1. Bone tumors II
MODERATOR- DR. S. S. THAKUR

2. GIANT CELL TUMOUR
• Epiphyseal mesenchymal neoplasm of low
malignant potential composed of stromal cells
and giant cells .
• Age/Sex: >20 yr/ F>M.
• Bone involved: lower end of femur & radius
and upper end of tibia.
• Location: Epiphysis

3. X -Ray
Typical radiograph of giant cell tumor of distal end of femur involving epiphysis and
metaphyseal area.

4. Gross
A, Gross appearance of giant cell tumor of lower end of femur. The lesion is
characteristically peripheral, expansile, well circumscribed, and hemorrhagic.
B, Giant cell tumor of lower end of femur. The lesion, which has a very hemorrhagic
quality, has destroyed the cortex and extended into the adjacent soft tissues

5. Microscopic
Giant cell tumor.-The nuclei of the giant cells are similar to those of the mononuclear cells

6. Tissue section
Cytology

7. Acid phosphatase

8. IHC
• lysozyme
• α-1-antitrypsin
• α-1-antichymotrypsin

9. Differential diagnosis
•
•
•
•
•
•
•
•
•
•
•
•
Metaphyseal fibrous defect
Nonossifying fibroma
Chondromyxoid fibroma
Chondroblastoma
Langerhans’ cell histiocytosis
Solitary bone cyst
Osteitis fibrosa cystica of hyperparathyroidism
Aneurysmal bone cyst
Osteoid osteoma
Osteoblastoma
osteosarcoma
Giant cell tumor of tendon sheath

10. Malignant GCT
• Clinical, topographic & general microscopic
features of GCT but exhibits clear cut evidence
of malignancy in the mononuclear stromal
component.

11. MARROW TUMORS
1- EWING’S SARCOMA/PRIMITIVE
NEUROECTODERMAL TUMOUR
• Malignant undifferentiated sarcoma of bone
in children. Closely related to primitive
neuroectodermal tumor of soft tissues.
• Age/Sex: 5-20 yrs/M>F.
• Bones involved: Long bones, pelvis, ribs,
vertebrae.
• Location: Medullary cavity.
• Askin tm

12. X-Ray
.

13. Gross
Gross appearance of Ewing sarcoma. It has a typical ill-defined quality, with
extensive involvement of medulla and cortex associated with elevation of
periosteum

14. Microscopic
Diffuse pattern of growth and monotonous cytologic appearance in
Ewing sarcoma/PNET.

15. PAS stain

16. CD 99

17. Differential Diagnosis
• Malignant Lymphoma and Related Lesions.
• Desmoplastic small cell tumour.
• Embryonal/alveolar rhabdomyosarcoma.
• Neuroblastoma
• Medulloblastoma
• Retinoblastoma
• Wilms tumor
• Osteosarcoma
• Metastatic Tumors [Bone]

18. IHC
• LMWK
• Neuron specific enolase
• Leu 7

19. Prognosis
•
•
•
•
•
•
•
•
•
Osseous versus extraosseous location
Direct soft tissue extension
Metastasis
Surgical margins
Therapy-induced necrosis
Microscopic features
Neural differentiation
p53
c-myc amplification.

20. Malignant lymphoma
• Primary lymphomas of bone occur mostly in
adults and are of large B-cell type. Any
systemic lymphoma or leukemia can involve
the skeletal system secondarily.

21. Large Cell Lymphoma
•
•
•
•
Age/Sex:>30 yrs/ M:F=1:1
Location: diaphysis or metaphysis
Bone involved: long bone or vertebrae
Gross :pinkish gray and granular tumour
producing patchy cortical and medullary
destruction with minimal to moderate
periosteal reaction

22. Malignant lymphoma involving lower end of femur associated with bone
destruction and bone production

23. Microscopic
IHC
CD 45 +
Bcl 2
Malignant lymphoma of bone. The
tumor is of large cell type and is
associated with some fibrosis

24. VASCULAR TM
Hemangiomas
• Benign neoplasm of blood vessels.
• Bones involved: vertebrae, skull and jaw
bones.
• Multiple hemangiomas are mainly seen in
children and young adults.
• Gross
– Often current jelly appearance

25. Microscoipc

26. Differential Diagnosis
• Angiosarcoma [Bone]
• Epithelioid Hemangioendothelioma.
• Aneurysmal Bone Cyst

27. Epithelioid hemangioendothelioma
• Most common epithelioid vascular neoplasm
of the bone.
• Borderline type of vascular neoplasm.
• Site: unique vascular tumour occuring around
medium and large sized veins in the soft tissue
of adults.

28. X-Ray
Multicentric hemangioendothelioma involving multiple
bones of the foot

29. Microscopic

30. Factor VIII

31. Angiosarcoma
• Malignant vascular neoplasm.
• Age/sex: older adults/M:F=1:1.
• Location: anywhere in body.

32. Microscopic
Angiosarcoma of bone. Anastomosing vascular channels lined by
highly atypical endothelial cells are seen

33. Factor VIII

34. Hemangiopericytoma
• It is extremely
uncommon to find
hemangiopericytoma as
a primary bone tumor
• Can present as primary
bone lesion.
• Age – 2nd-9th decade
Sex- M:F- 1:1
• Most common sitepelvis

35. Fibrous Tumours
• Benign: Fibrous Cortical
Defect, Non-Ossifying
Fibroma.
• Benign Aggressive:,
Fibrous Dysplasia.
• Malignant: Malignant
Fibrous Histiocytoma of
bone, Fibrosarcoma.

36. Fibrous cortical defect & Non ossifying
fibroma
• The name fibrous cortical defect is used when the
lesion is confined to the cortex; however if becomes
large enough to extend into adjacent medullary cavity
than the term non-ossifying fibroma is used.
• Benign lesion of bone composed of spindleshaped fibroblasts, arranged in a storiform pattern,
with a variable admixture of multinucleated osteoclastlike giant cells. Foamy cells (xanthoma), chronic
inflammatory cells and hemosiderin may be present

37. Mainly involve – long bones distal femur,
proximal and distal tibia.
• Lesion- Eccentric, well circumscribed and have
sclerotic borders. The overlying cortex is
thinned and may be completely eroded.

38. .
Eccentric, lytic lesions centered
within the metaphyseal cortex and
adjacent medullary cavity of long
tubular bones.
- Well demarcated with sclerotic
margins .

39. Microscopic
•
:
Stroma of spindle-shaped fibroblasts, arranged in a whorled, storiform
pattern,among which variable number of small, multinucleated, osteclasttype giant cells are scattered.
-
•
Foam (xanthoma) cells, with small, dark nuclei are frequently, but not
always found interpersed among the stromal cells individually, or in small
clusters. Scattered inflammatory cells, mainly lymphocytes.

40. Fibrous dysplasia
• Benign tumor occuring due to local
developmental arrest.
• All the components of normal bone present but
do not differentiate into their mature structures.
• Three clinical patterns- monostotic, polyostotic
with, & without endocrine dysfunction.
• Somatic gain of function mutation of GNAS gene.

41. Monostotic- 70 %
• M ~ F , early
adolescence
• Femur , tibia, ribs,
jawbones & humerus.
• Causes marked
enlargement &
destruction of bones.
• Doesn’t evolve into poly
form
Polyostotic- 27%
• Slightly earlier age
• Femur , mandible ,
vertebrae
• Craniofacial
involvement common

42. • McCune Albright syndromePolyostotic FD
Skin pigmentation
endocrinopathies

44. • Microscopically, narrow, curved, and
misshaped bone trabeculae, often having a
characteristic fishhook configuration, are
interspersed with fibrous tissue of variable
cellularity[.
• Rows of cuboidal appositional osteoblasts do
not appear on the surface of the trabeculae.

46. Malignant fibrous histiocytoma
•
•
•
•
Age: 40 years
Bones involved: long bones & jaw.
Location: medullary portion of metaphysis,
These tumors arise in bone infarcts (often
secondary to sickle cell disease)
• following irradiation in Paget disease,
• Or as expression of ‘dedifferentiation’ or
anaplastic transformation in chondrosarcoma,
chordoma, or giant cell tumor

47. • X-Ray: osteolytic with a soap bubble appearance.
Gross: large , haemorrhagic, tan white masses
destroying the underlying bone.
Malignant fibrous histiocytoma is composed of a
spindle cell proliferation arranged in a storiform
pattern and giant cells and even malignant giant
cells are almost always seen.
By definition, the presence of chondroid or osteoid
matrix rules out a diagnosis of MFH

48. Microscopic

49. MISCELLANEOUS TM
Chordoma
• Low grade mesenchymal malignancy thought to arise
from notochord remnants .
• Age:50-60 yrs M> F
• Bone involved: sacrococcygeal area> spheno-occipital
area> cervicothoracolumbar spine.
• The sacrococcygeal tumors are more common in the
fifth and sixth decades of life.
• Whereas many of the spheno-occipital neoplasms
occur in children and adolescents.
• Chordomas commonly show hypodiploid karyotype,
frequently with loss of chromosomes 3 , 4, 10, and 13

50. X-Ray
Osteolytic destruction of sacrum by chordoma.

51. Gross
Grossly, chordoma is gelatinous and soft and contains
areas of hemorrhage

52. • Microscopically, it closely resembles normal
notochord tissue in its different stages of
development.
• It grows in cell cords and lobules separated by a
variable but usually extensive amount of mucoid
intercellular tissue and by fibrous septa .
• Some of the tumor cells (known as physaliferous)
are extremely large, with vacuolated cytoplasm
and prominent vesicular nucleus; some of the
cytoplasmic vacuoles contain glycogen,
presumably in the process of being broken down.
• In many of the tumor cells, vacuolization of the
cytoplasm gives rise to a bubbly appearance
termed physaliphorous
• . Areas of cartilage and bone may be presen

53. Microscopic

54. Chordoma with the diagnostic features of lobulation,
myxoid matrix, and vacuolated cells in a cord arrangement

55. Keratin

56. Adamantinoma of long bones
• Low grade mesenchymal malignancy of long
bones with focal epithelial differentiation .
• Bones involved: characteristically involves the
tibia but has been reported in other long
bones.
• Location: Diaphysis or metaphysis.

57. X-Ray

58. • Microscopically- several patterns of growth
have been described.
• The most common consists of solid nests of
basaloid cells with palisading at the periphery
and sometimes a stellate configuration in the
center.
• Less frequent forms have been described as
spindle, squamoid, and tubular

59. Microscopic

60. Keratin
The keratins expressed by adamantinoma are mainly 14 and 19, In
contrast to other bone and soft tissue tumors with epithelial
phenotypes – such as synovial sarcoma, chordoma, and epithelioid
sarcoma – it lacks immunoreactivity for keratins 8 and 18.

61. TUMOR LIKE LESIONS
Solitary bone cyst
• < 20 yrs
• M>F
• Long bones ( femur,tibia
& humerus ),
Metaphysis

62. A- A large lesion located in the upper metaphysis of the humerus.
B- A triangular lesion located in the upper end of the tibia. There
has been secondary hemorrhage.

63. • The cyst contains a clear or yellow fluid and The fluid may be
hemorrhagic if a previous fracture has occurred.
• Microscopically- well-vascularized connective tissue,
hemosiderin (often within macrophages), and cholesterol
clefts are frequent. The bone surrounding the cyst may have a
dense quality, with irregular cement lines

65. Aneurysmal bone cyst
•
•
•
•
•
Benign , rapidly growing expansile tumor
10-20 yrs
M< F
Vertebrae & flat bones
Blue bone

66. .
Usually eccentric, expansile lesion
with well defined margins.
- Most lesions are completely lytic
and often contain thin shell of
reactive bone at the periphery.
-
CT and MRI may demonstrate
internal septa and characteristic
fluid-fluid level

67. Aneurysmal bone cyst of lower end of ulna. The large blood-filled
cavities expand the metaphysis

68. Microscopic appearance, showing two cavities lined by osteoclast-like
multinucleated giant cells. The intervening stroma is cellular.

69. Langerhans cell Histiocytosis
• Histiocytosis X, eosinophilic granuloma
• Infiltration by a cell of immune system known
as Langerhans cell.
• Young adults
• Cranial vault, jaw, humerus, ribs
• Birbecks granule on electron microscopy
• 3 types- solitary bone, multiple bone +/- skin,
multiple organ

70. • Langerhans cells have a characteristic
morphologic appearance . Their nuclei often are
lobulated or indented, sometimes with a
longitudinal groove; their cytoplasm is, for the
most part, distinctly acidophilic.
• A specific intracytoplasmic organelle, known as
Langerhans or Birbeck granule, is regularly
present on electron microscopic examination.
• Diagnostic immunohistochemical markers
include S100 protein, CD1a and langerin (CD207

71. A sharply circumscribed, dark brown lesion is seen
Osteolytic lesion of skull in a 25-year-old woman. Radiographically, the lesion
was thought to be metastatic carcinoma but proved to be a solitary lesion of
Langerhans cell histiocytosis

72. Langerhans cell histiocytosis. Polymorphic appearance resulting from an
admixture of Langerhans cells, nonspecific histiocytes, lymphocytes, and
eosinophils. There is a mild atypia in the Langerhans cells that can simulate a
malignant process

73. Myositis ossificans
• It is reactive condition that is sometimes
mistaken microscopically for osteosarcoma.
• The term is inaccurate because the muscle
may not be involved, and inflammation is
virtually absent.
• The most common locations are the flexor
muscles of the upper arm .

74. • Radiographic studies show periosteal reaction
and faint soft tissue calcification within 3–6
weeks of the injury;
• These are gradually replaced by mature
heterotopic bone by 10–12 weeks

75. Well-defined myositis ossificans , illustrating bone formation in
periphery

76. • Microscopically- there is a highly cellular stroma associated
with new bone and, less commonly, cartilage formation.
• In an early lesion, the centrally placed areas may be very
difficult to distinguish from osteosarcoma because of their
extreme cellularity. As the process evolves, osteoid appears
in an orderly pattern at the periphery of this mass and
subsequently matures into well-developed bone. Several
microscopic subtypes have been described, which
correspond to different stages of the process.
• The most important diagnostic feature is provided by the
maturation pattern (‘zonal phenomenon’), characterized by
a central cellular area, an intermediate zone of osteoid
formation, and a peripheral shell of highly organized bone

78. Metastatic disease of bone
• Most common form of skeletal malignancy.
• The pathway of spread include:
*direct extension
*lymphatic or hematogenous
*intraspinal seeding
• >75% of skeletal metastasis originate from
prostate ,breast ,kidney and lung.

79. • Most common bones involved are axial
skeleton, proximal femur and humerus.
• Metastatic bone lesions are usually osteolytic
but may be osteoblastic or mixed.

80. • Osteoblastic metastasis
*Prostatic carcinoma
*Carcinoid tumour
*Breast tumour (less
commonly)

81. • Osteolytic metastasis:
*Kidney
*Lung
*GIT
*Malignant
melanoma
*Thyroid

83. TUMOUR
LOCATION
FIBROUS
DYSPLASIA
RIBS,JAW,LB 10-30 IRREGULAR WOVEN BONE WITHIN FIBROBLASTIC
SSTROMA
MEDULLARY
NONOSSIYING
FIBROMA
LBS
5-15
BLAND SPINDLE CELLS IN STORIFORM
PATTERN+HISTIOCYTES+GIANT CELLS
BENIGN FIBROUS
HISTIOCYTOMA
LBS ,PELVIS
>20
IDENTICAL TO NONOSSIFYING FIBROMA BUT
VARIABLE
5-15
MIXED INFLAMMATORY CELLS AND EOSINOPHILS
.S100/CD1a-POSITIVE CELLS WITH GROOVED
/MULTILOBATED NUCLEI
LANGERHANS
CELL
HISTIOCYTOSIS
SKULL.JAW,
LBS
AGE
SALIENT PATHOLOGIC FINDING

84. TUMOUR
LOCATION
AGE
SALIENT PATHOLOGIC FINDING
GIANT CELL
TUMOR
EPIPHYSIS
OF LBS
20-45 EVENLY PLACED GIANT CELLS AMONG
MONONUCLEAR CELLS WITH IDENTICAL NUCLEI.
ANEURYSMAL
BONE CYST
VERTEBRAE
LBS
10-20 BLOOD FILLED SPACES SEPERATED BY FIBROUS
SEPTAE, GIANT CELLS
SIMPLE BONE
CYST
METAPHYSIS 10-20 FLUID FILLED CYSTS LINED BY CONNECTIVE TISSUE
OF LBS

85. TUMOUR
LOCATION
AGE
SALIENT PATHOLOGIC FINDING
ADAMANTINOMA
CORTEX OF
TIBIA
25-35 EPITHELIAL CELLS+FIBROBLAST+WOVEN OR
LAMELLAR BONE
EWING SARCOMA
DIAPHYSIS
OF LBS
5-20
SMALL ROUND BLUE CELLS+ROSETTES ,t (11,22)
CHORDOMA
BASE OF
SKULL
>30
LOBULES OR VACUOLATED CELLS EMBEDDED IN
MYXOID MATRIX.

86. Thank You

87. Chickenwire Pattern
A descriptor for a delicate plexiform or reticulated pattern imposed on
that of another density or appearance
Dermatology Livedo vasculitis pattern A pattern of cyanotic
discolouration of the skin with or without an underlying vascular
pathology, which may be triggered by the cold and associated with cold
cryoglobulinaemia
Imaging A descriptive term for a pattern of intralesional calcification
which is regarded as pathognomonic for chondroblastoma, a paediatric
tumour
Pathology—liver A pattern of fibrosis associated with alcoholic
hepatitis. Cf Bridging fibrosis
Pathology—soft tissue
The arrangement of capillaries in myxoid liposarcoma, which may also
be seen in low-grade fibromyxoid sarcoma, clear cell sarcoma of the
kidney, in which hyalinised cell cords are superimposed on a sclerotic
background

88. Storiform Pattern
A pattern seen by low-power light microscopy, which is
characterised by loosely-arranged whorls of elongated,
spindled fibroblast-like cells. The highly nonspecific
storiform pattern is often seen in fibrohistiocytic lesions. It
may be benign—e.g., dermatofibroma, giant cell tumour of
tendon sheath—of low malignant potential—e.g., atypical
fibroxanthoma, dermatofibrosarcoma protuberans—or
malignant—e,g, fibrosarcoma; it may also be seen in nonhistiocytic lesions—e.g., nodular fascitis, leiomyoma,
leiomyosarcoma, Schwann cell tumours, spindle cell
carcinoma