ROBBOY’S PATHOLOGY FEMALE of the REPRODUCTIVE TRACT Commissioning Editor: Michael J Houston Development Editor: Sharon Nash Project Manager: Rory MacDonald Design: Erik Bigland Illustration Manager: Merlyn Harvey Illustrator: Richard Prime Marketing Manager(s) (UK/USA): John Canelon/Radha Mawrie ROBBOY’S PATHOLOGY FEMALE of the REPRODUCTIVE TRACT SECOND EDITION Edited by Stanley J. Robboy Rex C. Bentley MD FCAP MD FCAP Professor and Vice Chairman, Department of Pathology, Associate Professor of Pathology, Department of Pathology, Chief, Division of Diagnostic Services, Professor of Obstetrics Duke University Medical Center, Durham, NC, USA & Gynecology, Duke University Medical Center, Durham, NC, Peter Russell USA MD FRCPA Professor of Pathology, The University of Sydney and Director, George L. Mutter MD FCAP Department of Anatomical Pathology, Royal Prince Alfred Associate Professor of Pathology, Harvard Medical School, Hospital, Camperdown, NSW, Australia Department of Pathology, Brigham and Women’s Hospital, Malcolm C. Anderson Boston, MA, USA FRCPath FRCOG Emeritus Consultant Histopathologist, Department of Jaime Prat MD PhD FRCPath Histopathology, University Hospital, Queen’s Medical Centre, Professor and Chairman, Department of Pathology, Hospital Nottingham, UK de la Santa Creu i Sant Pau, Autonomous University of Barcelona, Barcelona, Spain Notice Medical knowledge is constantly changing. Standard safety precautions must be followed, but as new research and clinical experience broaden our knowledge, changes in treatment and drug therapy may become CHURCHILL LIVINGSTONE necessary or appropriate. Readers are advised to check the most current An imprint of Elsevier Limited. product information provided by the manufacturer of each drug to be administered to verify the recommended dose, the method and duration © 2009, Elsevier Limited. All rights reserved. of administration, and contraindications. It is the responsibility of the practitioner, relying on experience and knowledge of the patient, to First edition 2002 determine dosages and the best treatment for each individual patient. Neither the Publisher nor the authors assume any liability for any injury The right of Stanley J Robboy, George L Mutter, Jaime Prat, and/or damage to persons or property arising from this publication. Rex C Bentley, Peter Russell & Malcolm C Anderson to be identifi ed The Publisher as editors of this work has been asserted by them in accordance with the Copyright, Designs and Patents Act 1988. No part of this publication may be reproduced or transmitted in any form or by any means, electronic or mechanical, including photocopying, recording, or any information storage and retrieval system, without permission in writing from the publisher. Permissions may be sought directly from Elsevier’s Rights Department: phone: (+1) 215 239 3804 (US) or (+44) 1865 843830 (UK); fax: (+44) 1865 853333; Working together to grow e-mail: [email protected]. You may also complete your libraries in developing countries request on-line via the Elsevier website at http://www.elsevier.com/permissions. www.elsevier.com | www.bookaid.org | www.sabre.org ISBN: 978-0-443-07477-6 British Library Cataloguing in Publication Data A catalogue record for this book is available from the British Library Library of Congress Cataloging in Publication Data A catalog record for this book is available from the Library of Congress The publisher’s policy is to use Printed in China paper manufactured from sustainable forests Last digit is the print number: 9 8 7 6 5 4 3 2 1 Contents Contributors xxix Clinical features 29 Foreword xxxiii Microscopic features 30 Psoriasis 30 Preface xxxv Clinical features 30 Dedication xxxvii Microscopic features 31 Acknowledgements xxxiii Less common dermatoses that frequently involve the vulva 31 Lichenoid dermatoses 31 Lichen sclerosus 31 CHAPTER 1 EMBRYOLOGY 1 Clinical features 31 Microscopic features 32 Francis Jaubert Stanley J. Robboy George L. Mutter Lichen planus 33 Marc Fellous Clinical features 33 Introduction 1 Cutaneous-pattern lichen planus 33 Mucosal lichen planus 34 Gonadal development 1 Mixed cutaneous and mucosal pattern lichen Role of germ cells 13 planus 35 Müllerian and wolffi an duct development 14 Pathology 35 The müllerian duct to week 8 14 Immunobullous disorders 35 External infl uence on the developing embryonic genital Pemphigus vulgaris 36 tract ducts 15 Clinical features 36 The müllerian duct after week 8 17 Microscopic features 36 The müllerian duct during the second trimester 18 Pemphigus vegetans 36 Clinical features 36 External genitalia 19 Microscopic features 37 Bullous pemphigoid and cicatricial pemphigoid 37 Clinical features 38 CHAPTER 2 VULVAR DERMATOSES AND Microscopic features 38 INFECTIONS 23 Chronic bullous dermatosis of childhood and adult linear IgA bullous dermatosis 38 Maria Angelica Selim Bruce R. Smoller Clinical features 38 Christopher R. Shea Stanley J. Robboy Microscopic features 39 Introduction 23 Inherited dermatoses 39 Hailey–Hailey disease 39 Common dermatoses affecting the vulva 23 Clinical features 39 Acute and chronic spongiotic dermatitis (eczemas) 23 Microscopic features 39 General clinical and pathologic features of spongiotic Epidermolysis bullosa 39 dermatitis 23 Dariers disease 39 Acute spongiotic dermatitis 23 Clinical features 39 Chronic spongiotic dermatitis 26 Microscopic features 40 Clinical patterns of vulvar spongiotic dermatitis 27 Other infl ammatory diseases affecting the vulva 40 Endogenous vulvar spongiotic dermatitis 27 Zoons vulvitis (plasmacytosis mucosae) 40 Exogenous vulvar spongiotic dermatitis 28 Clinical features 40 Hints on histopathologic interpretation of vulvar Microscopic features 41 spongiotic dermatitis 29 Erythema multiforme 41 Chronic dermatidites 29 Microscopic features 41 Lichen simplex chronicus 29 v CONTENTS Behçets disease 41 Other virus infections 52 Clinical features 42 Clinical features 52 Microscopic features 42 Microscopic features 53 Crohns disease 42 Clinical features 53 Clinical features 42 Microscopic features 54 Microscopic features 43 Protozoal infections 54 Hidradenitis suppurativa 43 Trichomoniasis 54 Vulvar pain syndromes 44 Infestations 55 Vulvar vestibulitis 44 Scabies 55 Clinical features 44 Clinical features 55 Microscopic features 44 Microscopic features 55 Dysesthetic vulvodynia 44 Pubic (crab) lice (Phthirus pubis) 55 Pigmentary alterations 44 Appendix 2.1 Common clinical dermatologic Hyperpigmented vulva 44 terms 57 Hyperpigmentation due to melanin in vulvar skin 44 Clinical features 44 Appendix 2.2 Common dermatopathologic Microscopic features 44 terms 57 Hyperpigmentation due to increased keratin in stratum Terms applied to the surface keratin layer 57 corneum 45 Terms applied to the epidermis 58 Clinical features 45 Terms applied to the dermis 58 Microscopic features 45 Hypopigmentation 45 Postinfl ammatory hypopigmentation 45 CHAPTER 3 VULVAR CYSTS, NEOPLASMS, AND Vitiligo 46 RELATED LESIONS 59 Clinical features 46 Microscopic features 46 Christopher R. Shea Maria Angelica Selim Stanley J. Robboy Drugs 46 Cysts 59 Stevens–Johnson syndrome variant of erythema Follicular (epidermoid) cyst 59 multiforme 46 Defi nition 59 Fixed drug eruptions 46 Clinical features 59 Clinical features 46 Microscopic features 59 Microscopic features 47 Steatocystoma multiplex 59 Vulvar infections and infestations 47 Defi nition 59 Bacterial infections 47 Clinical features 59 Bacterial vaginosis 47 Microscopic features 59 Staphylococcal infections 47 Bartholin cyst 59 Streptococcal infections 47 Defi nition 59 Clinical features 47 Clinical features 59 Microscopic features 48 Microscopic features 60 Syphilis 48 Mucinous cyst 61 Clinical features 48 Defi nition 61 Microscopic features 48 Clinical features 61 Gonorrhea 49 Microscopic features 61 Chancroid 49 Ciliated cyst 61 Chlamydial infection 49 Defi nition 61 Granuloma inguinale (donovanosis) 49 Paraurethral (Skene) gland cyst 61 Fungal infections 49 Defi nition 61 Candidiasis 49 Clinical features 61 Clinical features 49 Microscopic features 61 Microscopic features 49 Mesonephric-like cyst 61 Pityriasis versicolor 50 Defi nition 61 Tinea cruris 50 Clinical features 61 Clinical features 50 Cyst of the canal of nuck 61 Microscopic features 50 Defi nition 61 Viral infections 50 Clinical features 61 Herpes virus infections 50 Microscopic features 62 Clinical features 50 Microscopic features 51 Benign keratinocytic neoplasms 62 Clinical features 52 Seborrheic keratosis (verruca seborrheica, basal cell Microscopic features 52 papilloma) 62 vi CONTENTS Defi nition 62 Clinical features 78 Clinical features 62 Microscopic features 78 Microscopic features 62 Blue nevus (dermal melanocytoma) 78 Keratoacanthoma 62 Defi nition 78 Defi nition 62 Clinical features 78 Clinical features 63 Microscopic features 78 Microscopic features 63 Malignant melanoma 79 Defi nition 79 Premalignant keratinocytic neoplasms 63 Clinical features 79 Vulvar intraepithelial neoplasia (VIN) 63 Microscopic features 80 Defi nition 63 Clinical features 63 Skin appendage neoplasms 81 Microscopic features 64 Hidradenoma papilliferum (papillary Bowenoid papulosis 66 hidradenoma) 81 Defi nition 66 Defi nition 81 Clinical features 66 Clinical features 81 Microscopic features 66 Microscopic features 82 Syringoma 82 Malignant keratinocytic neoplasms 66 Defi nition 82 Microinvasive squamous cell carcinoma 66 Clinical features 82 Invasive squamous cell carcinoma 67 Microscopic features 82 Defi nition 67 Trichoepithelioma (epithelioma adenoides cysticum, Clinical features 67 superfi cial trichoblastoma) 82 Microscopic features 69 Defi nition 82 Natural history 69 Clinical features 83 Verrucous carcinoma 70 Microscopic features 83 Defi nition 70 Clinical features 70 Vascular lesions 83 Pathology 70 Infantile hemangioma (strawberry hemangioma, juvenile Differential diagnosis 71 hemangioendothelioma) 83 Extramammary paget disease 72 Defi nition 83 Defi nition 72 Clinical features 83 Clinical features 72 Microscopic features 83 Microscopic features 72 Venous malformation (cavernous hemangioma) 84 Basal cell carcinoma 74 Defi nition 84 Defi nition 74 Clinical features 84 Clinical features 74 Microscopic features 84 Microscopic features 75 Deep lymphatic malformation (cavernous Basosquamous carcinoma 75 lymphangioma) 84 Defi nition 75 Defi nition 84 Clinical features 75 Clinical features 84 Microscopic features 75 Microscopic features 84 Sebaceous carcinoma 76 Miscellaneous lesions 84 Defi nition 76 Acrochordon (fi broepithelial polyp, skin tag, squamous Clinical features 76 papilloma) 84 Microscopic features 76 Defi nition 84 Melanocytic lesions 76 Clinical features 84 Lentigo 76 Microscopic features 84 Defi nition 76 Endometriosis (endometrial implant) 85 Clinical features 76 Defi nition 85 Microscopic features 76 Clinical features 85 Common acquired melanocytic nevus 76 Microscopic features 85 Defi nition 76 Heterotopic breast 85 Clinical features 77 Defi nition 85 Microscopic features 77 Clinical features 85 Atypical melanocytic nevus of the genital type 77 Microscopic features 85 Defi nition 77 Rare tumors and tumor-like conditions 85 Clinical features 78 Langerhans cell histiocytosis (histiocytosis x) 86 Microscopic features 78 Defi nition 86 Atypical (dysplastic, clark) nevus 78 Clinical features 86 Defi nition 78 Microscopic features 88 vii CONTENTS Merkel cell carcinoma (trabecular carcinoma, small cell Clinical features 100 carcinoma of the skin, primary cutaneous neuroendocrine Pathology 100 carcinoma) 88 Differential diagnosis 100 Defi nition 88 Leiomyoma 100 Clinical features 88 Defi nition 100 Microscopic features 88 Clinical features 100 Metastatic tumors 89 Pathology 100 Lymphangioma circumscriptum 101 Miscellaneous lesions of affi liated structures 89 Defi nition 101 Bartholin gland 89 Clinical features 101 Paraurethral (skene) gland 90 Pathology 101 Urethra 90 Angiokeratoma 101 Urethral caruncle 90 Defi nition 101 Defi nition 90 Clinical features 101 Clinical features 91 Pathology 102 Microscopic features 91 Other rare tumors 102 Urethral carcinoma 91 Defi nition 91 Locally recurrent neoplasms 103 Clinical features 91 Deep angiomyxoma (deep ‘aggressive’ Microscopic features 91 angiomyxoma) 103 Defi nition 103 Clinical features 103 CHAPTER 4 VULVAR MESENCHYMAL Pathology 103 NEOPLASMS AND TUMOR-LIKE Differential diagnosis 104 CONDITIONS 95 Superfi cial angiomyxoma (cutaneous myxoma) 104 Defi nition 104 Marisa R. Nucci Christopher D.M. Fletcher Clinical features 104 Pathology 105 Tumor-like conditions 95 Differential diagnosis 105 Fibroepithelial–stromal polyp (pseudosarcoma Dermatofi brosarcoma protuberans 105 botryoides) 95 Defi nition 105 Defi nition 95 Clinical features 105 Clinical features 95 Pathology 105 Pathology 95 Differential diagnosis 106 Differential diagnosis 95 Nodular fasciitis 95 Malignant neoplasms 106 Defi nition 95 Leiomyosarcoma 106 Clinical features 95 Defi nition 106 Pathology 95 Clinical features 106 Pathology 106 Benign neoplasms 96 Proximal-type epithelioid sarcoma 106 Angiomyofi broblastoma 96 Defi nition 106 Defi nition 96 Clinical features 106 Clinical features 97 Pathology 106 Pathology 97 Differential diagnosis 107 Differential diagnosis 97 Liposarcoma 107 Cellular angiofi broma 97 Defi nition 107 Defi nition 97 Clinical features 107 Clinical features 97 Pathology 107 Pathology 98 Other rare sarcomas 107 Differential diagnosis 98 Prepubertal vulval fi broma 98 Defi nition 98 CHAPTER 5 VAGINA 111 Clinical features 98 Pathology 98 Stanley J. Robboy Peter Russell Dermatofi broma (fi brous histiocytoma) 99 Defi nition 99 Normal structure 111 Clinical features 99 Mesonephric ducts 112 Pathology 99 Developmental disorders 113 Differential diagnosis 99 Imperforate hymen 114 Granular cell tumor 100 Vaginal agenesis 114 Defi nition 100 viii CONTENTS Transverse vaginal septum 114 Malignant vaginal tumors in childhood 134 Miscellaneous congenital disorders 114 Embryonal rhabdomyosarcoma 134 Pathology 134 Infl ammatory disorders 115 Natural history 135 Vaginitis 115 Yolk sac tumor 135 Malacoplakia 116 Tampon-related lesions and toxic shock syndrome 117 Lymphoma 136 Vaginal cysts 117 Secondary tumors of the vagina 136 Cysts of the introitus 117 Benign effects of diethylstilbestrol on the CHAPTER 6 CERVICAL BENIGN AND vagina 117 NON-NEOPLASTIC CONDITIONS 141 Vaginal adenosis 118 Tumor-like conditions 120 Anais Malpica Stanley J. Robboy Fibroepithelial polyps 120 Normal structure 141 Prolapsed fallopian tube 121 Anatomy 141 Granulation tissue nodule 122 Original (native) squamous epithelium 141 Postoperative spindle cell nodule 122 Terminology 141 Microglandular hyperplasia 122 Cytologic correlation 142 Endometriosis 123 Hormonal infl uences on squamous epithelium 143 Benign tumors 123 Cytologic correlation 143 Benign mixed tumor 123 Basal cell hyperplasia 144 Müllerian papilloma 123 Squamous cell hyperplasia 144 Leiomyoma 124 Cervical glandular epithelium 144 Rhabdomyoma 124 Cytologic correlation 145 Angiomyofi broblastoma 124 Physiologic changes in the cervix and the formation Miscellaneous benign tumors 125 of the transformation zone 146 Squamous neoplasia 125 Squamous metaplasia 148 Human papillomavirus (HPV) infection 125 Cytologic correlation 150 Vaginal intraepithelial neoplasia (VaIN) 125 The congenital transformation zone 150 Defi nition 125 Defi nition 150 Clinical features 125 Histogenesis of the congenital transformation Pathology 126 zone 151 Microinvasive squamous cell carcinoma 126 Cytologic correlation 152 Invasive squamous cell carcinoma 126 Defi nition 126 Infl ammatory (cervicitis) to regenerative Clinical features 126 changes 152 Pathology 127 Infective cervicitis 152 Spread and natural history 127 Cytologic correlation 153 Squamous cell neoplasia and exposure to Chlamydia trachomatis 154 diethylstilbestrol 127 Cytologic correlation 154 Verrucous carcinoma 127 Tuberculosis 154 Warty carcinoma 128 Cytologic correlation 155 Small cell carcinoma 128 Syphilis 155 Herpes simplex virus (HSV) 155 Glandular lesions 128 Cytologic correlation 156 Atypical adenosis 128 Cytomegalovirus (CMV) 156 Clear cell adenocarcinoma 129 Human papillomavirus (HPV) 156 Clinical features 129 Schistosomiasis 157 Gross pathology 129 Cytologic correlation 157 Microscopic pathology 130 Natural history 131 Epithelial infl ammatory changes 157 Pathogenesis 132 Healing/regenerating epithelium 158 Differential diagnosis 132 Cytologic correlation 159 Adenocarcinoma, non-clear cell 132 Non-neoplastic changes 159 Other primary malignant tumors of Müllerian metaplasias 159 the vagina 133 Tubal, endometrioid, and tuboendometrioid Malignant melanoma 133 metaplasia 159 Leiomyosarcoma 133 Pathology 159 Miscellaneous tumors 133 ix