Aggressive epidermotropic cutaneous CD8+ lymphoma: a cutaneous lymphoma with distinct clinical and pathological features. Report of an EORTC Cutaneous Lymphoma Task Force Workshop.

Aims: Aggressive epidermotropic cutaneous CD8(+) lymphoma is currently afforded provisional status in the WHO classification of lymphomas. An EORTC Workshop was convened to describe in detail the features of this putative neoplasm and evaluate its nosological status with respect to other cutaneous CD8(+) lymphomas.

Methods And Results: Sixty-one CD8(+) cases were analysed at the workshop; clinical details, often with photographs, histological sections, immunohistochemical results, treatment and patient outcome were discussed and recorded.

When will selective lymphadenectomy become standard of care in melanoma?

In 2006 Meiron Thomas, writing in the British Journal of Surgery, made the following statement about the value of sentinel lymph node biopsy (SLNB) as a staging procedure in cutaneous malignant melanoma (1): "Perhaps a more important concern for those hoping to gain reassurance from accurate nodal staging relates to positive SN(S) that are prognostically inaccurate, information that can be devastating for the patient, leading to unnecessary lymphadenectomy and possibly unnecessary adjuvant therapy". In September 2011 Meyrick Ross and Gershenwald, writing in the Journal of Surgical Oncology, made the following statement about the management of patients with cutaneous malignant melanoma (2): "Sentinel node biopsy has become an important component of the initial management of many of these patients for accurate staging of regional lymph nodes, as well as enhanced regional disease control and improved survival in the patients with microscopically involved nodes." These two extremes have polarized the debate about the proper management of patients with malignant melanoma and have lead to widespread confusion and dismay amongst practicing clinicians, GP's and patient groups.

Predictors of patient satisfaction with initial diagnosis and management of malignant melanoma.

Background: Malignant melanoma (MM), accounts for around 10% of skin cancers. To date, there have been few data on patient satisfaction with initial management of MM.

Objective: To identify the predictors of patient satisfaction with initial diagnosis and management of MM.

Imiquimod and lentigo maligna: a search for prognostic features in a clinicopathological study with long-term follow-up.

Background: Melanoma in situ/lentigo maligna (LM) is a potential precursor of LM melanoma. It occurs most commonly in elderly individuals on sun-exposed skin of the head and neck. Although surgical excision is the treatment of choice, this may not be desirable or feasible for large lesions at functionally or cosmetically important sites.

Bexarotene therapy for mycosis fungoides and Sézary syndrome.

Background: Bexarotene (Targretin) is a synthetic retinoid which is licensed for the treatment of advanced refractory cutaneous T-cell lymphoma (CTCL).

Objectives: To summarize our experience with bexarotene for patients with CTCL with the aim of assessing efficacy and safety.

Methods: A retrospective study of 66 patients (44 male, 22 female) with mycosis fungoides (40 patients) or Sézary syndrome (26 patients) who were commenced on bexarotene prior to August 2007 was carried out.

Case report of four patients with erythrodermic cutaneous T-cell lymphoma and severe photosensitivity mimicking chronic actinic dermatitis.

The marked photosensitivity associated with chronic actinic dermatitis (CAD) is presumed to be due to a T cell-mediated response to ultraviolet (UV)-induced epidermal neoantigens. Photosensitivity is, however, a rare occurrence in cutaneous T-cell lymphoma (CTCL). We discuss a series of four patients with erythrodermic CTCL who exhibited marked photosensitivity mimicking CAD.

A genomic and expression study of AP-1 in primary cutaneous T-cell lymphoma: evidence for dysregulated expression of JUNB and JUND in MF and SS.

Activator protein 1 (AP-1) consists of a group of transcription factors including the JUN and FOS family proteins with diverse biological functions. This study assessed the genomic and expression status of the AP-1 transcription factors in primary cutaneous T-cell lymphoma (CTCL) by using immunohistochemistry (IHC), Affymetrix expression microarray, real-time reverse transcriptase-polymerase chain reaction (RT-PCR) and fluorescent in situ hybridization (FISH). IHC showed JUNB protein expression in tumor cells from 17 of 33 cases of Sezary syndrome (SS) and JUND protein expression in 16 of 23 mycosis fungoides cases.

Abnormal activator protein 1 transcription factor expression in CD30-positive cutaneous large-cell lymphomas.

Background: CD30+ cutaneous large-cell lymphomas (CLCL) represent a heterogeneous subgroup of skin lymphomas including primary cutaneous CD30+ anaplastic large-cell lymphoma (C-ALCL), lymphomatoid papulosis (LyP), transformed mycosis fungoides (T-MF) and Hodgkin's lymphoma (HL) with cutaneous involvement. The activator protein 1 (AP-1) transcription factor consists of JUN, FOS and other protein families. Recent studies have revealed upregulation of JUNB in both MF and C-ALCL and overexpression of JUNB and CD30 in systemic HL and ALCL.

Does deprivation of area of residence influence the incidence, tumour site or T stage of cutaneous malignant melanoma? A population-based and clinical database study.

This study aimed to document the incidence of malignant melanoma at specific subsites in men and women, stratified by deprivation of area of residence in southeast England, and to explore the association between deprivation and tumour thickness at diagnosis. Data were extracted on 6468 cases from the Thames Cancer Registry for the years 1998 to 2002, and data on, and 508 cases were extracted from the clinical database of the Skin Tumour Unit, St Thomas' Hospital, for the years 1996 to 2004. The postcode of residence was used to assign quintiles of deprivation based on the income domain stated in the Indices of Deprivation 2000.

Three cases of lymphomatoid papulosis with a CD56+ immunophenotype.

We report 3 cases of lymphomatoid papulosis (LyP) with a CD56+, cytotoxic immunophenotype. All 3 patients presented with clinical histories typical of LyP, with one patient having associated mycosis fungoides. Histologically, two cases were type A LyP and one was type B.

Molecular staging of lymph nodes from 60 patients with mycosis fungoides and Sézary syndrome: correlation with histopathology and outcome suggests prognostic relevance in mycosis fungoides.

Background: Histological evidence of lymph node involvement is associated with a poor prognosis in patients with cutaneous T-cell lymphoma (CTCL).

Objectives: To determine whether T-cell receptor (TCR) gene analysis is of prognostic relevance in CTCL.

Methods: TCR gene analysis was performed on lymph node specimens from 60 patients with mycosis fungoides (MF) and Sézary syndrome (SS) using a highly sensitive polymerase chain reaction (PCR)/single-strand conformational polymorphism analysis and results were correlated with skin, overall clinical and histological lymph node stages.

Durable responses to imatinib in patients with PDGFRB fusion gene-positive and BCR-ABL-negative chronic myeloproliferative disorders.

Fusion genes derived from the platelet-derived growth factor receptor beta (PDGFRB) or alpha (PDGFRA) play an important role in the pathogenesis of BCR-ABL-negative chronic myeloproliferative disorders (CMPDs). These fusion genes encode constitutively activated receptor tyrosine kinases that can be inhibited by imatinib. Twelve patients with BCR-ABL-negative CMPDs and reciprocal translocations involving PDGFRB received imatinib for a median of 47 months (range, 0.

Heterogeneous abnormalities of CCND1 and RB1 in primary cutaneous T-Cell lymphomas suggesting impaired cell cycle control in disease pathogenesis.

Upregulation of cyclin D1/B-cell leukemia/lymphoma 1 (CCND1/BCL1) is present in most mantle cell lymphomas with the t(11;14)(q13;q32) translocation. However, little is known about the abnormalities of CCND1 and its regulator RB1 in primary cutaneous T-cell lymphomas (CTCL). We analyzed CCND and RB status in CTCL using fluorescent in situ hybridization (FISH), immunohistochemistry (IHC), and Affymetrix expression microarray.

Primary cutaneous T-cell lymphoma occurring after organ transplantation.

Lymphoma occurring after organ transplantation has been well described. The majority of cases are B-cell lymphomas and are usually associated with Epstein-Barr virus. Only a minority of posttransplant lymphomas are of T-cell origin, and primary cutaneous T-cell lymphoma (CTCL) is extremely rare.

Evidence-based practice of photopheresis 1987-2001: a report of a workshop of the British Photodermatology Group and the U.K. Skin Lymphoma Group.

Photopheresis or extracorporeal photochemotherapy (ECP) is a novel immunomodulatory therapy which involves separation of the patient's leucocyte-rich plasma, followed by ex vivo administration of a photosensitizer and ultraviolet A radiation, before reinfusion. ECP has been used successfully for the treatment of cutaneous T-cell lymphoma (CTCL: Sézary syndrome), graft-versus-host disease (GVHD) and cardiac transplant rejection. ECP has a dose-sparing effect on concurrent immunosuppressive therapy.

WHO/EORTC classification of cutaneous lymphomas 2005: histological and molecular aspects.

Unlabelled: The new WHO/EORTC classification for cutaneous lymphomas comprises mature T-cell and natural killer (NK)-cell neoplasms, mature B-cell neoplasms, and immature hematopoietic malignancies. It reflects the unique features of lymphoproliferative diseases of the skin, and at the same time it is as compatible as possible with the concepts underlying the WHO classification for nodal lymphomas and the EORTC classification of cutaneous lymphomas. This article reviews the histological, phenotypical, and molecular genetic features of the various nosological entities included in this new classification.

The use of LYVE-1 antibody for detecting lymphatic involvement in patients with malignant melanoma of known sentinel node status.

Background: Sentinel node (SN) status is the most important prognostic indicator in patients with cutaneous melanoma without clinically evident metastatic spread, but the procedure is associated with considerable morbidity. The LYVE-1 lymphatic marker offers the possibility of studying lymphangiogenesis and tumour metastasis within the primary excision.

Aims: To establish whether lymphatic vessel numbers/distribution within the primary tumour correlated with SN status.

Mycosis fungoides with a CD56+ immunophenotype.

We report 3 cases of mycosis fungoides (MF) with a CD56+ cytotoxic immunophenotype. Each patient presented with a different clinical phenotype: one exhibited limited poikilodermatous patches (skin stage T1); one, widespread hypopigmented lesions (skin stage T2); and one, poikiloderma with a single cutaneous tumor (skin stage T3). MF was confirmed both histologically and by the presence of a T-cell receptor clone in lesional skin in all cases.