Cutaneous Adnexal Neoplasms Luis Requena • Omar Sangüeza

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Cutaneous Adnexal Neoplasms Luis Requena • Omar Sangüeza Cutaneous Adnexal Neoplasms Luis Requena • Omar Sangüeza Cutaneous Adnexal Neoplasms Luis Requena Omar Sangüeza Department of Dermatology Department of Pathology Fundación Jiménez Díaz, Universidad Autónoma Wake Forest University School of Medicine Madrid Winston Salem, North Carolina Spain USA ISBN 978-3-319-45703-1 ISBN 978-3-319-45704-8 (eBook) DOI 10.1007/978-3-319-45704-8 Library of Congress Control Number: 2017934337 © Springer International Publishing Switzerland 2017 This work is subject to copyright. All rights are reserved by the Publisher, whether the whole or part of the material is concerned, specifically the rights of translation, reprinting, reuse of illustrations, recitation, broadcasting, reproduction on microfilms or in any other physical way, and transmission or information storage and retrieval, electronic adaptation, computer software, or by similar or dissimilar methodology now known or hereafter developed. The use of general descriptive names, registered names, trademarks, service marks, 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 use. The publisher, the authors and the editors are safe to assume that the advice and information in this book are believed to be true and accurate at the date of publication. Neither the publisher nor the authors or the editors give a warranty, express or implied, with respect to the material contained herein or for any errors or omissions that may have been made. The publisher remains neutral with regard to jurisdictional claims in published maps and institutional affiliations. Printed on acid-free paper This Springer imprint is published by Springer Nature The registered company is Springer International Publishing AG The registered company address is: Gewerbestrasse 11, 6330 Cham, Switzerland To my daughter Cecilia, the best of my life. Luis Requena, MD To Catherine, Edith and Charles, for their support and love. Omar. P. Sangüeza, MD Preface Our hope is that we have created a book useful for both beginning and seasoned dermato- pathologists. We understand that there are many adnexal neoplasms and that they can be intim- idating particularly for those new to the field of study. The many conditions and names make it difficult to develop a good grasp of the subject without a systematic approach. Our intent is to provide a comprehensive review of all adnexal neoplasms including those will follicular, sebaceous eccrine and apocrine differentiation. The book begins with a chapter on the general principles of dermatopathology. In order to understand and learn about any area of medicine one needs to develop a basic foundation in the subject. Each section begins with the embryologic and physiologic features as well as the nor- mal anatomy of the different adnexal structures. Each condition is fully illustrated with both clinical and histological photographs and relevant immunohistochemical stains are often included. The photographs illustrate not only the classical features of the diseases but also important variants, which are useful for the reader. Recognizing adnexal neoplasms is important not just for academic reasons but also as markers of systemic diseases and their association with specific genetic alterations. The list is long but a few examples include: multiple hidrocystomas as a marker of Schöpf syn- drome; clear cell syringomas in diabetes mellitus; basaloid follicular hamartomas as mark- ers of myasthenia gravis; fibrofolliculomas and multiple trichodiscomas as markers of renal oncocytoma in Birt-Hogg-Dubé syndrome; multiple pilomatricomas associated with myo- tonic dystrophy and cystic neoplasms with sebaceous differentiation associated with Muir-­ Torre syndrome. Those associated with specific genetic alterations which in turn are associated with systemic conditions, such as Birt-Hogg-Dubé syndrome linked to altera- tions of folliculin interacting proteins, FNIP1 and FNIP2 which play a role in mTor and AMPK signaling or Brooke-Spiegler syndrome associated with mutations in the CYLD suppressor gene. This list illustrates the usefulness of a familiarity with adnexal neoplasms in the diagnosis of diseases. Our intent has been to create a book that is as comprehensive as possible. We hope that this book will be useful in the academic study of adnexal neoplasms as well as in the prac- tical application to provide improved patient care. Madrid, Spain Luis Requena Winston Salem Omar P. Sangüeza NC, USA vii Acknowledgements For the illustrations of this book, many colleagues from several countries have collaborated. They generously lent us clinical pictures of their patients or sent us histopathologic sections of many of the proliferations included in the book. We are especially grateful to the following doctors: A. Bernard Ackerman, Victor Alegre, Adolfo Aliaga, José Aneiros, Giorgio Annesi, Pablo Boixeda, Francisco Bravo, Rosario Carrillo, José Gabriel Casas, Lorenzo Cerroni, Isabel Colmenero, Félix Contreras, Damian DiConstazo, Laila El-Shabrawi-Caelen, Maite Fernández-­ Figueras, Manuel Fernández Guerrero, Amaro García Díez, Alfonso González, Mark Jacobson, Helmut Kerl, Katrin Kerl, Tseng-tong Kuo, Heinz Kutzner, Philip E. LeBoit, Mar Llamas, Félix Manzarbeitia, Sebastián Ortiz, Ramon Pujol, José Luis Ramírez Bellver, Celia Requena, José Luis Rodríguez Peralto, Eloy Rodríguez, Arno Rütten, Evaristo Sánchez Yus, Martín Sangueza, Onofre Sanmartín, Carlos Santonja, Angel Santos Briz, Andrés Sanz, Jörg Schaller, Luis Miguel Soares Almeida, Oscar Tellechea, Felicidade Trindade and Antonio Torrelo. Special acknowledgment to Julio Diaz Perez and Charles P. Sangüeza for their invaluable help. ix Contents Part I Neoplasms with Eccrine and Apocrine Differentiation 1 Apocrine and Eccrine Units ������������������������������������������������������������������������������������� 3 1.1 Apocrine Unit ����������������������������������������������������������������������������������������������������� 3 1.1.1 Historical Perspective ��������������������������������������������������������������������������� 3 1.1.2 Embryology ������������������������������������������������������������������������������������������� 4 1.1.3 Gross Anatomy ������������������������������������������������������������������������������������� 4 1.1.4 Histology ����������������������������������������������������������������������������������������������� 6 1.1.5 Immunohistochemistry ������������������������������������������������������������������������� 9 1.1.6 Electron Microscopy ����������������������������������������������������������������������������� 10 1.1.7 Biology ��������������������������������������������������������������������������������������������������� 10 1.2 Eccrine Units ����������������������������������������������������������������������������������������������������� 11 1.2.1 Historical Perspective ��������������������������������������������������������������������������� 11 1.2.2 Histology ����������������������������������������������������������������������������������������������� 13 1.2.3 Histochemistry and Immunohistochemistry ����������������������������������������� 13 1.2.4 Biology ��������������������������������������������������������������������������������������������������� 16 References ������������������������������������������������������������������������������������������������������������������� 16 2 General Principles for the Histopathologic Diagnosis of Neoplasms with Eccrine and Apocrine Differentiation. Classification and Histopathologic Criteria for Eccrine and Apocrine Differentiation ����������������������������������������������������������������������������������������������������������� 19 2.1 General Principles for Histopathologic Diagnosis ��������������������������������������������� 19 2.2 Classification of Neoplasms with Eccrine and Apocrine Differentiation ��������� 20 2.3 Histopathologic Criteria for Eccrine and Apocrine Differentiation ������������������� 21 References ������������������������������������������������������������������������������������������������������������������� 24 3 Hidrocystomas ����������������������������������������������������������������������������������������������������������� 25 3.1 History ��������������������������������������������������������������������������������������������������������������� 25 3.2 Clinical Features ����������������������������������������������������������������������������������������������� 26 3.3 Histopathologic Features ����������������������������������������������������������������������������������� 27 3.4 Histogenesis ������������������������������������������������������������������������������������������������������� 30 3.5 Biological Behavior and Treatment ������������������������������������������������������������������� 31 References ������������������������������������������������������������������������������������������������������������������� 31 4 Eccrine and Apocrine Nevi ������������������������������������������������������������������������������������� 33 4.1 Eccrine Nevus ��������������������������������������������������������������������������������������������������� 33 4.1.1 History ��������������������������������������������������������������������������������������������������� 33 4.1.2 Clinical Features ����������������������������������������������������������������������������������� 33 4.1.3
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