2. Classification (W.H.O.)
o Bone-forming tumours
o Cartilage forming tumours
o Giant-cell tumour
o Marrow tumours
o Vascular tumours
o Other connective tissue tumours
o Other tumours
o Secondary malignant tumours of bone
3. Bone forming tumours
Benign
Osteoma
Osteoid osteoma or
osteoblastoma
Intermediate
Aggressive
osteoblastoma
Malignant
Osteosarcoma
Central (Medullary)
Peripheral (Surface)
Parosteal
Periosteal
High grade surface
15. OSTEOSARCOMA
o Characterized by the production of osteoid by malignant cells.
o It is the second most common primary malignant tumor of bone, accounting for approximately
20% of primary bone cancers.
o The most common nonhematologic primary malignancy of bone.
o Onset can occur at any age; however, primary high-grade osteosarcoma occurs most commonly
in the second decade of life.
o Parosteal osteosarcoma has a peak incidence in the third and fourth decades.
o Arise from multipotent mesenchymal cells
16.
17.
18. Classification:
SECONDARY OSTEOSARCOMAS
Osteosarcomas occurring at the site of another disease process.
more common in >50 years of age
The most common causes are
Paget disease
Previous radiation treatment
Other associated conditions are
o Fibrous dysplasia
o Bone infarcts
o Osteochondromas
o Chronic osteomyelitis
o Dedifferentiated chondrosarcomas
o Osteogenesis imperfecta
19. Clinically
Pain– progresssive pain
due to microinfarction
night pain in 25 %
Swelling - Palpable mass is noted in up to 1/3
of patients at the first visit
Fever, malaise or other constitutional symptoms
are not typical of osteosarcoma
20. Clinically
Radiographic appearance of osteosarcoma can vary
lesion can be either predominantly blastic or predominantly lytic
The lesion usually is quite permeative, and the borders are ill defined.
May take the form of a “Codman triangle,” or it may have a “sunburst” or “hair-on-end”
appearance.
Magnetic resonance imaging (MRI)
They may be primarily osteoblastic, fibroblastic, or chondroblastic
22. Plain X-ray
Lesions are usually permeative
Associated with destruction of the cancellous and
cortical elements of the bone
Ossification within the soft tissue component, if
tumour has broken through cortex
Intra medullary
Borders are ill defined
22
23. Plain X-ray
Periosteal reaction may appear as the characteristic Codman triangle
Extension of the tumor through the periosteum may result in a so-
called “sunburst” or “hair on end” appearance.
23
24.
25.
26. PRIMARY OSTEOSARCOMAS are
Conventional /classic osteosarcoma (high grade, intra medullary)
Low-grade intramedullary osteosarcoma
Parosteal osteosarcoma
Periosteal osteosarcoma
High-grade surface osteosarcoma
Telangiectatic osteosarcoma, and
Small cell osteosarcoma.
Classification:
27. Treatment
Current standard of care
Radiological staging
Biopsy to confirm diagnosis
Preoperative chemotherapy
Repeat radiological staging (access chemo response, finalize surgical treatment plan)
Surgical resection with wide margin
Reconstruction using one of many
techniques
Post op chemo based on preop response
27
29. Ewing’s Sarcoma
Ewing sarcoma, a highly malignant neoplasm
Third most common nonhematologic primary malignancy of bone
The second most common in patients younger than 30 years of age and the
most common in patients younger than 10 years of age
30. Ewing’s sarcoma
Approximately 90% of Ewing sarcomas occur before age 25
Histogenesis: neurally derived small round cell malignancy very similar to the
so-called primitive neuroectodermal tumor (PNET)
Disease is extremely rare in black persons
32. Clinical presentation
Present as a localized painful mass
With systemic symptoms such as fever, malaise, weight loss, and
An increased erythrocyte sedimentation rate.
These systemic symptoms may lead to an erroneous diagnosis of osteomyelitis.
33. Radiological features
lesion is poorly defined,
Marked by a permeative or moth-eaten type of bone destruction,
Associated with an aggressive periosteal response that has an onionskin (or
“onion peel”)
less commonly, a “sunburst” appearance,
Large soft tissue mass, Occasionally, the bone lesion itself is almost
imperceptible, with the soft-tissue mass being the only prominent radiographic
finding
38. MRI
1. T1 : low to
intermediate signal
2. T1 C+ (Gd) :
heterogeneous but
prominent
enhancement
3. T2 : heterogeneously
high signal, may see
hair on end low signal
striations
39.
40. Nuclear medicine:
Ewing sarcomas demonstrate increased uptake on both
Gallium-citrate and
Technetium99m methylene diphosphonate scans
41.
42. Differential diagnosis
other Ewing sarcoma family of tumours
pPNET : large soft tissue component with extension into bone
Askin tumour : chest wall
osteosarcoma (ALP is not elevated in Ewing sarcoma)
Leukemia
Multiple myeloma
Ostyeomyelitis
45. Malignant tumour of cartilage producing cells
Chondrosarcoma is the second most common primary bone malignancy
(after osteosarcoma).
3 rd most common primary malignancy of bone after multiple myeloma and osteosarcoma
2 nd most common non hematological primary malignancy of bone
52. DIFFERENCE FROM ENCHONDROMA
o Endosteal scalloping of more than 2/3rd of cortical thickness- chondrosarcoma
o Aggressive changes such as cortical erosion , bone destruction, periosteal reaction, soft
tissue mass- chondrosarcoma
o size> 5 cm in axial skeleton- predictor of malignancy
o Apperance of lysis in previously calcified area- malignancy
54. MRI
Depict high water content of lesion with lobulation at margins
Best to see intramedullary extent
Cotical erosion, bone destruction, reactionary edema, soft tissue extention
are well depicted
56. TREATMENT:
Wide or radical resection or amputation
Chemotherapy
No role
Currently evaluated for treatment of mesenchymal and dediffrentiated
chondrosarcoma
Radiotherapy
Limited role
For palliative purpose
Proton beam therapy(recent advance)
Used for skull base chondrosarcoma
57. Primary lymphoma of bone
Rare malignant condition that accounts for less than 5% of all primary bone
tumors
It occurs in the second to seventh decades, with a peak age of occurrence from
45 to 75 years. M>F
It has also been called reticulum cell sarcoma, malignant lymphoma of the bone,
and more recently osteolymphoma.
Bone lymphoma is now known as large cell or histiocytic lymphoma
58. Histologically, lymphomas subdivided :+ non-Hodgkin lymphomas
+Hodgkin lymphomas
Primary Hodgkin bone lymphoma is extremely rare
Non-Hodgkin bone lymphomas are considered primary only if a complete systemic work-up reveals
no evidence of extraosseous involvement.
The tumor consists of aggregates of malignant lymphoid cells replacing marrow spaces and
osseous trabeculae. The cells contain irregular or even cleaved nuclei.
60. Clinical features
Insidious and intermittent bone pain that can persist for months.
Other signs and symptoms include local swelling, a palpable mass, and
systemic symptoms such as weight loss and fever
Vertebral involvement can cause radicular symptoms and can even lead
to compression of the spinal cord
61. Radiological features
wide spectrum of findings—from a near-normal-appearing bone to a focal lytic lesion with
geographic margins to a mixed sclerotic-lytic lesion to a diffusely permeative process with
cortical destruction and soft-tissue
a solitary lytic lesion near the end of a long bone that has a permeative or moth-eaten pattern
of destruction and aggressive periosteal reaction ()
62. MRI findings
T1-the best
T2-weighted images these areas generally appear bright
Peritumoral oedema and reactive marrow change can also
produce high signal intensity on T2-weighted images.
Post contrast study shows enhancement.
Better picturization of soft tissue involvement and Cortical
erosion (better than CT)
63. Patterns of bone changes
1. Lytic-Destructive Pattern
The lytic-destructive pattern is the most common radiographic
appearance of primary bone lymphoma,
It is believed to result from an osteoclast-stimulating factor.
The lytic pattern may be permeative—characterized by
numerous small, elongated rarefactions that are parallel to the
long axis of the bone and relatively uniform in size
64. Occasionally, the lesion may manifest with focal lytic areas
with well-defined margins
moth-eaten—a pattern of many medium to large areas of
radiolucency in a poorly marginated area of bone
66. Cortical breakthrough, pathologic
fractures
soft-tissue masses represent a more
aggressive pattern of involvement
and a poorer prognosis
Cortical breakthrough, sudden
interruption in the continuity of the
cortex, was better seen at computed ,
correlated with a more aggressive
tumor. CT also demonstrated
sequestra ,findings that have been
reported in osseous lymphomas
67. Periosteal reaction has been reported in
about 60% of cases and may be either
lamellated or layered,
Layers of periosteal bone are seen parallel
to the long axis of the bone, or broken,
when discontinuous or interrupted
periosteal new bone is seen.
The appearance of disrupted periosteal bone
is believed to be a helpful radiographic sign
that indicates a poorer prognosis
68. Myelogram shows that the disk is
normal, but the body of L-5 exhibits a
mottled appearance and its posterior
border is indistinct
CT section demonstrates a large,
osteolytic lesion extending from the
anterior to the posterior margins of
the vertebral body. Biopsy revealed a
histiocytic lymphoma
69. 2. Blastic-Sclerotic Pattern
Primarily blastic-sclerotic lesions are rare in
primary bone lymphoma compared with
metastatic bone lymphoma.
A mixed lytic lesion with sclerotic areas can be
seen
Sclerotic changes in primary bone lymphoma
may seem scarce, since of the two types of
lymphoma, it is Hodgkin disease of bone (the
less common subtype of primary bone
lymphoma) that tends to be sclerotic and even
Hodgkin disease, lytic lesions predominate).
Sclerotic areas can, however, develop in an
originally lytic pattern after therapy (irradiation
and chemotherapy
70. 3.Subtle or “Near-Normal” Findings
A third pattern seen and described in
bone lymphoma is the near absence of
detectable abnormalities on plain
radiographs
Cases with remarkably normal-appearing
radiographs may show striking abnormalities
on radionuclide bone scans and MR images.
As a result, in patients with symptoms but
negative radiographic findings, further
assessment with a second, more sensitive
modality such as scintigraphy or MR imaging
is essential.
73. Myeloma
Aka multiple myeloma” or “plasma cell myeloma,”
Aka Kahler’s disease
the most common primary malignant bone tumour.
It is usually seen between the fifth and seventh decades. M>F
Histologically, the diagnosis is made by finding sheets of atypical plasmacytoid cells replacing the
normal marrow spaces.
The plasma cell is recognized by the presence of eccentrically situated nucleus within a large
amount of cytoplasm that stains either light blue or pink. The neoplastic cells contain double or
even multiple nuclei, usually hyperchromatic and enlarged, with prominent nucleoli
75. Multiple myeloma may present in a
variety of radiographic patterns
Particularly in the spine,
It may be seen only as diffuse
osteoporosis with no clearly
identifiable lesion; multiple
compression fractures of the
vertebral bodies may also be
evident.
76. More commonly, it exhibits
multiple lytic lesions scattered
throughout the skeleton. In
the skull, characteristic
“punched-out” areas of bone
destruction, usually of uniform
size, are noted
Usually part of Myelomatosis
77. The ribs may contain lace-like areas of
bone destruction and small osteolytic
lesions, sometimes accompanied by
adjacent soft-tissue masses, which often
referred to as PLASMACYTOMAS
Areas of medullary bone destruction are
noted in the flat and long bones,
if these appear about the cortex, they are
accompanied by scalloping of the inner
cortical margin
Fewer than 1% of myelomas may be of a
sclerosing type called sclerosing
myelomatosis.
78.
79. POEMS syndrome, first described in y.
It consists of polyneuropathy (P), organomegaly (O), particularly of the liver and
the spleen, endocrine disturbances (E) such as amenorrhea and gynecomastia,
monoclonal gammopathy (M), and skin changes (S) such as hyperpigmentation
and hirsutism,
INCIDENCE OF POLYNEUROPATHY
3% in osteomyelitic
30-50% sclerotic
80. Differential Diagnosis
Metastatic carcinoma of spine: based on Involvement of pedicles
Metastatic carcinoma of other parts : cranium “punched out lesions” or
sharply defined multiple lytic lesions in skull.
However in later stages Bone scintigraphy alone can distinguish when
involvement is diffuse.
Solitary myeloma : hyperparathyroidism, giant cell tumor, fibrosarcoma.
Solitary mets (?from where)
81.
82. Fibrosarcoma & MFH
Fibrosarcoma and Malignant Fibrous Histiocytoma are malignant fibrogenic tumors that
have very similar radiographic presentations and histologic patterns
Both typically occur in the third to sixth decades,
both have a predilection for the pelvis, femur, humerus, and tibia.
Both fibrosarcoma and MFH can be either primary tumors or secondary to a preexisting
benign condition, such as Paget disease, fibrous dysplasia, bone infarct, or chronic draining
sinuses of osteomyelitis
83. These lesions may also arise in bones that were previously irradiated. Such
lesions are termed secondary fibrosarcomas (or secondary malignant fibrous
histiocytomas). Also radiation induced fibrosarcoma or sarcoma
Rarely, fibrosarcoma can arise in a periosteal location (periosteal fibrosarcoma.
Histologically, fibrosarcoma and MFH are characterized by tumor cells that
produce collagen fibers. Neither tumor is capable of producing osteoid matrix or
bone, a factor distinguishing them from osteosarcoma.
84. logical features
fibrosarcoma and MFH are recognized by
an osteolytic area of bone destruction and
a wide zone of transition.
the lesions are usually eccentrically
located close to or in the articular end of
the bone.
They exhibit little or no reactive sclerosis
and in most cases no periosteal reaction.
Soft tissue mass is present
87. Primary Leiomyosarcoma of Bone
Very rare, with fewer than 100 cases reported in the world literature.
More common are skeletal metastases from primary soft-tissue leiomyosarcoma.
(an extraosseous primary tumor, mainly from the gastrointestinal tract or uterus, must be ruled
out before a confident diagnosis of primary leiomyosarcoma of bone can be made)
predominantly spindle-cell, neoplasm that exhibits smooth muscle differentiation.
Although the patients reported range from 9 to 80 years of age, occurrence before age 20
is uncommon
88. The usual clinical presentation is pain of variable intensity and duration. A soft-tissue mass is
occasionally observed
The most common sites are the distal femur, proximal tibia, proximal humerus, and iliac bone
clavicle, ribs, and mandible occasionally may be affected .
the tumor most often presents either as a lytic area of geographic bone destruction (moth
eaten appearance is common)
Approximately 50% of reported lesions exhibit fine periosteal reaction
On MR imaging, the lesions are isointense to muscle on T1-weighted sequences, whereas on
T2 weighting they exhibit a heterogeneous signal
89.
90. Hemangioendothelioma
Hemangioendothelioma and a recently identified lesion called epithelioid
hemangioendothelioma are considered to represent true neoplasms because of
their independent growth potential.
they commonly recur after inadequate local excision
histopathology demonstrates of nuclear atypia accompanied by occasional mitotic
activity.
They can arise at any age within the range of 10 to 75 years, with a slight predilection
for males.
91. The most commonly affected sites are the calvaria, spine, and bones of the lower extremities.
Clinical symptoms include dull local pain and tenderness.
Some swelling and hemorrhagic joint effusion seen some cases
Radiologically
osteolytic appearance, either well circumscribed or with a wide zone of transition.
Variable degrees of peripheral sclerosis may sharply demarcate the lesion.
Occasionally a soap-bubble appearance with expansion of bone is observed, with extension into
the soft tissues
92. MRI findings
MRI reveals a mixed signal
on T1-weighted sequences,
with moderately increased
signal intensity on T2
weighting
93. Angiosarcoma
Angiosarcoma of bone represents the most malignant end of the spectrum of
vascular tumors.
aggressive neoplasm, characterized by frequent local recurrence and distant
metastases.
typically during the second to the seventh decade (with a peak in the fifth
decade). M>2F
Metastases to the lungs and other internal organs occur in approximately 66%
of cases.
94. Most common sites of occurrence are the long
bones, particularly the tibia, femur, and
humerus,
most common symptoms are local pain and
swelling.
Angiosarcoma has imaging features similar to
those of hemangioendothelioma.
Microscopically, angiosarcoma is composed of
poorly formed blood vessels that exhibit
complicated infoldings and irregular
anastomoses.
The endothelial cells that line these blood
vessels display features of frank malignancy,
with plump intraluminal cells showing nuclear
hyperchromation and atypical mitoses.
95. Chordoma
Tumor arising from developmental remnants of the notochord.
Represent from 1% to 4% of all primary malignant bone tumor
They arise between the fourth and seventh decades and affect men slightly more
often than women.
The three most common sites for a chordoma are the sacrococcygeal area, the
spheno-occipital area, and the C-2 vertebra.
Histologically, the tumor consists of loose aggregates of mucoid material
separating cord-like arrays and lobules of large polyhedral cells, along with
vacuolated cytoplasm and vesicular nuclei referred to as physaliphorous cells
96. Radiologically
a highly destructive lesion with irregular
scalloped borders;
it is sometimes accompanied by
calcifications in the matrix, probably as a
result of extensive tumor necrosis
Bone sclerosis has been reported in 64%
of cases. Soft-tissue masses are
commonly associated with the lesion
CT or MRI is required to demonstrate
soft-tissue extension and invasion of the
spinal canal
97. Lesions with malignancy potential
Medullary Bone Infarct sarcoma
bone destruction in the area of the medullary infarct with periosteal reaction and
soft-tissue mass confirm the diagnosis of malignant transformation
Chronic Draining Sinus Tract of Osteomyelitis squamous cell carcinoma
Sinus >20yrs duration.
Plexiform Neurofibromatosis neurosarcoma, neurofibrosarcoma
Paget’s Disease sarcomas
lytic lesion, often with evidence of cortical breakthrough and a soft-tissue mass
98. Radiation-Induced Sarcoma
normal bone exposed to radiation fields
may be caused by benign conditions treated by irradiation,
fibrous dysplasia or giant cell tumor
a sarcoma can develop only if at least 3,000 rads are administered within a
4-week span
