Bone and Soft Tissue Tumors: A Multidisciplinary Review with Case Presentations Therese J. Bocklage, Robert H. Quinn, Berndt P. Schmit, Claire F. Verschraegen
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1BONE AND SOFT TISSUE TUMORS: A Multidisciplinary Review with Case Presentations2
3BONE AND SOFT TISSUE TUMORS: A Multidisciplinary Review with Case Presentations
THERESE J. BOCKLAGE, MD Professor of Pathology University of New Mexico School of Medicine Health Sciences Center Director, University of New Mexico Human Tissue Repository Albuquerque, New Mexico, USA ROBERT H. QUINN, MD Department Chair and Professor Residency Program Director John J. Hinchey MD and Kathryn Hinchey Chair in Orthopaedics Department of Orthopaedic Surgery University of Texas Health Science Center at San Antonio San Antonio, Texas, USA BERNDT P. SCHMIT, MD Assistant Clinical Professor of Radiology University of Pittsburgh, UPMC East Radiology Department Monroeville, Pennsylvania, USA CLAIRE F. VERSCHRAEGEN, MD Irwin H. Krakoff Professor of Medicine Director, Hematology Division Director, Fletcher Allen Health Care Cancer Service Line Co-Director, Vermont Cancer Center Burlington, Vermont, USA
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5Preface
Patients with a bone or soft tissue tumor require a highly efficient, multidisciplinary team of physicians for optimal care. These tumors are rarely encountered in routine practice and often require diagnosis and treatment at specialized centers. However, patients frequently present to practices and institutions that are not specialized centers. Unfortunately, the information sources available to the frontline providers working in these centers are limited and rarely available in a concise, accessible format. We hope this textbook will meet the needs of these providers, and also serve as a valuable resource for specialty clinicians.
We wrote this book with two main objectives; firstly, we wanted to provide, in one volume, a practical guide to the diagnosis of bone and soft tissue tumors and the management of patients with these conditions; secondly, we wanted to optimize learning by presenting the information in two formats. The first format comprises the introduction to each chapter which presents reference information efficiently and informatively, allowing rapid review of a specific category of tumors. The second format comprises the remainder of each chapter, where the tumors are presented in more depth using case presentations that enhance learning thanks to their problem-based approach. The cases are not hypothetical and derive from our clinical experience. They illustrate that not every patient follows the textbook description in terms of presentation, diagnostic features and outcome.
This book grew out of a partnership of equals: an orthopaedic surgeon, an oncologist, a musculoskeletal radiologist, two radiation oncologists and a pathologist. We all found that the whole was, indeed, greater than the sum of the parts: that frank, respectful team discussion about each patient resulted in improved care. In light of this, our text is grounded in clinical reality, comprehensive and genuinely multidisciplinary; experts from participating disciplines have contributed to all chapters.
Having experienced for ourselves the increasing complexity of caring for patients in terms of new imaging modalities, surgical approaches, drugs, protocols and classification schemes, we understand the deluge of information a postgraduate medical trainee faces. We hope this book helps to alleviate this, while also engaging and educating the reader.
Therese J. Bocklage
Robert H. Quinn
Berndt P. Schmit
Claire F. Verschraegen
March 2014
6Acknowledgements
We wish to thank the contributing authors listed below for providing content as follows:
Angela Meisner, MPH: SEER epidemiology in all chapters.
Ben Liem, MD: Radiation oncology in chapters 7, 8, 12, 13, 14, 17, 18, 21, 22, 26, 27.
Thomas Schroeder, MD: Radiation oncology in chapters 4, 5, 6, 9, 11, 15, 16, 19, 24, 25.
Sagus Sampath, MD: Radiation oncology in chapters 10, 20, 23.
Thomas Williams, MD: Molecular genetics of bone and soft tissue tumors in chapter 1.
David Meredith, MD: UNM epidemiology data and bone and soft tissue statistics.
Samuel Reynolds, MD: UNM epidemiology data bone and soft tissue statistics.
Jennifer Pincus, MD: UNM epidemiology data bone and soft tissue statistics.
Aaron Pritchard, MD: UNM epidemiology data bone and soft tissue statistics.
Myra Zucker, PA: Gross pathology photographs.
We are deeply grateful to Teresa Quintana, our impeccably organized, efficient and dedicated administrative assistant who cheerfully and expertly performed multiple tasks for this project, and to Michael Grady, a specialist in optimizing images for publication who carefully improved the quality of every image in the text.
We thank Charles Key for his expertise and guidance with the SEER epidemiology portions and we appreciate the many hours of work Julia Kreger performed in sourcing radiology images and reviewing patient charts.
We thank Gavin Smith of JP Medical Publishers for his discerning editing and responsive, cordial communication.
TB, RQ, BS, CV
7Dedications
To our families who patiently supported us:
My parents, Jeanne and Bill, and my sisters, Cam and Paula (Therese Bocklage)
Stephanie, Taryn, Hunter and Devin (Robert Quinn)
Julie, my wonderful wife; Lukas, Karina and Carsten, my precious children (Berndt Schmit)
Ram Murthy, my husband, and my children, Victor and Adeline for tolerating my long stay at the computer (Claire Verschraegen)
My beautiful daughter, Brooke, and my husband, Ben (Angela Meisner)
My wife, Ann and my daughter, Sophia (Ben Liem)
Piper, Electra, & Remington; and especially Sabrina (Thomas Schroeder)
To our trainees whose enthusiasm inspired us.
To our patients who continue to teach us.8
WORLD HEALTH ORGANIZATION (WHO) 2013 CLASSIFICATION OF BONE TUMORS
Benign
Intermediate (locally aggressive)
Intermediate (rarely metastasizing)
Malignant
Chondrogenic tumors
1. Osteochondroma
2. Enchondrdoma
3. Periosteal chondroma
4. Osteochondromyxoma
5. Subungual exostosis
6. Bizarre parosteal osteochondromatous proliferation (BPOP)
7. Synovial chondromatosis
1. Chondromyxoid fibroma (CMF)
2. Atypical cartilaginous tumor/chondrosarcoma grade I
Chondroblastoma
1. Chondrosarcoma grade II, grade III
2. Dedifferentiated chondrosarcoma
3. Mesenchymal chondrosarcoma
4. Clear cell chondrosarcoma
Osteogenic tumors
1. Osteoma
2. Osteiod osteoma
1. Osteoblastoma
None
1. Low-grade central osteosarcoma
2. Conventional osteosarcoma including fibroblastic, chondroblastic, and osteoblastic types
3. Telangiectatic osteosarcoma
4. Small cell osteosarcoma
5. Secondary osteosarcoma
6. Parosteal osteosarcoma
7. Periosteal osteosarcoma
8. High-grade surface osteosarcoma
Fibrogenic tumors
None
1. Desmoplastic fibroma of bone
None
1. Fibrosarcoma of bone
Fibrohistiocytic tumors
1. Benign fibrous histiocytoma/non-ossifying fibroma
None
None
None
Hematopoietic neoplasms
None
None
None
1. Plasma cell myeloma
2. Solitary plasmacytoma of bone
3. Primary non-Hodgkin lymphoma of bone
Osteoclastic giant cell rich tumors
1. Giant cell lesion of the small bones
Giant cell tumor of bone
Giant cell tumor of bone
1. Malignancy in giant cell tumor of bone
Notochordal tumors
1. Benign notochordal tumor
None
None
1. Chordoma
Vascular tumors
1. Hemangioma
1. Epithelioid hemangioma
1. Epithelioid hemangioma
1. Epithelioid hemangioendothelioma (EHE)
2. Angiosarcoma
Myogenic tumors
1. Leiomyoma
None
None
1. Leiomyosarcoma
Lipogenic tumors
1. Lipoma
None
None
1. Liposarcoma
Tumors of undefined neoplastic nature
1. Simple bone cyst
2. Fibrous dysplasia
3. Osteofibrous dysplasia
4. Chondromesenchymal hamartoma
5. Rosai-Dorfman disease
1. Aneurysmal bone cyst
2. Langerhans cell histiocytosis, monostotic and polyostotic
3. Erdheim-Chester disease
None
None
Miscellaneous tumors
None
None
None
1. Ewing sarcoma
2. Adamantinoma
3. Undifferentiated high-grade pleomorphic sarcoma of bone
Rarely, other sarcomas of soft tissue, in addition to those listed in the WHO 2013 Classification of Bone Tumors, have been described as originating in bone (in scattered case reports).
9
WORLD HEALTH ORGANIZATION (WHO) 2013 CLASSIFICATION OF SOFT TISSUE TUMORS
Benign
Intermediate (locally aggressive)
Intermediate (rarely metastasizing)
Malignant
Adipocytic tumors
1. Lipoma
2. Lipomatosis
3. Lipomatosis of nerve
4. Lipoblastoma/lipoblastomatosis
5. Angiolipoma
6. Myolipoma
7. Chondroid lipoma
8. Extra-renal angiomyolipoma
9. Extra-adrenal myelolipoma
10. Spindle cell/pleomorphic lipoma
11. Hibernoma
1. Atypical lipomatous tumor/well differentiated liposarcoma
None
1. Dedifferentiated liposarcoma
2. Myxoid liposarcoma
3. Pleomorphic liposarcoma
4. Liposarcoma, NOS
Fibroblastic/myofibroblastic tumors
1. Nodular fasciitis
2. Proliferative fasciitis
3. Proliferative myositis
4. Ischemic fasciitis
5. Myositis ossificans
6. Fibro-osseous pseudotumor of digits
7. Elastofibroma
8. Fibrous hamartoma of infancy
9. Fibromatosis colli
10. Juvenile hyaline fibromatosis
11. Inclusion body fibromatosis
12. Fibroma of tendon sheath
13. Desmoplastic fibroblastoma
14. Mammary-type myofibroblastoma
15. Calcifying aponeurotic fibroma
16. Angiomyofibroblastoma
17. Cellular angiofibroma
18. Nuchal-type fibroma
19. Gardner fibroma
20. Calcifying fibrous tumor
1. Palmar/plantar fibromatosis
2. Desmoid-type fibromatosis
3. Lipofibromatosis
4. Giant cell fibroblastoma
1. Dermatofibrosarcoma protuberans (DFSP) including fibrosarcomatous and pigmented versions
2. Solitary fibrous tumor (SFT)
3. Inflammatory myofibroblastic tumor (IMFT)
4. Myxoinflammatory fibroblastic sarcoma (MIFS) including atypical variant
5. Infantile fibrosarcoma
1. Adult fibrosarcoma
2. Myxofibrosarcoma
3. Low-grade fibromyxoid sarcoma (LGFMS)
4. Sclerosing epithelioid fibrosarcoma (SEFS)
5. Malignant solitary fibrous tumor (malignant SFT)
So-called fibrohistiocytic tumors
1. Tenosynovial giant cell tumor comprising localized type and diffuse type (the latter also commonly called pigmented villonodular synovitis)
2. Deep benign fibrous histiocytoma
None
1. Plexiform fibrohistiocytic tumor
2. Giant cell tumor of soft tissues
1. Malignant variant of diffuse type of tenosynovial giant cell tumor
Smooth muscle tumors
1. Deep leiomyoma
None
None
2. Leiomyosarcoma (excluding skin)
Pericytic/perivascular tumors
1. Glomus tumor and benign variants
2. Myopericytoma including myofibroma
3. Angioleiomyoma
1. Glomangiomatosis
2. Myofibromatosis
None
1. Malignant glomus tumor
2. Malignant myopericytoma
Skeletal muscle tumors
1. Rhabdomyoma including adult type, fetal type, genital type
None
None
1. Embryonal rhabdomyosarcoma (E-RMS) including anaplastic and botryoid cases
2. Alveolar rhabdomyosarcoma (A-RMS) including solid and anaplastic cases
3. Pleomorphic rhabdomyosarcoma (pleomorphic RMS)
4. Spindle cell/Sclerosing rhabdomyosarcoma (spindle cell/sclerosing RMS)
10
Benign
Intermediate (locally aggressive)
Intermediate (rarely metastasizing)
Malignant
Vascular tumors
1. Hemangioma including synovial, venous, ateriovenous hemangioma/malformation
2. Intramuscular hemangioma
3. Epithelioid hemangioma
4. Angiomatosis
5. Lymphangioma
1. Kaposiform hemangioendothelioma (kaposiform HE)
1. Retiform hemangioendothelioma (retiform HE)
2. Papillary intralymphatic angioendothelioma
3. Composite hemangioendothelioma (composite HE)
4. Pseudomyogenic (epithelioid-sarcoma like) hemangioendothelioma (pseudomyogenic HE)
5. Kaposi sarcoma
1. Epithelioid hemangioendothelioma (EHE)
2. Angiosarcoma
Chondro-
osseous tumors
1. Soft tissue chondroma
None
None
1. Extraskeletal mesenchymal chondrosarcoma
2. Extraskeletal osteosarcoma
Gastrointestinal stromal tumors
1. Benign gastrointestinal stromal tumor (benign GIST)
None
1. Gastrointestinal stromal tumor, uncertain malignant potential (GIST-UMP)
1. Gastrointestinal stromal tumor, malignant (GIST-Mal)
Nerve sheath tumors
1. Schwannoma (including variants)
2. Melanotic schwannoma
3. Neurofibroma (including variants such as plexiform neurofibroma)
4. Perineurioma
5. Granular cell tumor
6. Dermal nerve sheath myxoma
7. Solitary circumscribed neuroma
8. Ectopic meningioma
9. Nasal glial heterotopia
10. Benign Triton tumor
11. Hybrid nerve sheath tumors
None
None
1. Malignant peripheral nerve sheath tumor (MPNST)
2. Epithelioid malignant peripheral nerve sheath tumor (epithelioid MPNST)
3. Malignant Triton tumor
4. Malignant granular cell tumor
5. Ectomesenchymoma
6. Malignant perineurioma
Tumors of uncertain differentiation
1. Acral fibromyxoma
2. Intramuscular myxoma (including cellular variant)
3. Juxta-articular myxoma
4. Deep (‘aggressive’) angiomyxoma
5. Pleomorphic hyalinizing angiectatic tumor (PHAT) [may have borderline behavior]
6. Ectopic hamartomatous thymoma
1. Hemosiderotic fibrolipomatous tumor
1. Atypical fibroxanthoma (AFX)
2. Angiomatoid fibrous histiocytoma (AFH)
3. Ossifying fibromyxoid tumor (OFMT)
4. Mixed tumor, NOS
5. Myoepithelioma
6. Phosphaturic mesenchymal tumor
7. Perivascular epithelioid cell tumor (PEComa)
1. Synovial sarcoma (spindle cell, biphasic and NOS)
2. Epithelioid sarcoma
3. Alveolar soft part sarcoma (ASPS)
4. Clear cell sarcoma of soft tissue
5. Extraskeletal myxoid chondrosarcoma (ESMCS)
6. Extraskeletal Ewing sarcoma
7. Desmoplastic small round cell tumor (DSRCT)
8. Extra-renal rhabdoid tumor
9. Perivascular epithelioid cell tumor, malignant (malignant PEComa)
10. Intimal sarcoma
11. Ossifying fibromyxoid tumor, malignant (malignant OFMT-M)
12. Myoepithelial carcinoma
13. Phosphaturic mesenchymal tumor, malignant
Undifferentiated/unclassified sarcomas
None
None
None
1. Undifferentiated spindle cell sarcoma
2. Undifferentiated pleomorphic sarcoma
3. Undifferentiated round cell sarcoma
4. Undifferentiated epithelioid sarcoma
5. Undifferentiated sarcoma, NOS