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Bruce R. Smoller - Dermal Tumors: The Basics

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Bruce R. Smoller Dermal Tumors: The Basics

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Dermal Tumors: The Basics will serve as an effective and efficient handbook for the student of dermatopathology, and as a practical bench reference for the practicing diagnostician who desires rapid access to criteria that are useful in differentiating histologically similar entities. The reader will be able to focus upon a single histologic observation, i.e., inflammatory conditions without epidermal changes, and use this as a starting point from which to build a differential diagnosis based upon pattern recognition. As each entity is addressed, there will be a concise discussion of the basic clinical findings and epidemiologic associations. This will be followed by a histologic description, highlighting areas that serve to discriminate between the entity under discussion and similar ones. Any immunologic studies that might augment the diagnostic sensitivity or specificity will be discussed.

The chapters are thematically based and consist of essential bullet points arranged in organized outlines allowing for easy access and direct comparison between entities. The salient histologic features are depicted with abundant high quality, full-color photomicrographs placed immediately adjacent to the appropriate histologic bullet points. This volume will serve as an effective and efficient handbook for the student of dermatopathology, and as a practical bench reference for the practicing diagnostician who desires rapid access to criteria that are useful in differentiating histologically similar entities. The elaborate pictorial documentation will also enable the book to serve as an atlas of the commonest dermatologic disorders.

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Bruce R. Smoller and Kim M. Hiatt Dermal Tumors: The Basics 10.1007/978-3-642-19085-8_1 Springer-Verlag Berlin Heidelberg 2011
1. An Overview of Cutaneous Lymphomas
Bruce R. Smoller 1
(1)
Department of Pathology, University of Arkansas for Medical Sciences, Little Rock, AR 72205, USA
Bruce R. Smoller (Corresponding author)
Email:
Kim M. Hiatt
Email:
Abstract
Classifications of cutaneous lymphomas, Two recent systems both had problems, Revised European-American Classification of Lymphomas (REAL), European Organization for Research and Treatment of Cancer (EORTC)
  • Classifications of cutaneous lymphomas
    • Two recent systems both had problems
      • Revised European-American Classification of Lymphomas (REAL)
      • European Organization for Research and Treatment of Cancer (EORTC)
    • REAL classification
      • Uses classification of systemic lymphomas and applies them to cutaneous lymphomas
      • Classification based upon pathologic, genetic, and clinical features and adds immunohistochemical criteria
      • Cutaneous involvement accounted for in clinical features
    • EORTC classification
      • Recognized cutaneous lymphomas as a distinct entity
      • Organ-based classification system
      • Only system that has been clinically validated for this group of diseases; classified as indolent, intermediate or, aggressive
    • WHO-EORTC classification
      • Consensus conference in 2005 incorporated features of both systems to acknowledge particulars of cutaneous lymphomas
    • WHO 2008 classification
      • Integrated the WHO-EORTC classification of cutaneous lymphomas in the general classification
      • Only minor changes in terminology, except blastic plasmacytoid dendritic cell neoplasm, previously CD4+/CD56+ hematodermic neoplasm, previously blastic NK cell lymphoma
  • Overview
    • 3% per year increase in incidence of cutaneous lymphomas every year since 1970
    • Cutaneous lymphomas represent a very heterogeneous group of diseases with very different clinical presentations, courses, treatment programs, and prognoses
    • Second most common (after GI tract) site of extra-nodal non-Hodgkins lymphomas
    • Large groupings include T cell lymphomas, B cell lymphomas, and Hodgkins lymphoma
    • Overall incidence estimate of 1:100,000
    • 75% of cutaneous lymphomas are T cell lymphomas, 25% B cell lymphomas
    • Hodgkins lymphoma is exceedingly rare in skin
    • 510% of T cell lymphomas are not mycosis fungoides
  • T cell lymphomas (Table )
    Table 1.1
    T cell lymphomas
    Mycosis fungoides/Sezary syndrome
    Primary cutaneous CD30+ lymphoproliferative disorders
    Subcutaneous panniculitis-like T cell lymphoma
    Extranodal NK/T cell lymphoma, nasal type
    Blastic plasmacytoid dendritic cell neoplasm
    (Peripheral T cell lymphoma)
  • Mycosis fungoides (MF)/Sezary syndrome
    • Epidemiologic features
      • 0.42 cases/100,000 population
      • Overall, more than 50% of all cutaneous lymphomas
      • Incidence rapidly increased in 1970s1980s, but now stabilized
      • Twice as common in African Americans as in Caucasian Americans
      • Twice as common in men as in women
      • Associated with 3.3X relative risk for second, non-cutaneous malignancy
    • Clinical features
      • Usual onset in middle age (mean 5560), but can appear at any age
      • Starts most commonly as erythematous, slightly scaly patches on flank and buttocks
      • Progress to plaques and ultimately tumors (in a minority of patients)
      • Poikilodermatous variant uncommon
      • Involvement of lymph nodes, spleen, liver only late in course
    • Histologic features (Figs. )
      Fig 11 Early lesions of mycosis fungoides show an epidermotropic lymphocytic - photo 1
      Fig. 1.1
      Early lesions of mycosis fungoides show an epidermotropic lymphocytic infiltrate. Single atypical lymphocytes are seen lining up along the basilar epidermis
      Fig 12 In more evolved lesions of mycosis fungoides small clusters of - photo 2
      Fig. 1.2
      In more evolved lesions of mycosis fungoides, small clusters of atypical lymphocytes and increased single lymphocytes are present in the epidermis which also shows only minimal spongiosis and no dyskeratosis
      Fig 13 This case of mycosis fungoides shows only a mild dermal infiltrate but - photo 3
      Fig. 1.3
      This case of mycosis fungoides shows only a mild dermal infiltrate but numerous Pautriers microabscesses
      Fig 14 Variable number of eosinophils and some plasma cells are often present - photo 4
      Fig. 1.4
      Variable number of eosinophils and some plasma cells are often present in the dermal infiltrate of mycosis fungoides
      • Epidermotropic proliferation of hyerconvoluted, hyperchromatic lymphocytes in clusters (Pautriers microabscesses) and as single cells within epidermis
      • Difficult diagnosis to make in early lesions
      • Later lesions with less epidermotropism and more cytologic atypia
      • Variable number of eosinophils and some plasma cells
    • Immunologic features (Figs. )
      Fig 15 The epidermotropic lymphocytes in mycosis fungoides most commonly - photo 5
      Fig. 1.5
      The epidermotropic lymphocytes in mycosis fungoides most commonly express CD4
      Fig 16 Loss of normal T-cell markers such as CD5 is common as in this case of - photo 6
      Fig. 1.6
      Loss of normal T-cell markers such as CD5 is common as in this case of mycosis fungoides
      • Mycosis fungoides is defined as a neoplastic proliferation of CD3+, CD4+ T helper lymphocytes
      • CD8+ tumors are rare but are now classified as MF
      • Loss of CD2, CD3, and CD5 often seen
      • Clonal rearrangements of T cell receptor is ultimately detected in most cases, but only 50% of early lesions.
    • Molecular features
      • T-cell gene rearrangements seen in 5080% of patch-stage lesions, nearly 100% of tumor stage and Sezary cases
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