(BQ) Part 1 book Pathology of the maxillofacial bones A guide to diagnosis presentation of content: Nonneoplastic diseases, cysts, epithelial odontogenic tumors, odontogenic tumors mesenchymal, odontogenic tumors mixed epithelial and mesenchymal, odontogenic tumors malignant.
Pieter Slootweg Pathology of the Maxillofacial Bones A Guide to Diagnosis 123 Pathology of the Maxillofacial Bones Pieter Slootweg Pathology of the Maxillofacial Bones A Guide to Diagnosis Pieter Slootweg Radboud University Nijmegen Medical Center Department of Pathology Nijmegen The Netherlands ISBN 978-3-319-16960-6 ISBN 978-3-319-16961-3 DOI 10.1007/978-3-319-16961-3 (eBook) Library of Congress Control Number: 2015941516 Springer Cham Heidelberg New York Dordrecht London © Springer International Publishing Switzerland 2015 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 Printed on acid-free paper Springer International Publishing AG Switzerland is part of Springer Science+Business Media (www.springer.com) Preface The maxillofacial skeleton, which comprises the jaws and the bony walls of the sinonasal cavities, is the site of a huge variety of lesions of a widely divergent nature Some of them have no counterparts elsewhere in the skeleton because the tissues from which they arise are confined to the maxillofacial bones Prime examples of these are the odontogenic cysts and tumors Other lesions are not confined to the maxillofacial skeleton but may pose differential diagnostic problems that are unique for this area; an illustrative example of this category forms craniofacial fibrous dysplasia that has a much wider differential diagnosis than shown by the same lesion at other body sites Furthermore, maxillofacial bone pathology still heavily relies on plain histomorphology Immunohistochemistry and molecular pathology only play a rather modest role, and, quite often, careful examination of a hematoxylinand eosin-stained slide is the only way to obtain the result that is needed to provide the clinician with the correct diagnosis upon which treatment should be based Therefore, the entities discussed in this book are lavishly illustrated in an attempt to show both the full spectrum of morphological varieties and the features that are decisive in making decisions among diseases sharing common histomorphological features Text and illustrations are based on specimens collected over a period of more than 40 years, and it is my privilege to acknowledge the courtesy of all colleagues who have contributed by either sending cases for consultation or otherwise by joining discussions on diagnostic issues during slide seminars or at other occasions Hopefully, the book will serve as a reliable guide for those practicing the pathology of the bones that are the building blocks of the maxillofacial skeleton Nijmegen, The Netherlands Pieter Slootweg v Contents Non-neoplastic Diseases 1.1 Introduction 1.2 Exostosis 1.3 Osteoma 1.4 Osteomyelitis 1.5 Odontomaxillary Dysplasia 1.6 Bullough’s Lesion References 1 1 10 Cysts 2.1 Introduction 2.2 Odontogenic Cysts – Inflammatory 2.2.1 Radicular Cyst 2.2.2 Paradental Cyst 2.3 Odontogenic Cysts – Developmental 2.3.1 Dentigerous Cyst 2.3.2 Lateral Periodontal Cyst 2.3.3 Glandular Odontogenic Cyst 2.3.4 Keratocystic Odontogenic Tumor 2.4 Non-odontogenic Cysts 2.4.1 Surgical Ciliated Cyst 2.5 Pseudocysts 2.5.1 Solitary Bone Cyst 2.5.2 Focal Bone Marrow Defect References 11 11 11 11 13 16 16 20 21 23 27 28 29 29 30 31 Epithelial Odontogenic Tumors 3.1 Introduction 3.2 Ameloblastoma 3.3 Calcifying Epithelial Odontogenic Tumor 3.4 Adenomatoid Odontogenic Tumor 3.5 Squamous Odontogenic Tumor References 33 33 33 47 51 57 58 vii Contents viii Odontogenic Tumors: Mesenchymal 4.1 Introduction 4.2 Odontogenic Myxoma 4.3 Odontogenic Fibroma 4.4 Cementoblastoma References 61 61 61 63 68 75 Odontogenic Tumors: Mixed Epithelial and Mesenchymal 5.1 Introduction 5.2 Calcifying Cystic Odontogenic Tumor 5.3 Ameloblastic Fibroma 5.4 Ameloblastic Fibro-Odontoma 5.5 Odontoma – Complex Type 5.6 Odontoma – Compound Type 5.7 Odonto-Ameloblastoma References 77 77 77 81 85 88 90 91 96 Odontogenic Tumors: Malignant 6.1 Introduction 6.2 Malignant Ameloblastoma 6.3 Ameloblastic Carcinoma 6.4 Primary Intraosseous Carcinoma 6.5 Clear Cell Odontogenic Carcinoma 6.6 Malignant Epithelial Odontogenic Ghost Cell Tumor 6.7 Sclerosing Odontogenic Carcinoma 6.8 Odontogenic Sarcomas References 99 99 99 99 99 106 109 111 115 121 Fibro-Osseous Lesions 7.1 Introduction 7.2 Fibrous Dysplasia 7.3 Ossifying Fibroma 7.4 Osseous Dysplasia References 123 123 123 132 148 155 Giant Cell Lesions 8.1 Introduction 8.2 Central Giant Cell Granuloma 8.3 Cherubism 8.4 Aneurysmal Bone Cyst References 157 157 157 164 167 170 Bone Tumors 9.1 Introduction 9.2 Osteoblastoma 9.3 Osteosarcoma References 171 171 171 178 195 Contents ix 10 Tumors of Cartilage 10.1 Introduction 10.2 Chondromyxoid Fibroma 10.3 Chondroblastoma 10.4 Chondrosarcoma 10.5 Mesenchymal Chondrosarcoma References 197 197 197 203 206 211 216 11 Other Lesions Involving the Maxillofacial Skeleton 11.1 Introduction 11.2 Desmoplastic Fibroma 11.3 Non-ossifying Fibroma 11.4 Melanotic Neuroectodermal Tumor of Infancy 11.5 Myoepithelial Tumors 11.6 Chordoma 11.7 Epitheloid Hemangioendothelioma References 217 217 217 220 221 222 224 229 232 12 Diseases of the Temporomandibular Joint 12.1 Introduction 12.2 Reactive Changes 12.3 Osteoarthritis 12.4 Inflammatory Disorders 12.5 Neoplasms 12.6 Synovial Chondromatosis 12.7 Condylar Hyperplasia References 235 235 235 235 237 239 239 240 248 Index 249 108 Fig 6.16 Histological picture of clear cell odontogenic carcinoma composed of cells with clear cytoplasm, hyperchromatic nuclei and well-defined borders, arranged in strands or large nests There is no peripheral palisading Fig 6.17 Clear cell odontogenic carcinoma may also contain smaller cells with compact nuclei and eosinophilic cytoplasm Odontogenic Tumors: Malignant 6.6 Malignant Epithelial Odontogenic Ghost Cell Tumor 109 Fig 6.18 Sometimes, the tiny strands in clear cell odontogenic carcinoma may be composed exclusively of cells with eosinophilic cytoplasm 6.6 Malignant Epithelial Odontogenic Ghost Cell Tumor Malignant epithelial odontogenic ghost cell tumor, also called ghost cell odontogenic carcinoma according to the latest WHO classification [1], is a tumor that combines the characteristics of a calcifying cystic odontogenic tumor with cytonuclear atypia indicating malignancy and that often arises through malignant transformation of a pre-existing benign calcifying cystic odontogenic tumor [23] As only a few cases of this tumor have been reported so far, its clinicopathologic features have not yet been well defined but its clinically malignant nature has become apparent Patients complain of progressive jaw expansion and rapid growth and extension into the adjacent tissue have also been reported This spread in soft tissues forms a clear distinction from ameloblastoma Radiologically, it appears as a radiolucent lesion with indistinct borders sometimes displaying multilocularity and tooth root displacement or resorption Histologically, the tumor is composed of strands of odontogenic epithelium, sometimes with an ameloblastoma-like appearance including columnar shape and nuclear polarity but showing atypical features and mitotic activity (Fig 6.19) Variable proportions of ghost cells are present, both intraepithelial as well as in the form of stromal aggregates (Fig 6.20) Also, eosinophilic calcified (dentinoid) amorphous material can be present in variable amounts, sometimes still containing remnants of ghost cell aggregates (Fig 6.21) Because of its ameloblastoma-like component with concomitant atypia, ameloblastic carcinoma enters the differential diagnosis but this latter lesion does not contain ghost cells and neither is there any differentiation of stromal cells to form juxtaepithelial dentinoid The malignant nature of this tumor is demonstrated by local invasion and the occasional occurrence of distant metastases [24] Therefore, wide surgical excision is the preferred treatment 110 Fig 6.19 Malignant epithelial odontogenic ghost cell tumor shows ghost cell aggregates in combination with cytonuclear atypia and mitotic activity Fig 6.20 In malignant epithelial odontogenic ghost cell tumor, ghost cells may lie either intraepithelially or occur as stromal aggregates Odontogenic Tumors: Malignant 6.7 Sclerosing Odontogenic Carcinoma 111 Fig 6.21 Similar to the calcifying cystic odontogenic tumor, the malignant epithelial odontogenic ghost cell tumor may contain mineralized collagenous matrix considered to be a dysplastic form of dentin in which remnants of ghost cells still are discernable 6.7 Sclerosing Odontogenic Carcinoma Sclerosing odontogenic carcinoma is a rare tumor in which sclerotic collagenous stroma predominates the picture to such a degree that the less conspicuous epithelial component may be easily overlooked [25] Whether it is an entity in its own right or only a pattern shared by a diversity of tumors has not yet been settled [26, 27] As provisionally defined, sclerosing odontogenic carcinoma is characterized by a dense sclerotic stroma that contains multiple infiltrating thin cords and small nests of cuboidal or polygonal epithelial cells with predominantly eosinophilic cytoplasm although some cytoplasmic clearing and a signet-ring appearance may occur as well Their nuclei show hyperchromatism and slight atypia but mitotic activity is low (Figs 6.22, 6.23 and 6.24) The malignant nature of the tumor also is demonstrated by invasion in skeletal muscle as well as by perineural spread (Figs 6.25 and 6.26) Recently, sclerosing odontogenic carcinoma in association with fibro-osseous lesions (osseous dysplasia, fibrous dysplasia and ossifying fibroma) of the jaws has been reported [27, 28] but the evidence is too meagre to exclude that this was just an incidental finding not warranting creating a separate category (Fig 6.27) The tumor cells express epithelial markers; when stained for keratin expression, the epithelial component quite often appears to be much more extensive than anticipated from the assessment of the slides routinely stained with hematoxylin and eosin (Fig 6.28) Desmoplastic ameloblastoma, calcifying epithelial odontogenic tumor (CEOT), primary intraosseous carcinoma, and clear cell odontogenic carcinoma should be considered in the differential diagnosis Desmoplastic ameloblastoma usually contains larger epithelial nests with central spindle cells that may show some whorling and moreover, occasionally, some peripheral palisading can be observed The neoplastic cells of CEOT have ample eosinophilic cytoplasm, show distinct cell borders and are embedded in a fibrous stroma containing matrix that stains like amyloid Primary intraosseous carcinoma consistently shows prominent squamous cell differentiation that is undetectable in sclerosing odontogenic carcinoma With clear cell odontogenic carcinoma, there is some morphological overlap as both clear cell odontogenic carcinoma and sclerosing odontogenic carcinoma contain clear cells as well as cells with eosinophilic 112 Fig 6.22 Low power view of sclerosing odontogenic carcinoma The epithelial component lies dispersed in a collagenous stroma Fig 6.23 At higher magnification, epithelial nests mainly composed of clear cells are shown Odontogenic Tumors: Malignant 6.7 Sclerosing Odontogenic Carcinoma Fig 6.24 High power view to show the cellular details of sclerosing odontogenic carcinoma Cells can have both eosinophilic as well as clear cytoplasm and nuclei show slight atypia Fig 6.25 The malignant nature of sclerosing odontogenic carcinoma is demonstrated by spread through the cortical bone into the adjacent soft tissues Tumor strands grow between muscle fibers 113 114 Fig 6.26 The malignant nature of sclerosing odontogenic carcinoma is also demonstrated by perineural invasion Fig 6.27 Occasionally, the stromal component of sclerosing odontogenic carcinoma may resemble a fibro-osseous lesion In these cases the epithelial component may be easily overlooked Odontogenic Tumors: Malignant 6.8 Odontogenic Sarcomas 115 Fig 6.28 When staining with keratin antibodies, the epithelial component of sclerosing odontogenic carcinoma can be seen to be much more extensive than supposed when looking only at routine hematoxylin and eosin stained slides cytoplasm and it has been questioned whether the finding that the former contains larger epithelial clusters represents enough morphological diversity to justify a complete separation between both lesions [26, 27] Figures 6.29 and 6.30 illustrate such an ambiguous case The malignant behavior of sclerosing odontogenic carcinoma requires surgical excision with an adequate margin that currently is considered curative [25] 6.8 Fig 6.29 Low power view of lesion showing features of both clear cell odontogenic carcinoma and sclerosing odontogenic carcinoma The invasive spread into both jaw bone and soft tissue is clearly displayed Odontogenic Sarcomas Odontogenic sarcomas are characterized by a malignant mesenchymal component and an epithelial component that may show either benign or malignant features They may occur at any age and their radiological appearance varies from radiolucent to mixed depending on the amount of hard tissues formed Various subtypes are discerned depending on the stage of dental development that is mimicked To accommodate for this, the WHO discerns between ameloblastic fibrosarcoma, ameloblastic fibrodentino- and fibro-odontosarcoma and odontogenic carcinosarcoma [1] Ameloblastic fibrosarcoma consists of malignant mesenchymal 116 Odontogenic Tumors: Malignant Fig 6.30 At higher magnification, the tumor shown in Fig 6.29 is shown to contain cells with eosinophilic as well as with clear cytoplasm, lying in a fibrous stroma, thus combining the cellular features of clear cell and sclerosing odontogenic carcinoma (fibrosarcoma-like) cells, showing nuclear pleomorphism and mitotic activity, dispersed in a loose myxoid stroma admixed with epithelial islands similar to those seen in ameloblastoma or ameloblastic fibroma [29] When dentin is also present, the lesion is designated ameloblastic fibrodentinosarcoma, and with the addition of enamel it becomes an ameloblastic fibroodontosarcoma (Figs 6.31 and 6.32) However, this subclassification has no prognostic relevance [30] These tumors may arise de novo or from a pre-existing ameloblastic fibroma or ameloblastic fibro-odontoma [31] Those extremely rare lesions that combine carcinomatous and sarcomatous elements, recognizable as odontogenic in view of the presence of an epithelial component resembling ameloblastic carcinoma, have been named odontogenic carcinosarcoma or odontogenic carcinoma with sarcomatous proliferation (Figs 6.33, 6.34, 6.35, 6.36 and 6.37) [29] Ghost cells may be found in odontogenic sarcomas as in so many other odontogenic neoplasms, either benign or malignant (Figs 6.38 and 6.39) The odontogenic sarcomas are considered low-grade neoplasms: local recurrence may occur but their metastatic potential is limited However, their rarity precludes any well-based statements 6.8 Odontogenic Sarcomas Fig 6.31 Low power view of ameloblastic fibroodontosarcoma The main tumor component consists of fibrocellular tissue, epithelium and dental hard tissues being a minor component Fig 6.32 High power view from Fig 6.31 to show the cytonuclear atypia and the mitotic activity restricted to the mesenchymal component 117 118 Fig 6.33 Low power view of a tumor in which both the epithelial and mesenchymal component are cell-rich and show cytonuclear atypia, hence a diagnosis of odontogenic carcinosarcoma is justified Fig 6.34 Deposition of dysplastic dentin at the interface between mesenchyme and epithelium illustrates that the neoplastic cells still possess some of the properties that are active during normal odontogenesis Odontogenic Tumors: Malignant 6.8 Odontogenic Sarcomas Fig 6.35 At higher magnification, the cellularity and atypia in odontogenic carcinosarcoma becomes more apparent Fig 6.36 High power view from Fig 6.35 to illustrate the mitotic activity in both epithelial as well as mesenchymal cells 119 120 Fig 6.37 Odontogenic carcinosarcoma; sections immunohistochemically stained for expression of the proliferation marker MIB-1 Extensive positivity is shown both by epithelial as well as mesenchymal cells Fig 6.38 Ghost cell differentiation within the epithelial islands in odontogenic carcinosarcoma Odontogenic Tumors: Malignant References 121 Fig 6.39 Ghost cells may also be seen at the interface of enamel epithelium and dysplastic dentin in odontogenic carcinosarcoma References Barnes L, Eveson JW, Reichart PA, Sidransky D, editors World Health Organization Classification of Tumours Pathology and Genetics of Head and Neck Tumours Lyon: IARC Press; 2005 Chapter 6, Odontogenic Tumours; p 283–327 Slootweg PJ Malignant odontogenic tumors; an overview Mund Kiefer Gesichtschir 2002;6(5):295–302 Richardson MS, Muller S Malignant odontogenic tumors: an update on selected tumors Head Neck Pathol 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A new case and comparative analysis of the literature J Oral Maxillofac Surg 2003;61(9):1004–10 Mosqueda-Taylor A, Neville BW, Tatemoto Y, Ogawa I, Takata T Odontogenic carcinoma with dentinoid: a new odontogenic carcinoma Head Neck Pathol 2014;8(4):421–31 Bilodeau EA, Weinreb I, Antonescu CR, Zhang L, Dacic S, Muller S, Barker B, Seethala RR Clear cell odontogenic carcinomas show EWSR1 rearrangements: a novel finding and a biological link to salivary clear cell carcinomas Am J Surg Pathol 2013;37(7):1001–5 Hirshberg A, Dayan D, Horowitz I Dentinogenic ghost cell tumor Int J Oral Maxillofac Surg 1987;16(5):620–5 Lu Y, Mock D, Takata T, Jordan RC Odontogenic ghost cell carcinoma: report of four new cases and review of the literature J Oral Pathol Med 1999;28(7):323–9 Koutlas IG, Allen CM, Warnock GR, Manivel JC Sclerosing odontogenic carcinoma: a previously unreported variant of a locally aggressive odontogenic neoplasm without apparent metastatic potential Am J Surg Pathol 2008;32(11):1613–9 Odontogenic Tumors: Malignant 26 Woolgar JA, Triantafyllou A, Ferlito A, Devaney KO, Lewis Jr JS, Rinaldo A, Slootweg PJ, Barnes L Intraosseous carcinoma of the jaws: a clinicopathologic review Part II: odontogenic carcinomas Head Neck 2013;35(6):902–5 27 Ide F, Ito Y, Muramatsu T, Saito I Sclerosing odontogenic carcinoma: a morphologic pattern or pathologic entity? Oral Surg Oral Med Oral Pathol Oral Radiol 2013;115(6):839 28 Iriè T, Ogawa I, Takata T, Toyosawa S, Saito N, Akiba M, et al Sclerosing odontogenic carcinoma with benign fibro-osseous lesion of the mandible: an extremely rare case report Pathol Int 2010;60(10):694–700 29 Slater LJ Odontogenic sarcoma and carcinosarcoma Semin Diagn Pathol 1999;16(4):325–32 30 Altini M, Thompson SH, Lownie JF, Berezowski BB Ameloblastic sarcoma of the mandible J Oral Maxillofac Surg 1985;43(10):789–94 31 Muller S, Parker DC, Kapadia SB, Budnick SD, Barnes L Ameloblastic fibrosarcoma of the jaws A clinicopathologic and DNA analysis of five cases and review of the literature with discussion of its relationship to ameloblastic fibroma Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1995;79(4):469–77 .. .Pathology of the Maxillofacial Bones Pieter Slootweg Pathology of the Maxillofacial Bones A Guide to Diagnosis Pieter Slootweg Radboud University Nijmegen Medical Center Department of Pathology. .. International Publishing Switzerland 2 015 P Slootweg, Pathology of the Maxillofacial Bones: A Guide to Diagnosis, DOI 10 .10 07/978-3- 319 -16 9 61- 3 _1 Non-neoplastic Diseases Fig 1. 2 Torus histologically... confined to the maxillofacial bones Prime examples of these are the odontogenic cysts and tumors Other lesions are not confined to the maxillofacial skeleton but may pose differential diagnostic