10 research outputs found
Prognostic model for patient survival in primary anorectal mucosal melanoma:stage at presentation determines relevance of histopathologic features
Pathological staging of primary anorectal mucosal melanoma is often performed according to the American Joint Commission on Cancer (AJCC) guidelines for cutaneous melanoma, as an anorectal melanoma-specific staging system does not exist. However, it remains unknown whether prognostic factors derived for cutaneous melanoma also stratify risk in anorectal melanoma. We retrospectively determined correlations between clinicopathological parameters and disease-specific survival in 160 patients. Patients were grouped by clinical stage at presentation (localized disease, regional or distant metastases). Cox proportional hazards regression models determined associations with disease-specific survival. We also summarized the somatic mutations identified in a subset of tumors analyzed for hotspot mutations in cancer-associated gene panels. Most of the patients were white (82%) and female (61%). The median age was 62 years. With a median follow-up of 1.63 years, median disease-specific survival was 1.75 years, and 121 patients (76%) died of anorectal melanoma. Patients presenting with regional (34%) or distant metastases (24%) had significantly shorter disease-specific survival compared to those with disease localized to the anorectum (42%). Of the 71 anorectal melanoma tumors analyzed for hotspot genetic alterations, somatic mutations involving the KIT gene (24%) were most common followed by NRAS (19%). Increasing primary tumor thickness, lymphovascular invasion, and absence of regression also correlated with shorter disease-specific survival. Primary tumor parameters correlated with shorter disease-specific survival in patients presenting with localized disease (tumor thickness) or regional metastases (tumor thickness, absence of regression, and lymphovascular invasion), but not in patients presenting with distant metastases. Grouping of patients according to a schema based on modifications of the 8th edition AJCC cutaneous melanoma staging system stratified survival in anorectal melanoma. Our findings support stage-specific associations between primary tumor parameters and disease-specific survival in anorectal melanoma. Moreover, the AJCC cutaneous melanoma staging system and minor modifications of it predicted survival among anorectal melanoma patients
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Hydroa vacciniforme: a rare photodermatosis
Hydroa vacciniforme (HV) is a rare photodermatosis characterized by a recurrent vesiculopapular eruption with varioliform scarring. The pathogenesis remains unknown. Herein we present a case to emphasize the importance of recognizing this condition and its clinical mimickers, which include other photodermatoses and lymphoma
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Transient iatrogenic immunodeficiency-related B-cell lymphoproliferative disorder of the skin in a patient with mycosis fungoides/SĂ©zary syndrome
Cutaneous Leishmaniasis in Cuban Immigrants to Texas who Traveled through the Darién Jungle, Panama
Processos linfoproliferativos da pele: parte 2 - linfomas cutâneos de células T e de células NK Processos linfoproliferativos da pele: part 2 - cutaneous T-cell and NK-cell lymphomas
Os linfomas cutâneos de cĂ©lulas T/NK constituem um grupo de doenças linfoproliferativas extranodais atualmente classificadas e subdivididas de acordo com o comportamento clĂnico segundo consenso da Organização Mundial de SaĂşde e da Organização EuropĂ©ia para Pesquisa e Tratamento do Câncer. Os linfomas cutâneos de cĂ©lulas T/NK de comportamento clĂnico indolente compreendem a micose fungĂłide clássica, a micose fungĂłide foliculotrĂłpica, a reticulose pagetĂłide, a cĂştis laxa granulomatosa, o linfoma cutâneo primário de grande cĂ©lula anaplásica, a papulose linfomatĂłide, o linfoma subcutâneo de cĂ©lula T paniculite-sĂmile e o linfoma cutâneo primário de pequena e mĂ©dia cĂ©lula T CD4+ pleomĂłrfica. Os linfomas cutâneos de cĂ©lulas T/NK de comportamento agressivo incluem a sĂndrome de SĂ©zary, o linfoma extranodal de cĂ©lula T/NK, tipo nasal, o linfoma cutâneo primário agressivo de cĂ©lula T CD8+ epidermotrĂłpica, o linfoma cutâneo de cĂ©lula T <FONT FACE=Symbol>gd</FONT> e o linfoma cutâneo primário de cĂ©lula T perifĂ©rica, nĂŁo especificado. O linfoma-leucemia de cĂ©lulas T do adulto e a neoplasia hematodĂ©mica CD4+CD56+, embora considerados linfomas sistĂŞmicos, sĂŁo aqui abordados por apresentarem-se inicialmente na pele em significativo nĂşmero de pacientes. O diagnĂłstico desses processos Ă© realizado pelo exame histopatolĂłgico complementado pela análise do fenĂłtipo das cĂ©lulas neoplásicas, imprescindĂvel no processo classificatĂłrio. O estadiamento para a avaliação da extensĂŁo anatĂ´mica da doença considera alĂ©m do envolvimento cutâneo, o estado clĂnico e histolĂłgico dos linfonodos e das vĂsceras. Avaliação hematolĂłgica Ă© fundamental na caracterização da sĂndrome de SĂ©zary. Os tratamentos preconizados incluem terapĂŞuticas dirigidas exclusivamente Ă pele, modificadores da resposta biolĂłgica e quimioterapia sistĂŞmica.<br>The cutaneous NKT/cell lymphomas are a group of extranodal lymphoproliferative disorders currently classified and subdivided based on their clinical behavior, according to a consensus reached between the World Health Organization and the European Organization for Research and Treatment of Cancer. The cutaneous NKT/cell lymphomas of indolent clinical behavior comprise the classical mycosis fungoides, folliculotropic mycosis fungoides, pagetoid reticulosis, granulomatous slack skin, primary cutaneous anaplastic large cell lymphoma, lymphomatoid papulosis, subcutaneous panniculitis-like T-cell lymphoma and primary cutaneous CD4+ small/medium-sized pleomorphic T-cell lymphoma. The aggressive clinical behavior cutaneous NKT/cell lymphomas include SĂ©zary syndrome, extranodal NK/T-cell lymphoma, nasal type, primary cutaneous aggressive epidermotropic CD8+T-cell lymphoma, cutaneous gamma-delta T-cell lymphoma and primary cutaneous peripheral T-cell lymphoma, unspecified. The adult T-cell leukemia lymphoma and CD4+ CD56+ hematodermic neoplasm are considered systemic lymphomas but are addressed in this article for their initial cutaneous manifestations in a significant number of patients. The diagnosis of these processes is based on histological examination complemented by phenotypic analysis of neoplastic cells, which is essential for classification. The recommended staging is based on type and extension of cutaneous involvement, clinical conditions and histological examination of lymph nodes and organs. Hematological assessment is fundamental to characterize SĂ©zary syndrome. The recommended therapies include exclusively cutaneous treatment, biological response modifiers and systemic chemotherapy