Bone tumors and tumor-like lesions:

Osteosarcoma, Osteoid Osteoma,

Osteochondroma, and Nonossifying
Fibromas
by Emily Maambo

Introduction
Bone tumors are diverse in their gross
and morphologic features and range
from benign to rapidly fatal. This
diversity makes it critical to accurately
diagnose and stage tumors.
Timely, accurate diagnosis allows
appropriate treatment so that the
patients can not only survive, but also
maintain optimal function of the
affected body parts.

Classification
Most bone tumors are classified according to the
normal cell or tissue of origin. Lesions that do not have
normal tissue counterparts are grouped according to
their distinct clinicopathologic features.
Overall, matrix-producing and fibrous tumors are the
most common. Among the benign tumors,
osteochondroma and fibrous cortical defect are most
frequent. Excluding malignant neoplasms of marrow
origin, osteosarcoma is the most common primary
cancer of bone, followed by chondrosarcoma and
Ewing sarcoma.

Epidemiology
The precise incidence of different bone tumors is
not known because many benign lesions are not
biopsied. Benign tumors outnumber malignant
tumors by at least several hundredfold.
Benign tumors have their greatest frequency within
the first three decades of life, whereas malignant
tumors are much more common in the elderly.
In the United States, about 2,100 new cases of bone
sarcoma are diagnosed annually, and approximately
1,300 deaths from bone sarcoma occur each year.

Clinical presentation
Clinically, bone tumors present in various
ways. The more common benign lesions
are frequently asymptomatic and are
detected as incidental findings. Many
tumors, however, produce pain or are
noticed as a slow-growing mass.
Sometimes, the first hint of a tumor's
presence is a sudden pathologic fracture.

Diagnosis
Radiographic analysis plays an
important role in diagnosing bone
tumors. In addition to providing
the exact location and extent of
the tumor, imaging studies can
detect features that help limit the
differential diagnosis and give
clues to the aggressiveness of the
tumor. Ultimately, in most
instances, biopsy and histologic
study are necessary.

Osteosarcoma
Osteosarcoma is defined as a malignant mesenchymal
tumor in which the cancerous cells produce bone
matrix. Osteosarcomas occur in all age groups but
have a bimodal age distribution; 75% occur in patients
younger than age 20.
In adolescence, and about half of them arise in the
metaphysis around the knee, either in the distal femur
or proximal tibia. These are the sites of greatest
skeletal growth activity. In persons over age 25, the
incidence in flat bones and long bones is almost equal.

Major sites of origin of osteosarcomas. The

numbers are approximate percentages.

Osteosarcoma
Osteosarcomas typically present as painful
and progressively enlarging masses.
Sometimes a
sudden fracture of the bone is the first
symptom.
Grossly, osteosarcomas are bulky tumors that
are gritty, gray-white, and often contain areas
of hemorrhage and cystic degeneration. The
formation of bone by the tumor cells is
characteristic of osteosarcoma.
Osteosarcoma of the upper end of the
tibia. The tan-white tumor fills most of
the medullary cavity of the metaphysis
and proximal diaphysis. It has infiltrated
through the cortex, lifted the periosteum,
and formed soft tissue masses on both
sides of the bone.

Osteosarcoma
Radiographs of the primary
tumor usually show a large,
destructive, mixed lytic and
blastic mass. The tumor
frequently breaks through the
cortex and lifts the periosteum,
resulting in reactive periosteal
bone formation. The triangular
shadow between the cortex and
raised ends of periosteum is
known radiographically as
Codman triangle and is
characteristic, but not
diagnostic of this tumor.

Distal femoral osteosarcoma with prominent bone formation

extending into the soft tissues. The periosteum, which has
been lifted, has laid down a proximal triangular shell of
reactive bone known as a Codman triangle (arrow).

Osteosarcoma

Central osteosarcoma. A, A destructive lesion is seen in the metaphysis on this anteroposterior view of the
knee in a young teenager with pain. B, A magnetic resonance scan of both legs shows the soft tissue extent
of the tumor (arrows).

Osteoid Osteoma
Osteoid osteomas are bone tumors less than 2 cm in greatest
dimension and usually occur in patients in their teens and
twenties. In fact, 75% of patients are under age 25.
Osteoid osteomas can arise in any bone but have a
predilection for the appendicular skeleton. 50% of cases
involve the femur or tibia, where they commonly arise in the
cortex.
Osteoid osteomas are painful lesions. The pain is caused by
excess prostaglandin E2 which is produced by the
proliferating osteoblasts. It characteristically occurs at night
and is dramatically relieved by aspirin.

Osteoid Osteoma
Osteoid osteoma. A lateral
view (A) of the proximal tibia
shows a very dense lesion in
the posterior cortex. A darker
central area contains a white
nidus. This lesion in a 20-yearold man caused pain in this
area, relieved by aspirin. B, A
nuclear medicine bone scan in
a different patient with an
osteoid osteoma in the left
lower tibia shows increased
activity (arrows) at the site of
the lesion.

Osteoid Osteoma

Specimen radiograph of intracortical osteoid

osteoma. The round radiolucency with central
mineralization represents the lesion and is
surrounded by abundant reactive bone that has
massively thickened the cortex.

Osteoid osteomas,
especially those that arise
beneath the periosteum,
usually elicit a tremendous
amount of reactive bone
formation that encircles
the lesion. The actual
tumor, known as the
nidus, manifests
radiographically as a small
round lucency that is
variably mineralized

Osteoid Osteoma
Osteoid osteomas are considered benign
and are normally treated by conservative
surgery. However there is a possibility of
malignant transformation. This is rare
except when treated with radiation, which
promotes this complication.

Osteochondroma
Osteochondroma, also known as an exostosis, is a
benign cartilage-capped outgrowth that is attached to the
underlying skeleton by a bony stalk. It is a relatively
common lesion and can be solitary or multiple.
Multiple osteochondromas become apparent during
childhood but solitary osteochondromas are usually not
diagnosed until late adolescence .
Men are affected 3X more often than women.
They arise from the metaphysis near the growth plate of
long tubular bones, especially the knee.

Osteochondroma
Clinically,
osteochondromas present
as slow-growing masses,
which can be painful if
they impinge on a nerve or
if the stalk is fractured. In
many cases, they are
detected as an incidental
finding.
Osteochondroma. On this lateral view of the ankle, a
benign osteochondroma is seen projecting posteriorly
on a stalk. The end (arrows) is often covered with a
cartilaginous cap. These lesions always occur near a
joint but point away from it.

Fibrous Cortical Defect and

Nonossifying Fibroma
Fibrous cortical defects are extremely common,
found in 30% to 50% of all children older than age
2 years. They are believed to be developmental
defects rather than neoplasms.
The vast majority arise in the metaphysis of the
distal femur and proximal tibia, and almost one half
are bilateral or multiple.
Fibrous cortical defects are small and those that
grow to 5 or 6 cm in size are called nonossifying
fibromas.

Fibrous Cortical Defect and

Nonossifying Fibroma
Fibrous cortical defects are asymptomatic and are
usually detected on x-ray as an incidental finding.
The vast majority have limited growth potential
and undergo spontaneous resolution within several
years.
The few that progressively enlarge into
nonossifying fibromas usually show up in
adolescence. They may present with pathologic
fracture and then require biopsy to exclude other
types of tumors.

Fibrous Cortical Defect and

Nonossifying Fibroma
Both fibrous cortical
defects and nonossifying
fibromas produce
elongated, sharply
demarcated radiolucencies
that are surrounded by a
thin zone of sclerosis.

Nonossifying fibromas of the distal tibial

metaphysis, producing an eccentric lobulated
radiolucency surrounded by a sclerotic margin.

References

Kumar: Robbins and Cotran: Pathologic Basis of Disease, 7th ed.,

Copyright 2005 Saunders, An Imprint of Elsevier

Mettler: Essentials of Radiology, 2nd ed., Copyright 2005 Saunders, An

Imprint of Elsevier