BONE TUMORS

Bone Tumors & Tumor-Like Lesions

Bone Tumors & Tumor-Like Lesions

 Osteoma
Osteoid Osteoma and Osteoblastoma
Osteosarcoma

Osteoma
Bosselated, round-tooval sessile tumors
Subperiosteal surface of
cortex
On or inside skull and
facial bones
Usually solitary
Middle age

Osteoid Osteoma and Osteoblastoma

Osteoid osteoma
<2cm, teens and 20s,
M>F 2:1
Appendicular skeleton,
posterior elements of
spine
Cortex>medullary cavity
Symptoms
Nocturnal pain
Relieved by aspirin

Osteoblastoma
>2cm
Spine
Symptoms
Dull, achy
Unresponsive to
salicylates

Osteoid Osteoma and Osteoblastoma

Osteosarcoma
Most common primary
malignant tumor (20%)
Mesenchymal tumor
Bimodal age distribution
M>F (1.6:1)
Metaphyseal region of long
bones

Osteosarcoma
Genetic abnormality
RB
P53
INK4a (p16)

Characteristics
Metaphysis of long
bones
Primary
Solitary
Intramedullary
Poorly differentiated

Osteosarcoma
Gross
Big, bulky tumors
Gritty, gray-white
Areas of hemorrhage &
cystic degeneration

Osteosarcoma
Microscopic
Large hyperchromatic
nuclei
Bizarre tumor giant cells
Mitosis
Formation of bone
Coarse, lace like
architecture

Osteosarcoma
Clinical Course
Painful
Progressively enlarging
mass
Reactive periosteal
formation
Hematogenous spread
(+) metastases: lungs,
bones, brain

Osteochondroma
Chondroma
Chondroblastoma
Chondromyxoid Fibroma
Chondrosarcoma

Osteochondroma
Exostosis
Benign
Attached to skeleton by
a bony stalk
Most common benign
bone tumor
Solitary
Autosomal dominant:
EXT1, EXT2

Late adolescencce, early

adulthood
M>F (3:1)
Endochondral origin
Metaphysis, near growth
plate of long tubular
bones (knee)

Osteochondroma
Sessile
Mushroom shaped
1-20cm
Cap: benign hyaline
cartilage covered by
perichondrium
Cortex of stalk merge
with host bone

Slow-growing mass
Can be painful
Incidental finding
Stop growing at time
of growth plate
closure
Can give rise to
chondrosarcoma

Osteochondroma

FIGURE 26-25 Osteochondroma. A, X-ray of an osteochondroma arising off the posterior

surface of the tibia. B, Axial CT scan shows continuity of the cortex of the bone and the
center of the osteochondroma. The fibula is adjacent to the mass. C, Gross specimen of
sessile osteochondroma composed of a cap of hyaline cartilage undergoing enchondral
ossification. D, The cartilage cap has the histologic appearance of disorganized growth
plate-like cartilage.

Chondroma and Enchondroma

Benign
Hyaline cartilage
Bones of endochondral
origin
Medullary cavity:
enchondromas
Surface of bone:
subperiosteal /
juxtacortical chondromas

20-40s
Solitary
Metaphyseal lesion of
tubular bones (short
tubular bones of hands
and feet)
Ollier disease
(enchondromatosis)
Maffucci Syndrome

Chondroma
<3cm
Gray-blue, translucent
Well-circumscribed
nodules

Chondroma

Asymptomatic
Cause pathologic fracture
Numerous, large tumors
C or O ring sign

Chondroblastoma
Rare, benign tumor
Teens, M>F 2:1
Knee
Epiphyses and apophyses
Sheets of chondroblasts
Well-defined cytoplasmic
borders
Moderate pink cytoplasm
Hyperlobulated nuclei
Chicken-wire mineralization

Scattered through the lesion are non-neoplastic

osteoclast-type giant cells.
Occasionally the tumors undergo prominent
hemorrhagic cystic degeneration.

Chondromyxoid firboma

Rarest
Teens, 20s
M>F
Metaphysis of long
tubular bones
3-8cm
Well-circumscribed
Solid
Glistening tan-gray

Chondrosarcoma
Neoplastic cartilage
Central (intramedullary)
vs peripheral
(juxtacortical and
surface)
Conventional (hyaline
and/or myxoid), clear
cell, dedifferentiated,
mesenchymal variants

Second most common

malignant matrixproducing tumor
>40s
M>F 2:1
Malignant hyaline and
myxoid cartilage
Nodules of gray-white
translucent glistening
tissue

Chondrosarcoma
Predominantly myxoid
Viscous, gelatinous
tumors
Spotty calcifications
present
Central necrosis
cystic spaces
Grading

Chondrosarcoma
Central portions of
skeleton
Rarely involves distal
extremities
Painful, enlarging mass
Imaging: endosteal
scalloping

Prognostic
As grade progresses,
chance of metastases
increases (lungs,
skeleton)
>10cm aggressive

Treatment
Wide surgical incision
Chemotherapy

Fibrous Cortical Defect and Non-Ossifying Fibroma

FCD common (30-50%, >2y/o)
Metaphysis of distal femur and proximal tibia
Bilateral, multiple
0.5cm 5 or 6cm (non-ossifying fibroma)

Fibrous Cortical Defect and NonOssifying Fibroma

Morphology
Elongated, sharply
demarcated
radiolucencies
Surrounded by thin rim
of sclerosis

Fibrous Cortical Defect and NonOssifying Fibroma

Gray-yellow brown cellular
lesions with fibroblast and
macrophage
Storiform (pinwheel)
pattern
Histiocytes are
multinucleated giant cells
or clusters of foamy
macrophages

Fibrous Dysplasia
Benign tumor
Patterns:
Does not differentiate to
1. Invovlement of single
mature structures
bone (monostotic)
Mutation in GNAS gene
2. Invovlement of multiple
bones (polyostotic)
3. Polyostotic disease, with
caf-au-lait skin
pigmentation and
endrocrine abnormalities

Monostotic fibrous dysplasia Polyostotic fibrous dysplasia

w/o endocrine dysfunction
70% of cases
27% of cases
M=F, early adolescence
Early age
Stops enlarging at time
Craniofacial involvement
of growth plate closure
Femur, tibia, ribs,
Crippling deformities
jawbones, calvaria,
Recurrent fractures
humerus
Asymptomatic
Cause pain, fracture,
discrepancy in limb
length

Polyostotic Fibrous Dysplasia

assoc. w/ caf-au-lait
pigmentation and
endocrinopathies
McCune-Albright syndrome
3% of cases
Precocious sexual
development (F>M)
Pigmentation: neck, chest,
back, shoulder, pelvic region

Polyostotic Fibrous Dysplasia assoc.

w/ caf-au-lait pigmentation and
endocrinopathies
Morphology
Well-circumscribed
Intramedullary
Tan-white and gritty
Curvilinear trabeculae of
woven bone
Moderately cellular
fibroblastic proliferation
Trabeculae mimic
Chinese letters

Fibrosarcoma variants
Collagen-producing with
fibroblastic phenotype
Middle-aged, elderly
M=F
Morphology
Gross
Large, hemorrhagic
Tan-white mass
Destroy bone and extend
to soft tissues

Microscopic
Malignant fibroblast
Herringbone storiform
pattern
Malignant fibrous
histiocytoma

Ewing sarcoma and Primitive

Neuroectodermal tumor (PNET)
primary malignant small round cell tumors of
bone and soft tissue
Identical chromosome translocation, differing
only in their degree of neural differentiation
neural differentiation PNETs
undifferentiated - Ewing sarcoma

Ewing sarcoma and Primitive

Neuroectodermal tumor (PNET)
2nd most common group of bone sarcomas in
children
10 to 15 years old (~ 80% are < 20 years)
Translocation: EWS gene on chromosome 22
and most commonly involved ETS gene is
FLI1, as part of a (11;22) (q24;q12)
translocation
abnormal cell proliferation and survival

Sheets of small round cells with small amounts of clear

cytoplasm
arises from the medullary cavity, invading the cortex, periosteum, and soft tissue.
tumor is soft, tan-white, and frequently contains areas of hemorrhage and necrosis
composed of sheets of uniform small, round cells that are slightly larger than lymphocytes with scant clear cytoplasm
Homer-Wright rosettes (tumor cells arranged in a circle about a central fibrillary space) is indicative of neural differentiation

Ewing sarcoma and Primitive

Neuroectodermal tumor (PNET)
Diaphysis of long tubular bones (femur and
flat bones of the pelvis)
Systemic findings: fever, sedimentation
rate, anemia, and leukocytosis,
Plain radiograms - destructive lytic tumor
periosteal reaction produces layers of
reactive bone deposited in an onion-skin
fashion
Treatment: chemotherapy and surgical
excision with or without irradiation

Giant-cell Tumor
Mixture of mononuclear cells and
multinucleated osteoclast-type giant cells
(osteoclastoma)
Uncommon benign-locally aggressive
neoplasm
RANK/RANKL signaling pathway

Giant Cell Tumor

Abundance of multinucleated giant cells with background mononuclear

stromal cells
large, red-brown tumors that frequently undergo cystic degeneration
composed of uniform oval mononuclear cells that constitute the proliferating component of the tumor
osteoclast-type giant cells having 100 or more nuclei that resemble those of the mononuclear cells
necrosis, hemorrhage, hemosiderin deposition, and reactive bone formation are common secondary
features

Giant Cell Tumor

Adults - epiphyses and the metaphyses

Adolescents -limited to the metaphysis
Majority arise around the knee
Most are solitary
Erode into the subchondral bone plate and
destroy the overlying cortex

Giant Cell Tumor

Magnetic resonance image
of a giant-cell tumor that
replaces most of the femoral
condyle and extends to the
subchondral
bone plate

Aneurysmal Bone Cyst

Benign tumor
Multiloculated blood-filled cystic spaces
17p13 translocations that result in upregulation of USP6, a deubiquitinating
enzyme

Aneurysmal Bone Cyst

multiple blood-filled cystic spaces separated by thin, tan-white septa

walls are composed of plump uniform fibroblasts (which may be mitotically active),
multinucleated osteoclast-like giant cells, and reactive woven bone
bone is lined by osteoblasts that follow the contours of the fibrous septa

Aneurysmal Bone Cyst

Generally occurs during the first 2 decades of
life
Metaphyses of long bones and the posterior
elements of vertebral bodies
Radiograph- eccentric, expansile lesion with
well-defined margins
Most lesions are completely lytic and often
with thin shell of reactive bone

Aneurysmal Bone Cyst

Soft-tissue component is delineated by a

thin rim of reactive subperiosteal bone

Characteristic fluid-fluid levels

Metastatic Disease
most common form of skeletal malignancy
Pathways
(1) direct extension
(2) lymphatic or hematogenous dissemination
(3) intraspinal seeding (via the Batson plexus of
veins)

Metastatic Disease
adults: > 75% from cancers of the prostate,
breast, kidney, and lung
children: neuroblastoma, Wilms tumor,
osteosarcoma, Ewing sarcoma, and
rhabdomyosarcoma
most involve the axial skeleton > proximal
femur > humerus

REACTIVE TUMOR-LIKE LESIONS OF

JOINTS

Ganglion Cyst
ganglion cyst is a small (1 to 1.5 cm) cyst that
is almost always located near a joint capsule
or tendon sheath
firm, fluctuant, pea-sized translucent nodule
Usually around wrist joints
Fluid-filled but non communicating
Myxoid degeneration and softening of
connective tissue

Ganglion Cyst

Synovial Cyst
Herniation of synovium through a joint
capsule or massive enlargement of a bursa
may produce a synovial cyst
Baker cyst (RA setting)
Synovial lining may be hyperplastic and
contain inflammatory cells and fibrin but is
otherwise unremarkable

Synovial Cyst

Villonodular Synovitis and Giant Cell

Tumor of Tendon Sheath
Group of benign neoplasms that develop in
the synovial lining of joints, tendon sheaths,
and bursae
Consistent chromosomal aberrations
(neoplastic nature)
Presents as a monoarticular arthritis that
affects the knee in 80% of cases

PVNS

GCT

SOFT TISSUE TUMORS

Sources

Fat
Fibrous tissue and Fibrohistiocytic
Skeletal muscle
Smooth muscle
Vascular
Peripheral nerve
Uncertain: synovial sarcoma, alveolar soft part sarcoma,

epitheliod sarcoma

Soft tissue tumors

Causes of most are unknown
Documented associations with radiation or
previous tissue injury
Genetic syndromes
neurofibromatosis type 1 (neurofibroma,
malignant schwannoma)
Gardner syndrome (fibromatosis)
Li-Fraumeni syndrome (soft tissue sarcoma)
Osler-Weber-Rendu syndrome (telangiectasia)

Soft tissue tumors

40% occur in the lower extremities, especially
the thigh
Fifteen per cent arise in children(4th most
common malignancy)
Grading (I to III) is based on degree of
differentation, mitoses, pleomorphism,
necrosis
Clinical staging involves the extent and size of
the tumor

Soft tissue tumors

Soft tissue tumors

FAT
lipoma
liposarcoma

Normal fat

Lipoma,
encapsulated

Liposarcoma, often
retroperitoneal

Lipoma
More common in women
Benign soft tissue tumor composed of
differentiated fat cells
Subcutaneous, mostly occur on the upper half
of the body (trunk, neck)
Can occur in deep tissues (intramuscular,
intermuscular)
5th to 6th decade of life

Liposarcoma
One of the most common sarcomas of adulthood
40s to 60s
Arise in the deep soft tissues of the proximal
extremities and retroperitoneum
Can be Well differentiated
Myxoid
Round cell type
Pleiomorphic type

Fibrous tissue
Nodular fasciitis
(pseudosarcomatous)
Fibromatoses
(plantar, palmar, penile)
Fibrosarcoma

Nodular fascitiis
infiltrative or pseudosarcomatous fasciitis
most common of the reactive pseudosarcomas
often occurs in adults on the volar aspect of the
forearm
arise in the deep dermis, subcutis, or muscle
several-week history of a solitary, rapidly
growing, and sometimes painful mass
Sometimes associated with preceding trauma

Nodular Fascitiis

Nodular fasciitis is richly cellular and consists of plump, immature-appearing fibroblasts

arranged randomly (simulating cells growing in tissue culture) or in short intersecting fascicles,
The cells vary in size and shape (spindle to stellate) and have conspicuous nucleoli and
abundant mitotic figures

Myositis Ossificans
presence of metaplastic bone
usually develops in athletic adolescents and
young adults
follows an episode of trauma >50% of cases
Early phase: swelling and pain
Later stage: well circumscribed, firm mass
Final stage: painless, hard, well-demarcated mass
Progress in as little time as 3 weeks

Myositis Ossificans

MYOSITOS OSSIFICANS can be thought of as being a METAPLASTIC process, often following inflammationi.e.:
Usual scenario:InflammationFibrosis
Myositis Ossificans:InflammationFibrosisOssification

Superficial Fibromatoses
Palmar (Dupuytren contracture)
irregular or nodular thickening of the palmar fascia
progressive flexion contracture develops
fourth and fifth fingers of the hand

Plantar
Similar to plantar fibromatoses, except contractures
and bilaterality are uncommon

Penile (Peyronie disease)

palpable induration or mass appears usually on the

dorsolateral aspect of the penis

Deep seated Fibromatoses

(Desmoid tumors)
Borderland behavior
Large, infiltrative masses recur after
incomplete excision
Extra-abdominal (shoulder, chest wall, back, and
thigh)
Abdominal (Abdominal musculoaponeurotic in
women)
Intra-abdominal (Intra-abdominal; associated with
FAP)

Fibrosarcoma
Rare, malignant, collagen-forming non
pleomorphic, fibroblastic cell tumor, mainly of
adulthood, occasionally congenital
Unencapsulated, well circumscribed, soft
masses
recurring in more than 50% of the cases and
metastasizing in more than 25%

Fibrosarcoma

Note the malignant spindle cells arranged in

a herringbone pattern

Skeletal Muscle
Rhabdomyoma

Rhabdomyosarcoma

Rhabdomyosarcoma
most common soft tissue sarcoma of childhood
and adolescence
<20 years old
most occur in the head and neck or
genitourinary tract
With embryonic, alveolar, and pleomorphic
variants
Involve t(2;13)(q35;14) translocations
Rhabdomyoblast - diagnostic cell

Rhabdomyosarcoma

Alveolar rhabdomyosarcoma
with numerous spaces lined by
tumor cells

Rhabdomyosarcoma composed of malignant

small round cells. The rhabdomyoblasts are
large and round and have abundant
eosinophilic cytoplasm.

Smooth Muscle

Leiomyomasarcoma
Leiomyoma

Leiomyoma
Benign
Most often arising from the uterus, but also
from erector pili muscles found in the skin,
nipples, scrotum, and labia
tendency to develop multiple lesions is
thought to be hereditary

Leiomyosarcoma
10% to 20% of soft tissue sarcomas
Skin and deep soft tissues of the extremities
and retroperitoneum
Painless firm masses
malignant spindle cells that have cigar-shaped
nuclei arranged in interweaving fascicles

Synovial Sarcoma
Cell of origin is still unclear
20s to 40s
deep soft tissue extremities
60% to 70% involve the lower extremities
chromosomal translocation t(x;18) producing
SYT-SSX1 or -SSX2 fusion genes
Commonly metastasizes to the lung, skeleton

Unknown- Synovial Sarcoma

-dual line of differentiation of the tumor cells (i.e., epithelial-like and spindle cells); The

epithelial cells are cuboidal to columnar and form glands or grow in solid cords or aggregates,
The spindle cells are arranged in densely cellular fascicles that surround the epithelial cells

END