Primary cutaneous T-cell lymphoma: experience from the Peruvian National Cancer Institute

Abstract: Background: Primary cutaneous T-cell lymphomas constitute a heterogeneous and rare group of diseases with regional particularities in Latin America. Objective: To determine the clinicopathological features, relative frequency and survival among patients from a Peruvian institution. Methods: Primary cutaneous T-cell lymphomas were defined based on the absence of extracutaneous disease at diagnosis. Classification was performed following the 2008 World Health Organization Classification of Neoplasms of the Hematopoietic and Lymphoid tissues. Risk groups were established according to the 2005 World Health Organization-EORTC classification for cutaneous lymphomas. Data of patients admitted between January 2008 and December 2012 were analyzed. Results: 74 patients were included. Mean age was 49.5 years. In order of frequency, diagnoses were: mycosis fungoides (40.5%), peripheral T-cell lymphoma not otherwise specified (22.95%), adult T-cell lymphoma/leukemia (18.9%), CD30+ lymphoproliferative disorders (6.8%), hydroa vacciniforme-like lymphoma (5.4%), extranodal NK/T-cell lymphoma (4.1%) and Sézary syndrome (1.4%). Predominant clinical patterns were observed across different entities. Mycosis fungoides appeared mainly as plaques (93%). Peripheral T-cell lymphoma not otherwise specified and adult T-cell lymphoma/leukemia presentation was polymorphic. All patients with hydroa vacciniforme-like lymphoma presented with facial edema. All cases of extranodal NK/T-cell lymphoma appeared as ulcerated nodules/tumors. Disseminated cutaneous involvement was found in 71.6% cases. Forty-six percent of patients were alive at 5 years. Five-year overall survival was 76.4% and 19.2%, for indolent and high-risk lymphomas, respectively (p<0.05). High risk group (HR: 4.6 [2.08-10.18]) and increased DHL level (HR: 3.2 [1.57-6.46]) emerged as prognostic factors for survival. Study limitations: Small series. Conclusion: Primary cutaneous T-cell lymphomas other than mycosis fungoides or CD30+ lymphoproliferative disorders are aggressive entities with a poor prognosis.

Análise comparativa da expressão e atividade das metaloproteinases 2 e 9 e de seus inibidores teciduais nas lesões cutâneas das variantes poiquilodérmica e clássica da micose fungoide / A comparative analysis of the expression and activity of metalloproteinases 2 and 9 and their tissue inhibitors in cutaneous lesions of poikilodermatous and classic variants of mycosis fungoides

Micose fungoide poiquilodérmica (MFp) é uma variante clínica de micose fungoide (MF). É mais indolente e caracterizada pela presença da poiquilodermia. As metaloproteinases (MMP) e seus inibidores específicos TIMP (Tissue Inhibitors of Metaloproteinases) estão envolvidos na oncogênese. Especificamente as MMP2 e MMP9 e seus inibidores, TIMP-2 e TIMP-1, respectivamente, foram relacionados ao prognóstico em tumores. Poucos trabalhos estudaram MMP e nenhum estudou a ação dos TIMP na MF. Objetivos: avaliar a relação entre MMP2 e MMP9 e seus inibidores TIMP2 e TIMP1 e a agressividade da MF e descrever a casuística de micose fungoide poiquilodérmica no ambulatório de linfomas cutâneos da Divisão de Clínica Dermatológica do Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo. Métodos: análise retrospectiva de 54 casos de MFp, sendo 25 de MFp localizada 14 de MFp generalizada e 15 de MFp mista. Para análise das MMP e TIMP, os grupos de MFp foram comparados com 7 amostras de pele normal (PN), 10 casos de MF clássica inicial (MFi), 9 casos de MF tumoral não-transformada (MFT nt) e 10 de MF tumoral transformada (MFT t). Resultados: A proporção de mulheres: homens foi 2,44. MFp apresentou maior tempo entre os primeiros sintomas e o diagnóstico. MFpG apresentou maior prevalência de lesões do tipo pitiríase liquenoide crônica (PLC) (79%). Houve alta prevalência de MF hipocromiante (62%) no grupo MFp mista. A histologia da MFp apresentou características típicas de MF e, adicionalmente, atrofia, telangectasias e derrame pigmentar, específicos da forma poiquilodérmica. Na imuno-histoquímica predominou o fenótipo CD3+, CD4+, CD7-, CD8- em todos os grupos, e MFp apresentou significantemente menor predomínio do fenótipo CD8+ que o grupo MFi. O grupo MFpG apresentou baixa positividade para pesquisa de clonalidade T da pele (12,5%). A MMP2 esteve mais presente na epiderme em MFi e MFp relativamente a MFT. Na derme superficial, os grupos MFi e MFp...

Poikilodermatous mycosis fungoides (pMF) is a clinical variant of mycosis fungoides (MF). It is more indolent than classic MF and is characterized by the presence of poikiloderma. The matrix metalloproteinases (MMPs) and their specific inhibitors TIMP (Tissue Inhibitors of Metalloproteinases) are involved in oncogenesis. Specifically, MMP2 and MMP9 and their inhibitors, TIMP-2 and TIMP-1, respectively, have been related to prognosis in tumors. There are few studies on MMP and none on the role of TIMPs in MF. Objectives: To evaluate if there is a relationship between the presence and activity of MMP2 and MMP9 and their inhibitors TIMP2 and TIMP1, and the aggressiveness of MF. To describe a casuistic of poikilodermatous mycosis fungoides in an outpatient clinic in the Dermatological Division of Hospital das Clinicas of University of Sao Paulo Medical School. Methods: Retrospective analysis of 54 cases of pMF, this included 25 localized pMF (LpMF), 14 generalized pMF (GpMF) and 15 mixed pMF. For the analysis of MMPs and TIMPs, the pMF groups were compared with 7 normal skin samples (NS), 10 cases of initial classical MF (cMF), 9 cases of non-transformed tumor MF (nt MFT) and 10 transformed tumor MF (t MFT). Results: The proportion of women : men was 2.44. The pMFs groups showed a longer period of time from the first symptoms to the diagnosis than the cMF group. The GpMF group had a higher incidence of pityriasis lichenoides chronica-like lesions (PLC) (79%) than the other groups. There was a high incidence of hypopigmented MF (62%) in the mixed pMF group. Histology showed typical characteristics of MF and, additionally, atrophy, telangiectasia and pigmentary alterations compatible with pMF. At immunohistochemistry the cases were predominantly CD3+, CD4+, CD7-, CD8- phenotype in all groups, and the pMF groups had a significantly lower prevalence of CD8+ phenotype than the cMF group. The GPMF group showed low positivity for clonality of the T-cell...

Linfoma de células cutáneas de células T, tipo micosis fungoide folicular: reporte de un caso / Cutaneous T-cell lymphoma type mycosis fungoides follicular: case report

La Micosis Fungoide (MF) es el linfoma cutáneo más común de células T. Tiene un comportamiento indolente, llevando a algunos a utilizar el término de linfoma cutáneo de células T (LLCT) como sinónimo de la MF. Se caracteriza por una erupción cutánea crónica, generalizada, y clínicamente por la evolución de los parches en placas y tumores. A continuación se presentará un caso clínico que tras un diagnóstico de liquen plano refractario a tratamiento, se diagnostica micosis fungoide folicular.

Mycosis fungoides (MF) is recognized as the most common cutaneous T-cell lymphoma (CTCL). It has an indolent behavior, leading some to use the term cutaneous T-cell lymphoma as synonymous of MF. It is characterized by chronic, widespread rash, and clinically by the evolution of patches in plaques and tumors. We describe our experience with a case that after a diagnosis of lichen planus refractory to treatment, we diagnosed follicular mycosis fungoides.

Radioterapia en cáncer no melanoma / Radiotherapy for non-melanoma skin cancer

La radioterapia es la disciplina que emplea radiaciones ionizantes en terapia médica. Es una herramienta ampliamente utilizada en Oncología y también dentro de la patología oncológica cutánea. Se trata de un procedimiento agresivo y no exento de riesgos; sin embargo, sus beneficios se encuentran claramente demostrados desde hace décadas. En los últimos años han aparecido nuevas alternativas terapéuticas menos agresivas y, por lo tanto, muy atractivas. El objetivo de este trabajo es explicar los principios básicos de la radioterapia aplicada a la Dermatología, mostrar los tratamientos más representativos y sus resultados en cáncer de piel.

Radiotherapy is the discipline that uses ionizing radiation in medical therapy. It is widely used in both oncology and cutaneous oncological pathologies. While it is an aggressive procedure and not risk-free, its benefits have been clearly demonstrated for years.In recent years, new, less aggressive - and therefore more popular - therapies have appeared. We review the basic principals of radiotherapy applied to dermatology, present the most representative treatments, as well as its results on skin cancer.

Câncer cutâneo ocupacional / Occupational skin cancer

O presente trabalho é baseado em revisão bibliográfica no período de 1990 a 1997 sobre os cânceres cutâneos de origem ocupacional. Seu objetivo é dissertar sobre as suas forma clínico-patológicas, agentes etiológicos e medidas preventivas inerentes aos agentes.

Edema crónico centrofacial con consumo del componente C1q del complemento / Centrofacial chronic edema with consumption of the component C1q of the complement

Se estudia un paciente de sexo masculino de 29 años, que padecía en el momento de la consulta edema de labios y párpados de un año y medio de evolución. Al confeccionar la historia clínica se determinó que los edemas se presentaban con reagudización, acompañados con úlceras de la mucosa yugal y fiebre de 40§C coincidente con las crisis. El edema de los párpados remitía casi en su totalidad con cada tratamiento esteroide, aunque no así el de labios. Los estudios de laboratorio mostraron anormalidades en la fracción C1q del complemento (0.3 mg ciento por ciento). El estudio histopatológico de un corte de labio halló infiltración mononuclear en el estroma, con disposición angiocéntrica. El infiltrado tenía células linfoides atípicas, que fueron positivas a la inmunomarcación con el anticuerpo monoclonal UCHL-1. Se diagnosticó linfoma T de presentación cutánea centrofacial. El caso se presenta por la interesante coexistencia en un individuo de un desorden linfoproliferativo con edema de labios inflamatorio crónico y edema de párpado recurrente por deficiencia adquirida del C1INH