COVER S THE MARROW TUMORS AND VASCULAR TUMORS AND SARCOMAS OF BONE.. OMITTED OSTEOSARCOMA AND OTHER COMMONLY DISCUSSED MALIGNANCIES .

1. Malignant bone Tumors
Dr Arif khan S
AJIMS

2. WHO CLASSIFICATION
• BONE FORMING – osteosarcoma;
• CARTILAGE FORMING - chondrosarcomas
• GIANT CELL TUMORS
• MARROW TUMORS – Ewing’s ; lymphomas; multiple myeloma
• VASCULAR & other connective tissue TUMORS – Angiosarcoma, ; Fibro
sarcoma, Malignant Fibrous Histio cytoma
• MISCELLANEOUS – Chordoma ; adamantinoma; neurosarcoma
Radiation induced sarcoma

3. Ewing’s Sarcoma
• Ewing sarcoma, a highly malignant neoplasm
• predominantly affecting children and adolescents
• 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

4. Areas of involvement
Ewing sarcoma has a predilection for the
diaphysis of the long bones
ribs
flat bones such as the scapula and pelvis

5. 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.

6. 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,
• and a large soft-tissue mass .
• Occasionally, the bone lesion itself is almost imperceptible, with the soft-tissue
mass being the only prominent radiographic finding

7. • Other, less common, manifestations
Thickened cortex or Saucerication
Expansion of bone
Pathologic fractures

8. permeative bone destruction associated with
an aggressive periosteal reaction
CT section through the lesion demonstrates
a large soft-tissue mass, which is not clear on
the conventional study.
obliteration of the marrow cavity by tumor.

9. saucerisation

10. Bone destruction is almost
imperceptible
shows a large soft-tissue mass

11. Onion skin appearance

12. 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

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

14. 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

15. 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
• The vast majority of cases are of the non-Hodgkin type, with Hodgkin disease
accounting for 6% of cases
• Distinguishing primary bone lymphoma from other bone tumors is important because
the former has a better response to therapy and a better prognosis.

16. • Histologically, lymphomas may be subdivided into non-Hodgkin lymphomas
and 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.

17. Areas of involvement
long bones
vertebrae
Pelvis
ribs

18. 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

19. Radiological features
• Coley’s:--lymphoma presenting in an osseous site with no evidence of
disease elsewhere for at least 6 months after diagnosis.
• 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 ()

20. MRI findings
• T1-weighted pulse sequences are the best (compared with
other sequences) for demonstrating marrow changes, as T1-
weighted images reveal areas of low signal intensity within
the marrow
• 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)_

21. 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

22. • 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

23. Geographic moth eaten appearance

24. • Cortical breakthrough, pathologic
fractures
• soft-tissue masses represent a more
aggressive pattern of involvement
and a poorer prognosis
• Cortical breakthrough, which refers
to a sudden interruption in the
continuity of the cortex, was better
seen at computed tomography (CT)
in our experience , correlated with a
more aggressive tumor. CT also
demonstrated sequestra ,findings
that have been reported in osseous
lymphomas

25. • 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 (also called
onion-peel appearance), or broken, when
discontinuous or interrupted periosteal
new bone is seen.
• The latter appearance of disrupted
periosteal bone is believed to be a helpful
radiographic sign that indicates a poorer
prognosis

26. 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

27. • 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 in
Hodgkin disease, lytic lesions predominate).
• Sclerotic areas can, however, develop in an
originally lytic pattern after therapy (irradiation
and chemotherapy

28. • 3.Subtle or “Near-Normal” Findings
• A third pattern seen and described in
primary 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.

30. Myeloma
• Aka multiple myeloma” or “plasma cell myeloma,”
• 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

31. • Multiple myeloma
may present in a
variety of
radiographic patterns

32. • 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.

33. • 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

34. • 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.

36. • 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

37. 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)

39. 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

40. • 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.

41. Radiological 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

43. Differential diagnosis
• GCT
• Telangiectatic osteosarcoma

44. 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

45. • 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

47. 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.

48. • 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

49. • MRI findings
MRI reveals a mixed signal
on T1-weighted sequences,
with moderately increased
signal intensity on T2
weighting

50. 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.

51. • 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.

52. 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

53. • 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

54. 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

55. 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

57. Spotters

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

63. Thalassemia

65. Admantinoma

67. Chronic osteomyelitis  sq cell Ca

69. Bone infarct  sarcoma

71. THANK YOU