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
Bone tumour seminar ,ewing sarcoma, chordoma,
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
Typical radiograph of giant cell tumor of distal end of femur involving epiphysis and
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
Giant cell tumor.-The nuclei of the giant cells are similar to those of the mononuclear cells
• 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
Malignant lymphoma involving lower end of femur associated with bone
destruction and bone production
CD 45 +
Malignant lymphoma of bone. The
tumor is of large cell type and is
associated with some fibrosis
• Benign neoplasm of blood vessels.
• Bones involved: vertebrae, skull and jaw
• Multiple hemangiomas are mainly seen in
children and young adults.
– Often current jelly appearance
• 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
Multicentric hemangioendothelioma involving multiple
bones of the foot
Fibrous cortical defect & Non ossifying
• 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
- Well demarcated with sclerotic
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.
• Benign tumor occuring due to local
• 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
• Femur , tibia, ribs,
jawbones & humerus.
• Causes marked
destruction of bones.
• Doesn’t evolve into poly
• Slightly earlier age
• Femur , mandible ,
• Microscopically, narrow, curved, and
misshaped bone trabeculae, often having a
characteristic fishhook configuration, are
interspersed with fibrous tissue of variable
• 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
• 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
Osteolytic destruction of sacrum by chordoma.
Grossly, chordoma is gelatinous and soft and contains
areas of hemorrhage
• Microscopically, it closely resembles normal
notochord tissue in its different stages of
• 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
• . Areas of cartilage and bone may be presen
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
• Location: Diaphysis or metaphysis.
• 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
• Less frequent forms have been described as
spindle, squamoid, and tubular
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
• Long bones ( femur,tibia
& humerus ),
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
Vertebrae & flat bones
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
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,
• 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
• 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
• 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
• 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:
*lymphatic or hematogenous
• >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.
RIBS,JAW,LB 10-30 IRREGULAR WOVEN BONE WITHIN FIBROBLASTIC
BLAND SPINDLE CELLS IN STORIFORM
IDENTICAL TO NONOSSIFYING FIBROMA BUT
MIXED INFLAMMATORY CELLS AND EOSINOPHILS
.S100/CD1a-POSITIVE CELLS WITH GROOVED
SALIENT PATHOLOGIC FINDING
SALIENT PATHOLOGIC FINDING
20-45 EVENLY PLACED GIANT CELLS AMONG
MONONUCLEAR CELLS WITH IDENTICAL NUCLEI.
10-20 BLOOD FILLED SPACES SEPERATED BY FIBROUS
SEPTAE, GIANT CELLS
METAPHYSIS 10-20 FLUID FILLED CYSTS LINED BY CONNECTIVE TISSUE
SALIENT PATHOLOGIC FINDING
25-35 EPITHELIAL CELLS+FIBROBLAST+WOVEN OR
SMALL ROUND BLUE CELLS+ROSETTES ,t (11,22)
LOBULES OR VACUOLATED CELLS EMBEDDED IN
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
Imaging A descriptive term for a pattern of intralesional calcification
which is regarded as pathognomonic for chondroblastoma, a paediatric
Pathology—liver A pattern of fibrosis associated with alcoholic
hepatitis. Cf Bridging fibrosis
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
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