A.C. Feller. J. Diebold Histopathology of Nodal and Extranodal Non-Hodgkin's Lymphomas

Transcription

1 A.C. Feller. J. Diebold Histopathology of Nodal and Extranodal Non-Hodgkin's Lymphomas

2 Springer Berlin Heidelberg New York Hong Kong London Milan Paris Tokyo

3 Alfred C. Feller Jacques Diebold in Collaboration with M. Paulli and A. Le Tourneau Histopathology of Nodal and Extranodal Non-Hodgkin's Lymphomas (Based on the WHO Classification) Third, Completely Revised and Updated Edition With 307 Figures in 345 Parts, Springer

4 Prof. Dr. med. Alfred Christian Feller FeIler Universitatsklinikum Universitätsklinikum Schleswig-Holstein, Campus Lubeck Lübeck Institut für fur Pathologie Ratzeburger Allee 160,23538 Lubeck Lübeck Professor emeritus Jacques Diebold Hotel-Dieu de Paris Service Central d' Anatomie et de Cytologie Pathologique 1, place du Parvis Notre Dame, Paris Cedex 04 Frankreich ISBN ISBN (ebook) DOI / Library of Congress Cataloguing-in-Publication Data Feller, Feiler, Alfred C. Histopathology of nodal and extra-nodal da! non-hodgkin's lymphomas/alfred C. Feller, Feiler, Jacques Diebold. - 3rd, completely rev. and updated ed. p.;cm. Rev. ed. of: Histopathology of non-hodgkin lymphomas/karl Lennert. 2nd ed Includes bibliographical references and index. ISBN (alk. paper) l. Lymphomas - Histopathology. I. Title: Histopathology of non-hodgkin lymphomas. II. H. Diebold, Jacques. III. Lennert, KarL Histopathology of non-hodgkin lymphomas. IV. Title. [DNLM: l. Lymphoma, Non-Hodgkin-classification. 2. Lymphoma, Non-Hodgkin - pathology. QZ 350 F318h 2003] RC280.L9L '446-dc This work is subject to copyright. All rights are reserved, whether the whole or part of the material is concerned, specifically the rights of translation, reprinting, reuse of illustrations, recitation, broadcasting, reproduction on microfilm or in any other way, and storage in data banks. Duplication of this publication or parts thereof is permitted only under the provisions of the German Copyright Law of September 9, 1965, in its current version, and permission for use must always be obtained from Springer-Verlag. Violations are liable for prosecution under the German Copyright Law. Springer-Verlag Berlin Heidelberg 2004 Originally published by Springer-Verlag Berlin Heidelberg New York in 2004 Softcover reprint of the hardcover 3rd edition 2004 The use of general descriptive names, registered names, trademarks, etc. in this publication does not imply, even in the absence of a specific statement, that such names are exempt from the relevant protective laws and regulations and therefore free for general genera! use. Product liability: The publishers cannot guarantee the accuracy of any information about the application of operative techniques and medications contained in this book. In every individual case the user must check such information by consulting the relevant literature. Cover design: E. Kirchner, D Heidelberg Typesetting: FotoSatz Pfeifer GmbH, D Grafelfing Gräfelfing Printed on acid-free paper 21/

5 Preface Roughly 10 years ago, the second edition of "Histopathology of Non-Hodgkin's Lymphomas" was published. The book gave an excellent overview of predominantly nodal non-hodgkin's lymphomas. Since then, advances in the field of immunology and the development of new techniques, including new monoclonal antibodies, have allowed a more precise definition of several lymphoma entities as well as a better understanding of the lymphoid system. Thus, in addition to the entities described in the updated Kiel Classification, reports of new, especially extranodal entities have appeared in the literature. Of these, the most important is MALT-lymphoma, which is one of the most frequently occurring extranodallymphomas. In addition, a large number of rare diseases have been identified, some of which seem to be well-defined clinicopathological entities. An increasing number of monoclonal antibodies that react with formalin fixed material, as well as PCR, sequencing techniques, and the detection of chromosomal abnormalities could aid in establishing the relevance of some of these newly described diseases. Meetings of The European Association of Hematopathology (EAHP) have opened Europe to the United States and have led to closer contact between the EAHP and its American counterpart, the Society for Hematopathology (SH). Based on these joint discussions, a group of well-known hematopathologists from both sides of the Atlantic suggested the Revised European-American Lymphoma Classification (REAL classification), which in many respects was based on the earlier Kiel classification. In turn, this encouraged the WHO to formulate a consensus classification, which was eventually approved by both hematopathological societies and issued in Around that time, Prof. Karl Lennert encouraged us to rewrite the second edition of the "Histopathology of Non-Hodgkin's Lymphomas". Thus, this new edition is completely based on the WHO classification. However, the reader who is familiar with the previous edition and with the Kiel Classification system will recognize that almost all of the nodal lymphoma entities described in the updated Kiel Classification appear in the WHO Classification. For this reason, we feel that the WHO Classification is, in many aspects, based on the Kiel Classification. In order to stress the importance of distinguishing between nodal and extranodal lymphomas and to clearly differentiate between them, we decided to organize discussions of the lymphoma entities according to the organs in which they primarily occur. Thus, a large chapter is devoted to describing primary nodal lymphomas. Since these lymphomas occur in extranodal sites as well, they are then also discussed in the chapters on the various organs, with cross-references to their extensive

6 VI Preface description in the chapter on nodal lymphomas, while the organ-specific lymphoma types are described in the respective organ chapters. We think that assigning the distinct lymphoma types to individual chapters makes the book easier to use. This new edition is intended chiefly for use by pathologists in every-day diagnostics, as was true for the previous edition. Although we have included the latest molecular genetic and cytogenetic results, the information provided is limited, as we feel that, even today, the most important diagnostic principle still lies in the morphology and the immunohistochemistry. Moreover, no attempt was made at providing a complete citation of the literature since an online search quickly renders citations in a printed book out of date. For diagnostic purposes, we have included a separate chapter that describes in detail the distinct patterns of lymphoma infiltration in the bone marrow. The last chapter of the book provides technical advice, but the reader should be well aware that the information is very incomplete and in some way provisional as new techniques are constantly being developed. However, basic techniques, especially those which are prey to pitfalls arising from the use and interpretation of results of molecular genetics, are discussed. We would not have been able to write this book without the tremendous work of Karl Lennert, who wrote the first and the second edition. We therefore are most grateful to Prof. Lennert for teaching us over many years and for giving us the possibility to write this third edition. We are very grateful for the help of many colleagues. We would like to thank Prof. Marco Paulli for writing the chapters on anaplastic large-cell lymphomas and lymphomatoid papulosis. Dr. Agnes Le Tourneau provided us with many photomicrographs. Prof. Philippe Gaulard supported us with the chapter on hepatosplenic NK/T-celllymphoma, first described by himself. We also want to thank Prof. Hartmut Merz for interesting discussions on many of the topics of this book. Dagmar Schmoe organized the bibliography with great enthusiasm. We are grateful to the many technicians from the institutes in Lubeck and Paris. It would not have been possible to design and write this book without the tremendous help of our secretaries, Catherine Belorgey, Birgit Kalus, Jaqueline Geron and Silja Klein. It was a great pleasure to co-operate with Springer-Verlag. First of all, we want to thank Dr. Julia Heidelmann for her continuous stimulation and support in finalizing this book. We also thank Wendy Ran for her excellent copy-editing, and Joachim W. Schmidt for his support during the production process as well as his careful attention to the quality of the illustrations. Lubeck and Paris, Summer 2003 Alfred C. Feller, Jaques Diebold

7 Contents 1 History of Lymphoma Classification 1.1 Introduction The Kiel Classification The REAL Classification Proposal The WHO Classification References Current Status of Lymphoma Classification 2.1 Introduction Small- and Large-Cell Versus Low- and High-Grade Primary Versus Secondary Lymphoma Definition of Immunocytoma and Its Border with Marginal Zone B-Cell Lymphoma Indistinct Border Between Nodal Marginal Zone B-Cell Lymphoma and Follicular Lymphoma with Marginal Zone Differentiation Grading of Follicular Lymphoma Distinction Between Diffuse Large B-Cell Lymphoma and Burkitt's Lymphoma: Reproducibility and Value of Subclassification Problems in Reproducibly Recognizing Anaplastic Large-Cell Lymphoma and Lymphoblastic Lymphoma on Purely Morphological Grounds Is Peripheral T-Cell Lymphoma, Unspecified a Distinct Clinicopathological Entity or a Catchall Term? Neither the WHO nor the EORTC Classification of Cutaneous Lymphoma Covers Today's Knowledge References Epidemiology 3.1 Introduction Statistical Data of the Kiel Lymphoma Registry Age and Sex Distribution Malignant Lymphoma of Childhood References Nodal B-Cell Lymphoma 4.1 Precursor B-Cell Lymphoblastic Leukemia Peripheral B-Cell Lymphoma

8 VIII Contents Small-B-Cell Lymphoma Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma l.2 Lymphoplasmacytic Lymphoma - Partially Corresponding to Macroglobulinemia Waldenstrom Hairy Cell Leukemia Plasmacytoma Nodal Marginal Zone B-Cell Lymphoma Follicular Lymphoma Mantle Cell Lymphoma Large-B-Cell Lymphoma Diffuse Large-B-Cell Lymphoma - Common Features Diffuse Large-B-Cell Lymphoma - Morphological Variants Diffuse Large-B-Cell Lymphoma - Clinical Variants Burkitt's Lymphoma References Nodal and Leukemic NK/T -Cell Lymphoma 5.1 Precursor T-Lymphoblastic Leukemia/Lymphoma Peripheral NK/T-Cell Lymphoma Peripheral NK/T-Cell Lymphoma - Predominately Leukemic T-Cell Chronic Lymphocytic/Prolymphocytic Leukemia l.2 Adult T-Cell Leukemia/Lymphoma Peripheral NK/T-Cell Lymphoma Predominately Nodal Angioimmunoblastic T-Cell Lymphoma Peripheral T-Cell Lymphoma, Unspecified Mycosis Fungoides/Sezary's Syndrome Secondary to the Lymph Node Anaplastic Large-Cell Lymphoma (T and Null Types) References Extranodal Lymphoma Introduction Gastrointestinal Lymphoma B-Cell Lymphoma Extranodal Marginal Zone B-Cell Lymphoma of MALT Intestinal Follicular Lymphoma Multiple Lymphomatous Polyposis - Intestinal Mantle Cell Lymphoma 198 Diffuse-Large-B-Cell Lymphoma of Gastrointestinal Tract Burkitt's Lymphoma of the Gastrointestinal Tract Primary Gastrointestinal T-Cell Lymphoma Enteropathy-Type T-Cell Lymphoma NK/T-Cell Lymphoma, Nasal Type Anaplastic Large-Cell Lymphoma Lymphoma of the Upper Aerodigestive Tract Lymphoma of the Oral Cavity Lymphoma ofwaldeyer's Ring and the Pharynx Lymphoma of the Nasal Cavity and Paranasal Sinuses B-Cell Lymphoma Extranodal NK/T-Cell Lymphoma - Nasal Type

9 Contents IX Lymphoma of the Larynx and Trachea Malignant Lymphoma of the Major Salivary Glands Lymphoma of the Eye, Lachrymal Glands, and Orbit Lymphoma of the Conjunctiva, Eyelids, Lachrymal Glands, and Orbit 227 Lymphoma of the Uvea and Retina (Similar to CNS Lymphoma) Primary Lymphoma of the Eye, Lachrymal Glands, and Orbit Lymphoma of the Mediastinum Mediastinal (Thymic) Large-B-Cell Lymphoma Extranodal Marginal Zone B-Cell Lymphoma of the Thymus Mediastinal Involvement in Precursor T- or B-Cell Lymphoblastic Lymphoma Mediastinal Involvement in Small-B-Cell Lymphoma Mediastinal Involvement in Large-B-Cell Lymphoma Other Than Primary Mediastinal Large-B-Cell Lymphoma and Burkitt's Lymphoma Mediastinal Involvement in NK/T -Cell Lymphoma Lymphoma of the Lung Primary Lymphoma of the Lung Extranodal Marginal Zone B-Cell Lymphoma of MALT Primary Diffuse Large-B-Cell Lymphoma of the Lung Primary Lung Intravascular Large-B-Cell Lymphoma Large B-Cell Lymphoma Secondary to Liebow's Lymphomatoid Granulomatosis Primary Lung Plasmacytoma Primary NK/T-Cell Lymphoma of the Lung Lymphoma of the Pleura Primary Lymphoma of the Pleura Primary Effusion Lymphoma Pyothorax-Associated Primary Lymphoma Secondary Pleural Lymphoma Lymphoma of the Heart Primary Cardiac Lymphoma Splenic Lymphoma Primary Splenic Lymphoma or Lymphoma with a Splenic Predominance Hairy Cell Leukemia Splenic Marginal Zone Lymphoma Primary Splenic Lymphoplasmacytic Lymphoma Plasmacytoma B-Prolymphocytic Leukemia Large B-Cell Lymphoma of the Spleen Hepatosplenic T-Cell Lymphoma T-Cell Large Granular Lymphocytic Leukemia Other Types of Lymphoma Secondary Splenic Lymphoma Small-B-Cell Lymphoma Other Types of B-Cell Lymphoma Peripheral NK/T-Cell Lymphoma Precursor Cell Neoplasias: B- and T-Lymphoblastic Lymphoma

10 X Contents 6.11 Lymphoma of the Liver Primary Lymphoma of the Liver Primary Lymphoma Presenting as a Tumorous Infiltrate of the Liver Primary Hepatic Lymphoma Presenting as Severe Hepatic Disease l.2 Secondary Lymphoma of the Liver Lymphoma of the Breast, Reproductive, and Urinary Systems Malignant Lymphoma of the Breast Primary Lymphoma of the Breast l.2 Secondary Lymphoma of the Breast Malignant Lymphoma of the Uterus Primary Uterine Lymphoma Addendum: Primary Lymphoma of the Vagina Secondary Uterine and Vaginal Lymphoma MalignantLymphoma of the Ovary Primary Lymphoma of the Ovary Secondary Lymphoma of the Ovary Lymphoma of Testis Malignant Lymphoma of the Prostate Lymphoma of the Kidney Primary Lymphoma of the Kidney Secondary Kidney Lymphoma Malignant Lymphoma of the Urinary Bladder Endocrine Gland Lymphoma Malignant Lymphoma of the Thyroid Gland Malignant Lymphoma of the Adrenal Gland Cutaneous Lymphoma Primary Cutaneous B-Cell Lymphoma Cutaneous Marginal Zone B-Cell Lymphoma/SALT Follicular Lymphoma Plasmacytoma Diffuse Large-B-Cell Lymphoma Cutaneous T-Cell Lymphoma Mycosis Fungoides - Classical Type Sezary's Syndrome Primary Cutaneous CD30+ T-Cell Lymphoproliferative Disorders Primary Cutaneous NK/T-Cell Lymphoma Lymphoma of Soft Tissues Lymphoma of the Bone Primary Lymphoma of Bone Secondary Bone Lymphoma Lymphoma of the Central Nervous System Intravascular Large-B-Cell Malignant Lymphoma References Plasma Cell Proliferations 7.1 Plasma Cell Myeloma/Bone Marrow Plasmacytoma Clinical Variants Diffuse Decalcifying Myelomatosis Osteosclerotic Myeloma

11 Contents XI Nonsecretory Myeloma Solitary Myeloma Plasma Cell Leukemia Variants with Peculiar Clinical Behavior Extraosseous (Extramedullary) Plasmacytoma Associated Diseases Castleman's Disease POEMS Syndrome Primary Amyloidosis Light- and/or Heavy-Chain Deposition Diseases Heavy-Chain Diseases References Lymphoma Occurring in a Setting of Immunodeficiency Lymphoproliferative Disorders Associated with Congenital Immunodeficiencies Ataxia-Telangiectasia Wiskott-Aldrich Syndrome Common Variable Immunodeficiency Disorder Severe Combined Immunodeficiency X-linked Lymphoproliferative Disorder Hyper-IgM Syndrome JOB Syndrome Nijmegen Breakage Syndrome Lymphoma and Lymphoproliferative Disorders Associated with Acquired Immunodeficiency HIV -Related Lymphoma Post-transplant Lymphoproliferative Disorders Plasmacytic Hyperplasia and Infectious-Mononucleosis-like PTLD Polymorphic PTLD Monomorphic B-Cell PTLD Monomorphic T-Cell PTLD Hodgkin's-Like PTLD References Practical Guidelines for Lymphoma Diagnosis in Bone Marrow Patterns of Involvement Paratrabecular Infiltrates Intertrabecular Infiltrates Interstitial Infiltrate Nodular Infiltrate Massive Infiltrate Monocellular Dispersion Intrasinusoidal Infiltrate Associated Lesions or Modifications Reticulum Fibers Framework Vascular Modifications Reactive Changes Interstitial Edema

12 XII Contents Polyclonal Plasmacytosis Reactive Lymphoid Nodules Chronic Inflammation Eosinophilic Necrosis Modifications of the Normal Hematopoietic Cell Lines Diagnosis of Bone Marrow Involvement According to the Different Types of Lymphoma Small B-Cell Lymphoma (Kroft et ai. 1995) B-Cell Chronic Lymphocytic Leukemia B-Prolymphocytic Leukemia Lymphoplasmacytic Lymphoma Follicular Lymphoma Mantle Cell Lymphoma Primary Splenic and Nodal and Extranodal Marginal Zone Lymphoma Hairy Cell Leukemia Myeloma Differential Diagnosis Large B-Cell Lymphoma T-Cell-RichlHistiocyte-Rich Large-B-Cell Lymphoma Intravascular Large B-Cell Lymphoma Burkitt's Lymphoma Precursor Cell Lymphoma (B- and T-Cell Lymphoblastic Lymphoma or Acute Leukemia) NK/T-Cell Lymphoma T-Prolymphocytic Leukemia T-Cell Large Granular Lymphocytic Leukemia Peripheral T-Cell Leukemia Lymphoma, Unspecified Peripheral T-Cell-Lymphoma, Angioimmunoblastic Anaplastic Large-Cell Lymphoma Hepatosplenic T-Cell Lymphoma General Comments Differential Diagnosis Reactive Lymphoid Nodules..., Reactive Intravascular Lymphocytosis Hodgkin's Lymphoma Non-lymphoid Acute Leukemia Systemic Mastocytosis..., Undifferentiated Carcinoma..., 407 References..., Practical Advice: Methods for the Diagnosis of Malignant Lymphoma 10.1 The Diagnosis of Malignant Lymphoma Practical Tips Immunohistochemistry and Molecular Clonality Analysis in the Diagnosis oflymphoma..., 414 References..., 420 Subject Index