Bone and Soft Tissue Tumors Clinical Features, Imaging,
Pathology and Treatment, 2nd Edition
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CONTENTS
Foreword to the first edition by W.F. Enneking· · · · · · · · · · · · · · · · · · · · · · · · · · · · XV
Preface to the first edition (1990) · · · · · · · · · · · · · · · · · · · · · · · · · · · · · · · · · · XV/1
Preface to the second edition ( 1999) · · · · · · · · · · · · · · · · · · · · · · · · · · · · · · · · · XIX
1. Introduction 1
Terminology . 1
Hyperplasia (reactive or reparative process). 1
Hamartoma, dysplasia . . . . 1
Benign tumor . . . . . . . . . 1
Low-grade malignant tumor . 2
High-grade malignant tumor . 2
Metaplasia . . . . . . . . . . 2
Anaplasia . . . . . . . . . . . 3
Tumor progression, dedifferentiation 3
Periosteal, parosteal, juxtacortical . . 3
Normal ultrastructure of the cell . . . 3
Normal tissues and their embryonal development . 4
Fibroblasts, myofibroblasts, fibrocytes 5
Histiocytes and macrophages . . . 6
Chondroblasts, chondrocytes. . . . 6
Osteoblasts, osteocytes, osteoclasts 7
Smooth muscle cells . . . . . . 8
Striated (skeletal) muscle cells . . . 8
Lipoblasts, lipocytes . . . . . . . . 9
Angioblasts, vascular endothelium, pericytes, glomus bodies . 9
Synovium . . . . . . . . . 10
Mast cells . . . . . . . . . 11
Neural crest derived cells . 11
Tumor classification 12
Frequency . . . . . . 16
Bone tumors. . . . 16
Soft tissue tumors . 18
Data (other than biopsy) contributory to diagnosis. 20
History and clinical features 21
Radiography. . . . . . . 29
Angiography . . . . . . . . 38
Radionuclide bone scan . . . 38
Computerized tomography (CT) 39
Magnetic resonance imaging (MRI) 39
VI CONTENTS
Echography . 40
Laboratory tests . 41
Gross pathology. 41
Biopsy . . . . . . . 41
Histology and cytology 43
Staging . . . . . . . . . 46
Tumor growth and encapsulation . 47
Anatomic compartments . . . . . 48
Surgical staging system (Enneking) 49
Pathologic fracture . . . . . . . . . . 52
Surgical contamination, transplantation 53
Local recurrence and metastases . . . . . 53
Surgical margins . . . . . . . . . . . . . 54
Surgical indications according to the stage . 56
Local adjuvants . . . . . . . . . . . . . . . . 57
Curettage (phenol and cement as adjuvants) . 57
Resections. General principles . . . 58
Reconstruction. General principles . 63
Following curettage . . . . . . . . . 63
Following osteo-articular resections 64
Reconstruction in children 66
Chemotherapy. 67
Radiotherapy . . . . . . . . 67
BONE TUMORS
2. Histiocytic fibroma . . . . . . . . . . . . . . . . . . . . . 73
Multiple histiocytic fibromas with extraskeletal anomalies 85
3. Benign fibrous histiocytoma . . . . . . . . 93
4. Giant cell tumor . . . . . . . . . . . . . . 99
Sarcoma associated with giant cell tumor . 133
Giant cell tumor in pagetic bone. 136
5. Desmoid fibroma 143
6. Fibrosarcoma . . 149
7. Malignant fibrous histiocytoma 161
8. Leiomyosarcoma 175
9. Exostosis . . . . . 179
10. Multiple hereditary exostoses. 197
11. Hemimelic epiphyseal dysplasia 207
12. Chondroma. . . . . . . 213
13. Periosteal chondroma . 229
CONTENTS VII
14. Multiple chondromas 235
15. Chondroblastoma . . 247
16. Chondromyxoid fibroma 265
17. Fibrocartilaginous mesenchymoma 279
18. Central chondrosarcoma . . . . . . 283
19. Dedifferentiated central chondrosarcoma . 319
20. Peripheral chondrosarcoma. 335
21. Periosteal chondrosarcoma . 363
22. Clear cell chondrosarcoma . 369
23.Mesenchymalchondrosarcoma. 375
24. Osteoma and bone islands. 381
25. Osteoid osteoma . 391
26. Osteoblastoma . . 415
27. Fibrous dysplasia 435
28. High grade osteosarcomas. 463
Classic osteosarcoma . . . . 464
Telangiectatic osteosarcoma. 491
Osteosarcoma of the jaw-bones . 500
Secondary osteosarcoma. . . . . 500
Small cell osteosarcoma . . . . . 501
High-grade surface osteosarcoma . 503
Intracortical osteosarcoma. 505
Multifocal osteosarcoma. . 505
29. Periosteal osteosarcoma . 517
30. Parosteal osteosarcoma . 525
31. Central low-grade osteosarcoma . 549
32. Primary lymphoma of bone . 559
33. Hodgkin's disease 575
34. Leukemia . . . . . 579
35. Multiple myeloma . 581
Solitary plasmacytoma of bone 593
Extramedullary plasmacytoma 595
36. Hemangioma . . . . . . . . . 599
37. Limphangioma, cystic angiomatosis 619
VIII CONTENTS
38. Hemangioendothelioma, benign, low-grade, high-grade (angiosarcoma) . 623
39. Hemangiopericytoma . . . . . 643
40. Neurilemoma, neurofibroma . 647
41. Ewing's sarcoma, primitive neurectodermal tumor (PNET). 653
42. Lipoma, liposarcoma, malignant mesenchymoma. 683
43. Chordoma . . . . . . . . . . . . . . . . . . . 689
44. Osteofibrous dysplasia and adamantinoma . 707
Osteofibrous dysplasia. . . . 714
Adamantinoma. . . . . . . . 721
45. Sarcoma in Paget's disease 733
46. Carcinoma and sarcoma in chronic osteomyelitis . 737
47. The effects of radiation on the skeleton and radiation induced sarcomas
(of bone and soft tissues) . . . . . . . . . . . . . . . . . . . . . . . . . . . 741
48. Sarcomas on bone infarct, bone necrosis, or at the site of metallic implants . 751
49. Metastatic bone disease . . 755
Bone metastases in children . 780
TUMORLIKE LESIONS OF BONE
50. Simple bone cyst . . . . . 791
"Cyst" of the calcaneus . 807
51. Aneurysmal bone cyst . 813
52. Intraosseous and periosteal mucous cyst. 841
53. Massive idiopathic osteolysis . 849
54. Langherans' cell histiocytosis. 857
Eosinophilic granuloma of bone. 857
Hand-Schiiller-Christian disease 869
Letterer-Siwe disease . . . . . . 874
55. "Brown tumors" in primary hyperparathyroidism . 877
56. Reparative giant cell granuloma . . . . . . . . . . . 901
SOFT TISSUE TUMORS
57. (Subdermal) fibrous hamartoma of infancy . 909
58. Infantile digital fibromatosis . . . . . . . . . 913
CONTENTS IX
59. Calcifying aponeurotic fibroma. 917
60. Infantile myofibroma, myofibromatosis . 921
61. Aggressive fibromatosis . 925
62. Fibrosarcoma . . . . . . 937
63. Benign fibrous histiocytoma 949
64. Dermatofibrosarcoma protuberans 953
65. Atypical fibroxanthoma of the skin. 959
66. Subcutaneous angiomatoid fibrous histiocytoma . 961
67. Malignant fibrous histiocytoma. 965
68. Lipomas. . . . . . . . . . 983
Lipoma . . . . . . . . . . . 983
Subcutaneous angiolipoma 991
Subcutaneous spindle cell lipoma and subcutaneous pleomorphic lipoma . 993
Benign lipoblastoma and lipoblastomatosis . 993
Intranervous and perinervous fibrolipoma. 995
Diffused lipomatosis. 996
Hibernoma . . . . . . . . . . . . . . . . . 996
69. Liposarcoma . . . . . 1001
70. Leiomyoma . . . . . . 1021
Cutaneous leiomyomas. 1021
Subcutaneous vascular leiomyoma. 1021
Leiomyoma of the deep soft tissues 1023
71. Leiomyosarcoma . . . . . . . . . 1025
Cutaneous and subcutaneous leiomyosarcoma. 1025
Deep leiomyosarcoma . 1027
72. Rhabdomyoma . . . . 1033
73. Rhabdomyosarcoma . 1037
74. Angiomas and angiodysplasias 1051
Superficial hemangiomas . . . . 1052
Single deep hemangioma. . . . . 1053
Multifocal and diffused hemangioma 1063
Synovial hemangioma . 1068
Angiodysplasias 1072
Lymphangioma. 1077
75. Glomus tumor 1083
76. Epithelioid hemangioendothelioma and angiosarcoma 1089
Epithelioid hemangioendothelioma 1089
Angiosarcoma . . . . . . . . . . . . . . . . . . . . . . . 1091
X CONTENTS
77. Kaposi's sarcoma. 1097
78. Hemangiopericytoma. 1101
79. Synovial sarcoma . 1109
80. Neurilemoma . . . 1127
81. Neurofibroma, neurofibromatosis. 1135
Solitary neurofibroma 1135
Neurofibromatosis . . 1136
82. Granular cell tumor 1145
83. Malignant peripheral nerve sheath tumor . 1149
84. Clear cell sarcoma of tendons and aponeuroses . 1161
85. Ganglioneuroma, Ganglioneuroblastoma, neuroblastoma . 1167
Ganglioneuroma . . . . . . . . . . . . . . . . . . . . . . . . . 1167
Neuroblastoma and ganglioneuroblastoma . . . . . . . . . . . 1168
86. Primitive neurectodermal tumor (PNET) and extraskeletal Ewing's sarcoma 1175
87. Extraskeletal chondrosarcomas . 1181
E.s. myxoid chondrosarcoma. . . . 1181
E.s. mesenchymal chondrosarcoma 1184
88. Extraskeletal osteosarcoma 1189
89. Alveolar soft part sarcoma. 1193
90. Epithelioid sarcoma . . . . 1199
TUMORLIKE LESIONS OF THE SOFT TISSUES
91. Palmar fibromatosis (Dupuytren's d.), plantar fibromatosis (Ledderhose's d.). 1209
92. Nodular fasciitis, proliferative fasciitis, proliferative myositis 1213
93. Elastofibroma. 1221
94. Xanthoma . . . 1223
95. Intramuscular myxoma 1227
96. Mucous cyst. . . . . . 1233
97. Amputation neuroma 1239
98. Synovial chondromatosis, extraskeletal chondroma, synovial chondrosarcoma. 1243
Synovial chondromatosis, extraskeletal chondroma. 1243
Synovial chondrosarcoma 1253
99. Tumoral calcinosis . . . 1257
CONTENTS XI
100. Pseudotumoral soft tissue and periosteal ossifications . 1265
Myositis and periostitis ossificans . . . . . . . . . . . . . . 1265
Bizarre parosteal osteochondromatous proliferation (Nora's lesion) 1280
Subungual "exostosis" . . . . . . . . . . . . . . . . . . . . . . 1281
Hyperplastic bone callus in neuropathy . . . . . . . . . . . . . 1282
Myo-periostitis ossificans associated with congenital disorders. 1284
Fibrodysplasia ossificans progressiva (Munchmeyer' s disease) . 1284
101. Pigmented villo-nodular synovitis . . . . . . . . . . . . . . 1289
Subject index . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1307
To Giuliana
FOREWORD
to the first edition (1990)
This is an extraordinary book by an extraordinary author. Mario Campanacci first pub-
lished three volumes on musculoskeletal neoplasms and other tumor-like processes in bone
and soft parts in Italian in 1981-1985. This book is an update and expansion of that book,
published for the first time in English. In this book Dr. Campanacci brings to the readers the
vast experience in musculoskeletal oncology of the Rizzoli Orthopaedic Institute in Bologna
where he has been head of the Oncology Unit for many years. As such, he has had at his dis-
posal the patient records, radiographs and pathologic material dating back to 1905. In fact, a
visitor to the Institute will be shown the radiograph made of the first tumor case on records -
that of a giant cell tumor of the distal femur. The wealth of clinical material that has been ac-
cumulated at the Rizzoli Institute, with exquisite documentation and maintenance is a unique
resource and testimonial to not only the author but his predecessors. Under Campanacci' s
leadership, the Institute has provided care to the majority of patients with neoplasms through-
out Italy. Over the past two decades a treatment team with extraordinary ability in radiology,
imaging, pathology, chemotherapy, as well as orthopedic surgery has been assembled. The In-
stitute has been a major contributor to the literature of musculoskeletal oncology, has played
an important role in the Musculoskeletal Tumor Society of North America, and taken a lead-
ership role in the establishment of the European Musculoskeletal Oncology Society. This
book brings to the reader an almost unparalleled experience from one of the leading centers of
musculoskeletal oncology in the world.
The book first deals with the general principles of musculoskeletal oncology in an exem-
plary fashion as befits their experience. It then presents an extensive series of chapters, each
devoted to a particular entity. Within each chapter the lesion in question is clearly defined in
its frequency, sexual predilection, age of occurrence, and the anatomic localization with a de-
tailed discussion of each point. Figures quoted are those derived from the experience at the In-
stitute. The reader is then presented with the clinical picture and symptoms, the imaging
characteristics of the lesion, and the gross and histopathologic features, all beautifully illus-
trated. Following this, the differential diagnosis is discussed in detail. Next, the clinical course
without treatment is outlined. The next section of each chapter is a discussion of the treatment
of the lesion in question and includes both surgical, chemotherapeutic and radiotherapeutic
modalities. The final portion of each chapter is devoted to a discussion of the prognosis as it is
currently known. Each chapter is beautifully illustrated with exceptional clarity that allows
the reader an accurate portrayal of the lesion under discussion.
The manuscript is not simply a translation from Italian into English, rather an expansion
of the previous text written in English. It retains the author's clarity of thought, conciseness,
and sharp focus on the important issues, and, in this context, brings to the reader an excep-
tionally cosmopolitan international view of this subject. This book is a must for all those in-
XVI FOREWORD
volved in the care of patients with musculoskeletal neoplasms regardless of their medical
speciality. To those students, residents and fellows in orthopaedic surgery, radiology, medical
oncology, pathology and radiation oncology, it will provide an invaluable resource and inspi-
ration. We are greatly in Dr. Mario Campanacci's debt for the prodigious effort this singularly
authored authoritative text has required.
W.F. ENNEKING
PREFACE
to the first edition (1990)
At the Istituto Ortopedico Rizzoli the interest on bone tumors was pioneered by V. Putti,
who created a pathology laboratory and museum, successfully performed extrarticular resec-
tion of the proximal femur and acetabulum in 1914, devised and widely used a technique of
resection/arthrodesis of the knee (1923) whose principles have been in use until our days. His
pupil, 0. Scaglietti, fostered the laboratory of pathology and the study of bone tumors up to
1941. F. Delitala introduced in 1945 the replacement of large segmental resections with origi-
nal stainless steel articular endoprostheses, which functioned for decades. In 1950 R. Zanoli
used intercalary massive xenografts. I.F. Goidanich founded in 1955 the Tumor Centre at the
Institute, reviewing and redefining all previous cases according to the new concepts intro-
duced by H. Jaffe in those years. I started working with Goidanich both in orthopaedic sur-
gery and in pathology in 1958 and shortly after studied with L. Lichtenstein in San Francisco.
To both those admired and beloved teachers I am indebted for everything I have been able to
do thereafter and for this book.
Today the Tumor Centre of the Institute records about 15,000 cases, complete with clini-
cal charts, original X -ray and histological slides. More than 90% of those cases have been
treated at the Institute, the remaining are consultation cases.
The experience with these cases constitutes the matter of this book. Any case where diag-
nosis was debatable was excluded. No mention will be made of exceptional lesions which we
never saw, nor of lesions of the jaws, with which we have hardly any experience. Some illus-
trations will present cases where the treatment adopted is incorrect according to our present
standards; those cases are decades old.
We think that a book has to be simple to be readable. We therefore avoided all unneces-
sary details and not well-established facts. The bibliography has also been limited to the most
significant and recent publications.
Musculo-skeletal oncology is a multidisciplinary speciality. Our work and consequently
this book would not have been possible without the joint effort of the pathologists ( P. Bac-
chini, F. Bertoni, P. Picci), the medical oncologist (G. Bacci), the general surgeons (A. Eric-
coli, N. Guernelli), the orthopaedic surgeons (S. Boriani, R. Capanna, A. Giunti, F.
Gherlinzoni, A. Guerra, C. Leonessa, M. Mercuri, G. Padovani, A. Toni). To all these friends
I am greatly in debt because I profited greatly from their work, enthusiasm and ingenuity. A
special acknowledgment to doctors M. Laus, E. Lorenzi, P. Ruggieri and N. Fabbri for their
precious and essential help in the preparation of the pictures.
M. CAMPANACCI
PREFACE
to the second edition (1999)
The first English edition of Bone and Soft Tissue tumors appeared in 1990. This was a
translation, although up-dated, of a prevoius book published in Italian in 1986.
This second english edition, on the contrary, is an entirely new book. Indeed, we found
the text of the first edition so uncomplete in informations, so unbalanced in the distribution,
and so poor in the language, that it immediately appeared more practical or, better, necessary
to write everything again and directly in English. This edition infact has been thoroughly re-
written, from the first to the last word. About 30% of the pictures are new.
The new book incorporates the accumulated personal experience of the Author, covering
over 20000 inpatients and many more outpatients, the perusal of the literature of the last I 0
years, the recent developments in imaging (particularly MRI), microscopic diagnosis (espe-
cially immunohistochemistry and electron microscopy) and the ultimate progress in surgical
and non surgical treatment modalities.
We have followed, for each tumor or tumor-like lesion, the rule of starting the study in
the outpatient clinic and hospital wards (clinical findings and imaging), continuing it at the
operating table and on the entire and cross-sectioned resected specimen (gross surgical patho-
logy), than at the microscope (histopathology), and finally reviewing the long-term results of
surgical and combined surgical + local and/or general adjuvant therapies. All these observa-
tions are substantially based on the personal experience of the Author.
The text has been further digested to the essential, and the strict and uniform distribution,
for each entity, in paragraphs (from definition to prognosis) is meant to avoid any overlapping
or repetition, and to facilitate the study and the consultation. Also the references have been
trimmed to the more recent and significant papers. Infact, notwithstanding the accrued new
informations, the text is shorter as compared to the previous first English edition. The number
of pages is slightly increased because of the pictures, which were 2249 in the first edition, and
2820 in this second edition.
In comparison with all the other books published until now on the same subject, it can be
noticed that some chapters (for instance hemangioma and angiodysplasias, simple bone cyst,
aneurysmal bone cyst, "brown tumors" in primary hyperparathyroidism, "myositis ossificans"
and exuberant bone callus, synovial chondromatosis, pigmented villo-nodular synovitis) have
a more extensive description. This reflects the personal experience of the Author and the im-
portance that such lesions have in the orthopedic practice and differential diagnosis with tu-
mors. We have also described together the hamartomas, dysplasias, and benign tumors,
including instead in a section of tumor-like lesions those caused by hyperplastic (reparative),
or metaplastic, or degenerative processes which may mimic a tumor.
The subject of this book reflects the experience of an Orthopedic Hospital. Therefore,
bone tumors cover two thirds, and soft tissue tumors one third of the volume. For the same
XX PREFACE
reason, the lesions of the skull, jaws, ribs, cutis and subcutis are largely underestimated. The
tumors of the retroperitoneum, mucosae, and visceral organs are not represented. Systemic tu-
mors as multiple myeloma and lymphoma are also underestimated.
Our epidemiology charts are influenced by other bias. Because we have considered, in the
preparation of these charts, the inpatients only, mostly operated on and having a histological
confirmation, the frequency of some lesions is widely underestimated: histiocytic fibroma,
exostosis, chondroma, simple bone cyst, fibrous dysplasia, myositis ossificans, and others.
The age indicated in the charts refers to the age at the time of diagnosis. In the cases of ha-
martomas, or developmental abnormalities which initiate during childhood, and in the case of
slow-growing tumors, the age of inception of the lesion is presumably much younger than in-
dicated here.
We are deeply grateful to prof. Franco Bertoni and dr. Patrizia Bacchini for the contribu-
tion to the histological illustrations, and to dr. Piero Picci for the preparation of the epidemiol-
ogy charts.
M. CAMPANACCI
1. INTRODUCTION
TERMINOLOGY
Hyperplasia
(reactive or reparative process)
Hyperplasia is an accumulation of cells due to either accelerated proliferation or slowed
maturation and degeneration. Hyperplasia is caused by a stimulus, and it ceases when the
stimulus is exhausted. Hyperplasia is functional: as such, it has an organoid structure and it
tends to differentiate and mature. Examples are exuberant bone callus, myositis ossificans,
brown tumors in hyperparathyroidism, and, probably, aneurysmal bone cyst, pigmented vil-
lonodular synovitis, synovial chondromatosis.
Hamartoma, dysplasia
During the embryonal, fetal or infantile development an island of tissue may be excluded
from regional organization, and left unused. This developmental error is known as hamartia'.
Hamartia may continue to grow independently and the product of this growth, similar to a be-
nign tumor, is known as a hamartoma. Similar to hamartoma is dysplasia, resulting in tu-
morlike overgrowth of normal or abnormal tissue initiating during the developmental age.
Like hyperplasia, hamartoma and dysplasia have a relatively orderly structure, and often tend
to exhaust their growth and mature after puberal age. Examples include exostoses, chondro-
mas, fibrous dysplasia, angiomas and neurofibromas. Another feature of hamartomas and dys-
plasias is that they may be multicentric or diffused, often prevalently involving one side of the
body, and may associate with other congenital anomalies.
Benign tumor
Its growth is autonomous, usually slower than that of malignant tumors. Cellular mor-
phology is typical, tissue structure tends to be less disorderly and more organoid than that of
malignant tumors. The cells tend to differentiate, and often preserve a considerable amount of
their specific function. Growth is by pushing rather than permeating, thus the benign tumor is
1From the Greek amarta =error, fault.
2 BONE AND SOFT TISSUE TUMORS
usually well demarcated from the surrounding tissues. It does not recur after it has been re-
moved completely, and it does not metastasize. Examples: giant cell tumors of bone, osteoid
osteomas, osteoblastomas, chondroblastomas, chondromyxoid fibromas, and, in the soft tis-
sues, lipomas, leiomyomas, neurilemomas.
Low-grade malignant tumor
Its growth is rather slow, but more progressive than that of benign tumors as it may even
become of enormous size. Growth is partially permeative, thus, the limits are less well-
defined than those of benign tumors. If surgical removal does not include a layer of healthy
surrounding tissues, the tumor easily recurs. It infrequently metastasizes; nonetheless, the tu-
mor may manifest progressive malignancy in time, becoming high-grade. Examples: grades 1,
2 chondrosarcomas, grades 1, 2 parosteal osteosarcomas, and, in the soft tissues, dermatofi-
brosarcoma protuberans, low-grade liposarcomas.
High-grade malignant tumor
Its growth is generally rapid and aggressive. Cellular morphology is atypical, tissue struc-
ture anarchical. The cell's differentiation, maturation and specific function are absent or de-
creased, and more or less anomalous. Growth is invasive and permeative, thus the tumor lacks
well-defined limits with the surrounding tissues. It locally recurs if removal does not include a
wide layer of healthy surrounding tissues, or the entire original anatomical compartment, and
it tends to easily metastasize. Examples: classic osteosarcomas, Ewing's sarcomas, grade 3
chondrosarcomas, and, in the soft tissues, high-grade liposarcomas, malignant fibrous histio-
cytomas, rhabdomyosarcomas, synovial sarcomas.
* * *
These definitions are schematic and approximate. At times it is difficult to define the lim-
its between hyperplasia, hamartoma, dysplasia and benign tumor. A hamartoma or dysplasia
may, even after many years, constitute the site where malignant tumors originate (see exosto-
sis, chondroma, fibrous dysplasia and, in the soft tissues, von Recklinghausen' s neurofibro-
matosis). There are benign lesions which grow rapidly, or (such as aggressive fibromatosis of
the soft tissues) which have permeative growth. On the contrary, some malignant tumors are
characterized by slow growth; others remain small and clearly circumscribed, but they none-
theless metastasize; others achieve a high degree of differentiation (chondrosarcomas of
bone), or have an organoid structure (alveolar soft part sarcoma).
Metaplasia
Is a change in which one differentiated cell type transforms into differently differentiated
type. For example, fibroblasts may transform into chondroblasts or osteoblasts.
