Professional Documents
Culture Documents
Introduction
Staging Techniques
Surgical Staging System
Radiology of Bone Tumors Bone Tumors
Cystic Lesions of Bone
Other Tumors & Cancers
Bone Tumors: A Quick Reference Chart Phases in Cancer
Development Benign vs. Malignant
TUMORS OF BONE, BONE MARROW
AND CARTILAGE
Introduction
Diagnosis of bone tumors is not the singular effort of the clinician, but rather the
work of a team consisting of attending surgeon, radiologist, and pathologist
who will evaluate the history, provide a comprehensive examination, and order
appropriate tests. Differential diagnosis on x-ray is an interesting academic
excercise, but actual diagnosis is more important. Radiologic study has been
compared to physical examination of the lesion in its context. Laboratory
adjunctive testing will provide clues to some conditions. MRI and scans will help to
delineate tumors. Radionuclide uptake procedures can help to locate occult
metastatic or synchronous lesions. Occasionally, even angiography can be helpful.
Bone tumors are either primary or metastatic. Primary bone tumors can be either
benign or malignant. After a complete history and physical examination, and lab
studies, the management of bone tumors can be divided into 3 phases: (1)
radiographic staging, (2) diagnosis, and (3) treatment. The radiographic staging is
designed to provide information concerning the diagnosis and the anatomic
extent of the lesion. The definitive diagnosis always requires biopsy. Only after
these first 2 phases are complete can a rational treatment plan be established
and carried out.
In formulating a treatment plan, the. histogenetic type of tumor, local extent, and
possibility of distant disease must be considered. Clinical factors such as age, size,
occupation, life-style, and expectations also play a key role in the treatment
options available
Staging Techniques
The purpose of radiographic staging of a bone tumor is two-fold. The first is to
obtain information concerning a possible diagnosis, and the second is to define
the anatomical extent of the lesion
1. Staging techniques:
a. Plain radiograph demonstrates the bone involved, the region of the bone, the
extent and type of destruction, and the amount of reactive bone formed. It also
gives some information of benign vs malignant tumors (plain film x-rays are a
must in the staging of bone tumors)
b. Radioisotope scans (99m Tc MDP) can estimate the local intramedullary extent
of the tumor and screen for other skeletal areas of involvement. It can give
information about the biologic aggressiveness of the tumor via its uptake. This
study is also important in post-treatment followup
c. CT: Greatly aids in the diagnosis and anatomic location. Using contrast with CT aids
in the identification of the major neurovascular structures as well as the
enhancement of well-vascularized lesions. CT is the best study for evaluating cortical
penetration and osseous detail, and valuable in the assessment of matrix calcification
or ossification. CT is the best technique for detecting pulmonary metastasis,
replacing whole lung tomography.
d. MRI: The best information is obtained when MRI and CT are interpreted together. MRI more
accurately demonstrates the local intramedullary extent of tumors than with gallium-67 citrate
or any other method. MRI is the best technique for demarcating the soft tissue component of
bone tumor and its relationship to neurovascular structures without utilization of contrast
material
e. Arteriography: Since the advent of CT anti MRI with contrast, this technique is not used
much, except in a difficult anatomic location such as the shoulder girdle or pelvis
f. Biopsy:, The purpose of biopsy is to obtain adequate tissue for accurate diagnosis and grading
while avoiding potential detrimental effects to the patient or compromise of the definitive
surgical procedure
Surgical Staging System
The information generated by the history, physical examination, radiographic studies, and
biopsy has been incorporated into a staging system for musculosketetal tumors. The surgical
staging system reflects the biologic behavior of the lesion and its degree of aggressiveness. It is
based on a combination of the histologic grade (G), anatomic site (T), and the presence of
distant metastasis (M) 1. Staging system:
a. Grade (G): Divided into benign (GO), low grade malignant (G1), and high grade malignant
(G2). Grade is based upon a combination of histologic, radiographic, and cytologic
characteristics and biologic characteristics
b. Anatomic site (T): Is divided into intracapsular (TO),
extracapsular /intracompartmentaI (T1), and extracapsular/ extracompartmentaI
(T2)
c. The stages of malignant lesions are designated by numerals 1, II, and III
Radiology of Bone Tumors
1. Making a diagnosis: When attempting to diagnosis bone tumors it is important to know: the
age and sex of the patient, which bone is involved, is it well demarcated, its size and shape
and location, type of periosteal reaction, any soft tissue involvement, any pain and what
relieves the pain, pattern of bone destruction, appearance of the tumor matrix, appearance of
trabeculation, cortical erosion penetration or expansion, and a good medical history. a. Pattern
of destruction: geographic, motheaten or permeative
i. Geographic- usually seen with benign tumors, least aggressive pattern of bone destruction,
indicative of a slow growing lesion, has a smooth or irregular but well-defined margin which is
easily separated from the surrounding normal bone by a short zone of transition. Some
metastatics can look like this.
ii. Motheaten- may be demonstrated by a malignant bone tumor, OM, or . hematopoietic
tumors, indicative of rapidly growing lesion, less well-defined with a longer zone of transition
from normal to abnormal bone
iii. Permeative- demonstrated by certain malignant bone tumors and rarely OM, most aggressive
pattern of bone destruction, rapid growth potential, poorly demarcated and not easily
separated from the normal surrounding bone b. Size, shape and the margin of the tumor
1. Size- in general, malignant primary bone tumors are larger than benign ones and when first
discovered primary malignant tumors may be greater than 6 cm in size (in some cases)
ii. Shape- tumor with greatest diameter at least 1.5 times its least diameter may include Ewing's
sarcoma, chondrosarcoma and large cell lymphoma (reticulum cell sarcoma)
iii. Margin- sclerosis of the surrounding normal bone indicates a slow growing lesion, indicating
pressure with periosteal or endosteal reaction c. Tumor matrix: calcification or ossification
1. Matrix calcification- cartilage tumor, may appear centrally located, ring-like, or fleck-like
radiodensity, seen with chondromas, chondrosarcomas and chondroblastomas
ii. Matrix ossification- bone tumor, seen with osteoid osteoma and osteogenic sarcoma
d. Trabeculation:
i. Giant cell- delicate and thin
ii. Chondromyxoid fibroma- course and thick
iii. Aneurysmal bone cyst- delicate and horizontal iv. Non-ossifying fibroma- loculated
v. Hemangioma- striated and radiating
e. Cortical Erosion: Penetration and Expansion
The bone cortex serves as a barrier to the further lateral growth of "certain tumors such as non-
aggressive medullary lesions which generally expand in the direction of least resistance within
the medullary canal. Other lesions, however, may penetrate the cortex in variable lengths to
those aggressive bone lesions which may penetrate the entire thickness of the cortex in one or
more places
f. Type of periosteal response: Buttressing, Codman's triangle, sunburst, onion skin and hair-on-end
i. Buttressing (thick periostitis)- indicative of a slow growing tumor whereby an expansile mass
presses against the periosteum, which thickens the cortex with new layers of bone
ii. Codman's triangle- a triangular elevation of the periostium seen in osteogenic sarcoma
and other conditions (see Quick Reference Chart)
iii. Sunburst pattern- delicate rays of periosteal bone formation separated by spaces containing
blood vessels, seen with hemangioma, Ewing's sarcoma and osteogenic sarcoma
iv. Onion skin pattern- production of multiple layers of new periosteal bone,
exemplified by Ewing's sarcoma, eosinophilic granuloma, and lymphoma of bone
v. Hair-On-End pattern- similar to sunburst pattern, indicating the most malignant type of tumor,
where rays of periosteal bone project in a perpendicular direction to the underlying bone
(Ewing's sarcoma)
g. Part of the bone involved: Epiphyses, metaphyses or diaphysis
i. Epiphyses- chondroblastoma, giant cell tumor, subchondral cyst, and hemangioma
ii. Metaphyses- solitary bone cyst, osteogenic sarcoma, chondromyxoid fibroma, non-
ossifying fibroma, and chondrosarcoma
iii. Diaphysis- solitary bone cyst, aneurysmal bone cyst, non-ossifying fibroma, Ewing's sarcoma,
enchondroma, fibrous dysplasia, giant cell tumor, and eosinophilic granuloma
Bone Tumors
1. Osteoma (hyperostosis, periosteal osteoma):
a. Is a benign, reactive, expansile tumor-like lesion that may be pedunculated
b. It usually arises from intramembranous bones and may occur posttraumatically
c. Some may represent osteochondromas whose cartilage cap has ossified d. May occur
subungually
e. These may be:
i. Ivory: compact
ii. Spongy: trabecular
iii. Mixed
f. Multiple osteomas may occur in Gardner's Syndrome g. Excision is curative
2. Enostosis:
a. An uncommon, benign, intramedullary island of ossification
b. It may be reminiscent of osteoblastic metastatic visceral cancers c. These are often
asymptomatic, incidental radiologic findings
d. They may occur with Multiple Enchondromatosis and with keloid formation
3. Osteoid Osteoma:
a. Is a benign, osteoblastic, slow-growing lesion that may occur in cortical or medullary bone
b. The soft tissue center is its NIDUS
c. Some (but not all) patients experience nocturnal, intermittent pain that becomes a "boring"
type of constant pain
d. It many cases (but not all), pain is relieved by salicylates
e. X-rays often show a sclerotic rim of bone around the radiolucent nidus f. Sometimes the
nidus is sclerotic and not visable unless MRI or scans are done
g. Pain may be referred to adjacent joints
h. Lesions adjacent to joints may erode the cartilage
i. Surgery consists of en-bloc resection to include the nidus, (x-rays should be done of the
specimen removed to make sure the nidus was removed) j. Lesions usually are under 2 cm in
diameter, affecting patients in the 1025 year age group (mostly males)
4. Osteoblastoma (osteogenic fibroma, spindle cell variant of giant cell tumor, giant
osteoid osteoma):
a. Is a benign, fast-growing tumor that usually affects males (ages 10-20) b. Pain is
not relieved by salicylates in most cases c. Lesions are usually metaphyseal or diaphyseal
d. It usually exceeds 2 cm in diameter, and lacks the sclerotic rim around the soft tissue center
e. Older lesions may show a mottled pattern of thin calcifications
f. There is a debate as to whether these lesions can undergo malignant transformation
NOTE* Extraosseous osteogenic sarcoma is a well recognized entity. The rare juxtacortical type
may show a periosteal "string sign" on x-rays
6. Osteochondroma (osteocartilaginous exostosis): a. Is the most common benign bone tumor
b. The most frequent form in the foot is the subungual (Dupuytren's) exostosis
c. Multiple forms exist and are known as multiple cartilaginous exostoses, hereditary multiple
exostosis, hereditary deforming dyschondroplasia, and diaphyseal aclasis
d. While the classic type has a hyaline cartilage cap, the subungual types have fibrocartilage
caps
e. Excision is curative if the overlying soft tissue which acts as perichondrium (and therefore is a
source of recurrence) is removed with the lesion
f. Malignant transformation is rare, but is recorded
7. Chondromas:
a. Are benign neoplasms
b. Solitary enchondromas appear as intramedullary (central) lucencies derived from rests of
epiphyseal cartilage
c. They may become symptomatic after local trauma, usually in adults between 30 & 40 years of
age
d. Pain without history of local trauma suggests malignant transformation e. Multiple
enchrondatosis (Ollier's disease) is a cartilage dysplasia that can be quite deforming, and carries
a high risk for malignant transformation f. Multiple enchrondromatosis occur as part of
Maffuci's (Kast-Maffuci) syndrome (angiochondromatosis). Persons with this syndrome may
show areas of vitiligo, as well as pigmented lesions
g. Metachondromatosis is a dominantly inherited mime of multiple enchondromatosis, but the
lesions regress
h. Periosteal (juxtacortical/eccentric) chondroma is a rare lesion and is also called ecchondroma
i. The lesions are sharply circumscribed on x-ray j. Size and location determine surgical approach
9. Chondromyxoid Fibroma:
a. In general, is a rare tumor, but does affect the feet
b. Most frequently seen between the ages of 10 & 20 years
c. Foot lesions seem to be more frequent in the 30-40 age group
d. These are painful lesions that are lucent on x-rays, and which may appear lobular or bubbly
e. Some lesions are quite aggressive
f. En-bloc resection or amputation may be necessary
10. Chondrosarcoma:
a. Is a malignant, potentially metastatic cartilage tumor that may show myxoid and/or osseous
elements
b. It is the third most common malignant bone tumor c. Most frequently seen between the
ages of 40 & 60
d. Lesions may be primary or secondary, the latter arising in such preexisting lesions as Paget's
disease of bone, osteochondroma (single or multiple), Maffuci's (Kast-Maffuci) syndrome, Ollier's
multiple enchondromatosis, and bone cysts
e. The types range from the well differentiated through the clear cell and mesenchymal
(anaplastic) types
f. Patients demonstrate a painful limp and a mass may be palpable
g. On x-ray, the tumors may be lucent or may demonstrate calcifications h. Angiograms, scans,
and MRI are helpful adjuncts i. Amputation is the indicated treatment,. the level depending upon
the location and size of the tumor
NOTE* Secondary malignant bone tumors are metastases from visceral carcinomas or other
soft tissue sarcomas. These intraosseous malignancies cause pain and bone destruction,
appearing irregular on x-ray
17. Soft Tissue Benign Tumors:
a. May also arise as primary bone tumors
b. These include hemangiomas (solitary or multiple), lymphangiomas, glomus tumors, and giant cell tumors
of bone
c. Tumor-like lesions affecting bone include penetrating epidermal inclusion cysts, pseudoepitheliomatous
hyperplasia in fistulas leading from bone, hypertrophic pulmonary osteoarthropathy, and giant cell
reparative granuloma of the digits (considered by some to be a variant of giant cell
tumor of bone)
Cystic Lesions of Bone
These have to be differentiated from bone tumors 1. Unicameral (Simple) Bone Cyst:
a. Generally are asymptomatic lytic lesions, that are thought to be interosseous synovial cysts as a result of
entrapment if synovium during bone development
b. Classically, these have been treated by curettge and packing. Curettings are very sparse. Cryosurgery is
risky due to possible damage to nearby epiphyses
c. Recently, treatment success has been reported with one or more aspirations of fluid followed by
introduction of a steroid d. Most cases arise in males over 20 years old e. Those in the calcaneus arise in
Ravelli's triangle
f. Pain may suggest a fracture of the wall, and a "fallen fragment sign" represents a fracture chip within the
cyst
2. Intraosseous Ganglion:
a. May give a similar x-ray appearance as a unicameral cyst b. May be painful
c.. A synovial cell lining is seen in the excised tissue (which is not seen in the curettings from a simple bone
cyst)
NOTE' Of course, even with such a history, a suspect lesion may not be a metastasis, it may be
an independent and entirely coincidental lesion
Bone Tumors: A Quick Reference Chart
1. Most Common Malignant Primary Bone Tumors: #1 multiple myeloma
#2 osteogenic sarcoma
#3 chondrosarcoma
#4 Ewing's sarcoma
NOTE The most common bone tumor is metastatic tumors from other sites, but this group
does not constitute primary malignancy in bone
Ill. "Onionskin" Periosteal Reactivity Can Be Seen In: #1 osteomyelitis (especially Garre sclerosing
type #2 eosinophilic granuloma
#3 Ewing's sarcoma
#4, lymphoma of bone
Vl. "Paget's" sarcomas that arise in bone in the setting of Paget's disease of bone:
#1 osteogenic sarcoma
#2 chondrosarcoma
#3 fibrosarcoma
#4 malignant fibrous histiocytoma
NOTE* They may also arise in bone independent of Paget's disease of bone
V11. "Tempo Phenomenon" Is Associated With:
#1 aneurysmal bone cyst: (rapid growth=quick tempo phenomenon) #2 eosinophilic
(Langerhans cell) granuloma: (interosseous lesions may appear and disappear without
treatment=tempo phenomenon)