This document provides information on various types of malignant bone tumors. It classifies tumors based on the WHO classification and discusses bone-forming tumors, cartilage-forming tumors, giant cell tumor, marrow tumors, vascular tumors, and other connective tissue tumors. It provides details on osteosarcoma, Ewing's sarcoma, chondrosarcoma, and primary lymphoma of bone including areas of involvement, clinical presentation, radiological features, diagnosis, and treatment options.

1. Malignant bone
Tumors
DR HARSHWARDHAN DAWAR
MGM MEDICAL COLLEGE AND MY HOSPITAL, INDORE

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

4. Cartilage forming tumours
Benign
 Chondroma
Enchondroma
 Osteochondroma
 Chondroblastoma
 Chondromyxoid fibroma
Malignant
 Chondrosarcoma
 Differentiated
chondrosarcoma
 Juxtacortical
chondrosarcoma
 Mesenchymal
chondrosarcoma
 Clear cell chondrosarcoma

5. Giant cell tumour
 Osteoclastoma

6. Marrow tumours
 Ewing’s sarcoma
 Neuroectodermal tumour
 Malignant lymphoma of bone (Primary/secondary)
 Myeloma

7. Vascular tumours
Benign
 Haemangioma
 Lymphangioma
 Glomus tumour
Intermediate
 Haemangio endothelioma
 Haemangio pericytoma
Malignant
 Angiosarcoma
 Malignant haemangio pericytoma

8. Other connective tissue tumours
Benign
 Benign fibrous
histiocytoma
 Lipoma
Intermediate
 Desmoplastic fibroma
Malignant
 Fibrosarcoma
 Malignant fibrous
histiocytoma
 Liposarcoma
 Malignant mesenchymoma
 Leiomyosarcoma
 Undifferentiated sarcoma

9. Other tumours
Benign
 Neurilemmoma
 Neurofibroma
Malignant
 Chordoma
 Adamantinoma

10. Secondary malignant tumours of bone
From primary in:
 Thyroid
 Breast
 Bronchus
 Kidney
 Prostate

11. Staging of the tumor
 By Enneking (1986)
 Based on aggressiveness of the tumor and Spread

13. Correlation of staging and management
 I-A - Wide excision
 I-B - Wide excision with larger clearance
 II-A - Wide excision/amputation
 II-B - Radical resection or disarticulation
 III - Palliative treatment
 Low grade intra compartmental lesions – wide
resection and management of metastases

14. OSTEOSARCOMA

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

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

21. Plain X-ray (Most valuable)
 sclerotic Lytic
21
Mixed (most common)

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

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

28. EWING’S
SARCOMA

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

31. Areas of
involvement

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

34. Radiological
Features

37. Onion skin appearance

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

40. Nuclear medicine:
 Ewing sarcomas demonstrate increased uptake on both
Gallium-citrate and
 Technetium99m methylene diphosphonate scans

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

43. Treatment Ewings
 Radiosensitive
 Chemosensitive

44. CHONDROSARCOMA

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

47. TYPES:
TYPES
 Primary chondrosarcoma
 Secondary chondrosarcoma
 Periosteal chondrosarcoma
 Dedifferentiated chonrosarcoma
 Clear cell chondrosarcoma
 Mesnchymal chondrosarcoma

48. RADIOLOGICALLY
 Frequently diagnostic
 Arising in medullary cavity with irregular matrix calcification- punctate , popcorn, comma shaped
calcification( rings and arcs appearance)
 Bone destruction, cortical erosions, periosteal reaction, soft tissue mass

49. Chondrosarcoma from iliac bone with soft
tissue extention

51. POP CORN CALCIFICATION

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

53. Intramedullary ,soft
tiisue extention of tumour
with calcification

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

55. Intramedullary
extention & soft
tissue extention

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.

59. Areas of involvement

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

65. Geographic moth eaten appearance

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.

72. MYELOMA

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

74.  Multiple myeloma
may present in a
variety of
radiographic patterns

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.

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)

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

86. Differential diagnosis
 GCT
 Telangiectatic osteosarcoma

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

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

100. Spotters

102. Ewing’s sarcoma preop, post chemo, post
op

106. Thalassemia

108. Admantinoma

110. Chronic osteomyelitis  sq cell Ca

112. Bone infarct  sarcoma

114. THANK YOU