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Bone tumors II
MODERATOR- DR. S. S. THAKUR
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
X -Ray

Typical radiograph of giant cell tumor of distal end of femur involving epiphysis and
metaphyseal area.
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
Microscopic

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

Cytology
Acid phosphatase
IHC
• lysozyme
• α-1-antitrypsin
• α-1-antichymotrypsin
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
Malignant GCT
• Clinical, topographic & general microscopic
features of GCT but exhibits clear cut evidence
of malignancy in the mononuclear stromal
component.
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
X-Ray

.
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
Microscopic

Diffuse pattern of growth and monotonous cytologic appearance in
Ewing sarcoma/PNET.
PAS stain
CD 99
Differential Diagnosis
• Malignant Lymphoma and Related Lesions.
• Desmoplastic small cell tumour.
• Embryonal/alveolar rhabdomyosarcoma.
• Neuroblastoma
• Medulloblastoma
• Retinoblastoma
• Wilms tumor
• Osteosarcoma
• Metastatic Tumors [Bone]
IHC
• LMWK
• Neuron specific enolase
• Leu 7
Prognosis
•
•
•
•
•
•
•
•
•

Osseous versus extraosseous location
Direct soft tissue extension
Metastasis
Surgical margins
Therapy-induced necrosis
Microscopic features
Neural differentiation
p53
c-myc amplification.
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.
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
Malignant lymphoma involving lower end of femur associated with bone
destruction and bone production
Microscopic

IHC
CD 45 +
Bcl 2
Malignant lymphoma of bone. The
tumor is of large cell type and is
associated with some fibrosis
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
Microscoipc
Differential Diagnosis
• Angiosarcoma [Bone]
• Epithelioid Hemangioendothelioma.
• Aneurysmal Bone Cyst
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.
X-Ray

Multicentric hemangioendothelioma involving multiple
bones of the foot
Microscopic
Factor VIII
Angiosarcoma
• Malignant vascular neoplasm.
• Age/sex: older adults/M:F=1:1.
• Location: anywhere in body.
Microscopic

Angiosarcoma of bone. Anastomosing vascular channels lined by
highly atypical endothelial cells are seen
Factor VIII
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
Fibrous Tumours
• Benign: Fibrous Cortical
Defect, Non-Ossifying
Fibroma.

• Benign Aggressive:,
Fibrous Dysplasia.

• Malignant: Malignant
Fibrous Histiocytoma of
bone, Fibrosarcoma.
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
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.
.

Eccentric, lytic lesions centered
within the metaphyseal cortex and
adjacent medullary cavity of long
tubular bones.
- Well demarcated with sclerotic
margins .
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.
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.
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
• McCune Albright syndromePolyostotic FD
Skin pigmentation
endocrinopathies
• 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.
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
• 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
Microscopic
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
X-Ray

Osteolytic destruction of sacrum by chordoma.
Gross

Grossly, chordoma is gelatinous and soft and contains
areas of hemorrhage
• 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
Microscopic
Chordoma with the diagnostic features of lobulation,
myxoid matrix, and vacuolated cells in a cord arrangement
Keratin
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.
X-Ray
• 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
Microscopic
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.
TUMOR LIKE LESIONS
Solitary bone cyst
• < 20 yrs
• M>F
• Long bones ( femur,tibia
& humerus ),
Metaphysis
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.
• 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
Aneurysmal bone cyst
•
•
•
•
•

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

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
Aneurysmal bone cyst of lower end of ulna. The large blood-filled
cavities expand the metaphysis
Microscopic appearance, showing two cavities lined by osteoclast-like
multinucleated giant cells. The intervening stroma is cellular.
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
• 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
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
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
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 .
• 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
Well-defined myositis ossificans , illustrating bone formation in
periphery
• 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
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.
• Most common bones involved are axial
skeleton, proximal femur and humerus.
• Metastatic bone lesions are usually osteolytic
but may be osteoblastic or mixed.
• Osteoblastic metastasis
*Prostatic carcinoma
*Carcinoid tumour
*Breast tumour (less
commonly)
• Osteolytic metastasis:
*Kidney
*Lung
*GIT
*Malignant
melanoma
*Thyroid
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
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
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.
Thank You
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
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

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Bone tumour seminar ,ewing sarcoma, chordoma,

  • 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
  • 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
  • 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.
  • 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
  • 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.
  • 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
  • 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
  • 43.
  • 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.
  • 45.
  • 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
  • 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
  • 54. Chordoma with the diagnostic features of lobulation, myxoid matrix, and vacuolated cells in a cord arrangement
  • 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
  • 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
  • 64.
  • 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
  • 77.
  • 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)
  • 82.
  • 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.
  • 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

Editor's Notes

  1. microscopically by the presence of epithelial- or histiocyte-like endothelial cells with abundant acidophilic and often vacuolated cytoplasm, large vesicular nucleus (sometimes with prominent grooves), modest atypia, scanty mitotic activity, inconspicuous or absent anastomosing channels, recent and old hemorrhage, and an inconstant but sometimes prominent inflammatory component rich in eosinophils