoriasis, as currently defined in textbooks, can progress to mycosis fungoides. The benefits of phototherapy are equivocal in parapsoriasis treatment as far as progression to cancer is concerned.
Assuntos
Micose Fungoide/epidemiologia , Micose Fungoide/etiologia , Psoríase/complicações , Neoplasias Cutâneas/epidemiologia , Neoplasias Cutâneas/etiologia , Adulto , Idoso , Transformação Celular Neoplásica , Estudos de Coortes , Feminino , Finlândia/epidemiologia , Humanos , Masculino , Pessoa de Meia-Idade , Micose Fungoide/tratamento farmacológico , Micose Fungoide/patologia , Terapia PUVA , Psoríase/patologia , Sistema de Registros , Estudos Retrospectivos , Neoplasias Cutâneas/tratamento farmacológico , Neoplasias Cutâneas/patologiaRESUMO
Mycosis fungoides, an uncommon form of cutaneous T-cell lymphoma, arises in the skin and frequently progresses to generalized lymphadenopathy Although the cause of cutaneous T-cell lymphoma is unknown, chronic immunosuppression may play a role. A few cases have been reported in renal transplant recipients; however, ours appears to be the 1st report of cutaneous T-cell lymphoma in a cardiac transplant recipient. In our patient, cutaneous manifestations of the disease were noted less than 1 year after transplantation. Seven years after transplantation, Sézary syndrome, a variant form of mycosis fungoides, was diagnosed by tissue biopsy and flow cytometry analysis. Photopheresis improved symptoms but was not well tolerated because of hemodynamic sequelae. Psoralen and ultraviolet A therapy also improved the patient's skin condition, but a generalized lymphadenopathy developed. The maintenance immunosuppressive regimen was changed from cyclosporine (3 mg/kg/day) and azathioprine to cyclosporine (1.5 mg/kg/day) and cyclophosphamide. Although effective in the short-term, the results of this therapeutic strategy could not be fully evaluated because the patient died of acute myocardial infarction.