BONE TUMORS Osteochondroma

aka Exostosis
Benign Bone Tumors
May present in a number of ways
The more common benign lesions are often
asymptomatic incidental findings. Many tumours
however produces pain or slow growing mass.
First hint of tumor presence: Pathologic Fracture
Benign tumors greatly outnumber their malignant
counterparts
Occur with greater frequency within the first 3
decades of life.
Osteoma
A benign neoplasm that frequently involves the skull
and facial bones
Is a new piece of bone usually growing on another
piece of bone
Hyperostosis frontalis interna
-Describes an osteoma that extends into the orbit or
sinuses
Osteoma can be associated with Gardner Syndrome
familial colorectal polyposis
Presence of multiple polyps that sometimes
includes the bone.
Osteoid Osteoma
Benign painful growth of the diaphysis of a long bone,
often the tibia or femur [80 to 90%]
most common cause of painful scoliosis in the adolescent
population
Tend to be <1.5 to 2 cm in size
Male > Female : Peak age 5 to 25 yo
Pain worse at night, relieved by aspirin
Dx: X ray : shows central radiolucency surrounded by a
sclerotic rim.
Benign bony metaphyseal growth capped with cartilage
that originates from epiphyseal growth plate.
Present in adolescent males who have firm, solitary
growths at the ends of long bone.
May be asymptomatic, cause pain, produce deformity, or
undergo malignant transformation [rare]
Incidence : the most common benign bone tumor
demographics: common in adolescents and young adults
Location
occur on the surface of the bone and often at
sites of tendon insertion
common locations include
knee (proximal tibia, distal femur)
proximal femur
proximal humerus
Etiology
mutation in EXT gene affects prehypertrophic
chondrocytes of growth plate
Inheritance:
autosomal dominant
Dx: X ray:
>sessile (broad base) or pedunculated (narrow
stalk) lesions found on the surface of bones
>cartilage cap is usually radiolucent and involutes at
skeletal maturity
Microscopically similar to a normal physis with:
cartilage cap consists of hyaline cartilage
well defined perichondrium around the cartilage
cap
normal primary trabeculae
linear clusters of active chondrocytes

Osteoblastoma
Similar to osteoid osteoma clinically and histologically
but is larger [>2cm]
Often involves the vertebrae
demographics
males > females (2:1)
majority of patients 10-30 years of age
Endochondroma Giant Cell Tumor of Bone
Benign cartilaginous growth within the medullary cavity aka as Osteoclastoma
of the bone, usually involves the hand and feet. An uncommon benign neoplasm containing
Tumor is typically solitary, asymptomatic, and requires multinucleated giant cells admixed with stromal cells.
no treatment. Age & sex
Incidence more common in females
2nd most common benign cartilage lesion ages 30-50 years
(osteochondroma is most common) Location
demographics distal femur > proximal tibia > distal radius >
male:female ratio is 1:1 sacral ala
most common in 20-50 year olds 50% occur around knee (distal femur or
Location proximal tibia)
usually found in the medullary cavity of 10% in sacrum and vertebrae (sacral ala is
the diaphysis or metaphysis most common site in axial skeleton)
the most common location is the hand (60%) in the mobile spine, it usually occurs
the most common bone tumor in the in the vertebral body
hand is the enchondroma distal radius is third most common location
other locations include the distal phalanges of the hand is also a very
femur (20%), proximal humerus (10%) and tibia common location
rare in the spine and pelvis Physical exam
Pathophysiology : palpable mass
Enchondromas represent incomplete endochondral decreased range of motion around affected
ossification joint
chondroblasts and fragments of Clinically:
epiphyseal cartilage escape from the The tumor produces bulky mass with pain
physis and is displaced into the and fractures
metaphysis and proliferate there Locally aggresive with high rate of
reccurrence [40 to 60%]
Multiple endochondromas can occur as part of both: -approximately 4% will metastasize to the
Ollier Disease: enchondromas throughout lungs.
the metaphyses and diaphyses of long bones Dx:
Maffuci Syndrome: markedly expand the bone X ray:
and angiomas are seen as small, round calcified Expanding lytic lesion surrounded by a thin
phleboliths rim of bone, which has soap bubble
appearance.
Dx: Radiology
"pop-corn" stippled calcification and rings Microscopically:
hallmark giant cells are numerous, nuclei of
giant cell appears same as stromal cells
MALIGNANT BONE TUMORS

OSTEOSARCOMA
This is the most common primary malignant EWINGS SARCOMA
tumor of bone.
The peak incidence is in males 10 to 20 years of
age.
The tumor occurs most frequently in the
metaphysis of long bones; the proximal portion of
the tibia and most distal portion of the femur
(around the knee 50%) are preferred sites.
Main histologic variants:
Osteoblastic Osteosarcoma show prominent
osteoid formation
Chondroblastic Osteosarcoma form cartilaginous
matrix and with scant bony differentiation.
This extremely anaplastic small blue cell malignant
tumor has a morphologic resemblance to malignant
lymphoma.
It occurs most often in long bones, ribs, pelvis, and
scapula.
It has a peak incidence in boys younger than 15 years
of age.
It follows an extremely malignant course with early
metastases.
Genetic Mutations:

CODMAN TRIANGLE is a term used to describe the

triangular area of subperiosteal bone that is created
when a lesion or tumor raises the periosteum away from
the bone.
Histopathology
Predisposing Factors
Homogenous and densly packed
Paget disease of bone
undifferentiated small round blue cells like
Ionizing radiation
lymphocytes; regular nuclei, infrequent mitoses,
Mutations
scant clear cytoplasm
p53 (Li-Fraumeni syndrome)
Abundant glycogen: PAS + diastase digestible
Rb (Hereditary Retinoblastoma)
Homer-Wright pseudorosettes

CHONDROSARCOMA
This is a malignant cartilaginous tumor
The peak incidence is in men 30 to 60 years of age
In its conventional form, not found in children
Characteristic sites of origin include the pelvis, spine,
or scapula; the proximal humerus or proximal femur;
and femur or tibia near the knee.
Mesenchymal chondrosarcomas occur in younger
patients and may present as a bone tumor but is
often extraskeletal
Gross:
It is a well defined lobulated cartilage mass with
stippled calcification and endochondral
ossification
Histopathology:
It shows a cartilaginous pattern lobular pattern
limited to surface and rarely infiltrating cortex of
the bone
Areas of spotty calcification and endochondral
ossification is seen