Cartilagenous Tumors of Bone

D r. A t h m a r a m. M,
athmaramadisesh@yahoo.com
Government Medical College ,
Anantapur.

There is confusion among many about cartilaginous tumors and cartilage

forming tumors. When I was requested to deliver this lecture, I came to
understand from the postgraduates that they wanted me to cover all the
cartilage forming tumors rather than the cartilaginous tumors of bone.
Broadly the cartilage forming tumors can be classified as follows:
• Benign:
o Solitary Osteocartilaginous Exostosis or Solitary Osteochondroma
o Multiple Exostosis or Dyaphyseal Aclasis
o Solitary Enchondroma
o Juxtacortical (periosteal) Chondroma (Enchondroma)
o Multiple enchondromatosis (Ollier’s Disease)
o Chondroblastoma
o Chondromyxoid Fibroma
• Malignant:
o Chondrosarcoma
 Spindle celled
 Myxoid
 Mesenchymal

I intend to confine my lecture on the main points rather than routine

statistics of incidence, age, region and common mode of treatment.

Osteochondroma:

Def: An osteochondroma is a cartilage capped bony protrusion on external

surface of a bone.

First described by Sir Astely Cooper,(surgeon and anatomist at St. Thomas

Guy’s Hospital, London in 1818) is the commonest benign bone tumor. This
is usually a painless swelling. Pain can be caused by various factors as
follows;
• Irritation and pressure on surrounding structures
• Osteonecrosis of the osseous component (Infracted Osteochondroma)
• Perilesional bursal inflammation and swelling
• Pseudoaneurysms due to pressure on adjacent major vessels
• Fracture thru the base of stalk (Commonest cause of pain)
• Kyphosis (if the location is in spine)

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 • Spondylolisthesis if it is attached to intervertebral joint
• Fascial asymmetry and masticatory dysfunction in lesions in head and
neck
• Malignant transformation
Spontaneous resolution of these lesions, are well documented. They can be
due to
• cessation of lesional growth,
• incorporation of lesion by appositional growth into adjacent cortex,
• termination of cartilaginous cap and active resorption,
• loss of blood supply due to the fracture.

Pathogenesis: Osteochondromas form from bones developing from cartilage

like all the long tubular bones. They arise on the cortex next to epiphysis
from zone of endochondral growth. It rotates and moves towards shaft away
from the epiphysis. At the site of origin cortex is absent and the bone of
osteochondroma merges into the underlying spongy bone.

Hypotheses on mode of development:

Lichtenstein proposed anomalous periosteal development that produces
displaced nests of cartilage. Continued stimulation of these foci along with
endochondral bone growth forms an exostosis. This theory was proved by
reproducing these lesions by implanting epiphyseal cartilage next to
epiphysis towards metaphysis in rabbits.
These can also be caused due to irradiation in children – well documented.
Osteochondromas are considered by many a perversion in the direction of
bone growth, not a true neoplasm but rather an enchondromatous
hyperplasia, because the lesion is formed by endochondral ossification and
the bone substance produced is in every way normal. A congenital anomaly
of the diaphyseal blood vessels is postulated as being responsible for the
development of osteochondromas.

The four most popular hypotheses include – origin in epiphyseal growth

plate, origin in the periosteum, origin in the perichondral groove of Ranvier
(which surrounds the growth plate) or a defect at the periphery of the growth
plate in the perichondral groove.

The common sites of occurrence are familiar to all. Apart from the ends of
long bones they are seen in scapula, ribs, innominate bone, mandibular
condyle, coronoid process, tip of spinous process, vertebral arch, pedicle,
costovertebral joint, subungual (below nails) and as ivory exostosis on
craniofascial bones. Bilateral sessile osteochondromas were found in
external auditory canal in cold water swimmers! Intra articular
osteochondroma like lesions are demonstrated in femoral neck in Perthese
disease. Wide based lesions in pelvis undergoing sarcomatous change are
termed as Epiexostotic Chondromas. Some lesions can occur in the capsule

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of the knee – periarticular chondroma or osteochondroma – as nodules
deeply embedded in the synovium. They can present as a single large loose
body or multiple small synovial chondromatosis. In the early stage the
process is intra-synovial without any loose bodies. Later in the advanced
stage, free osteochondral bodies appear in the joint cavity.
DD –
Peripheral Chondrosarcoma, Juxtacortical osteosarcoma, Myositis ossificans
Epiphyseal Dysplasia – Dysplasia epiphysealis hemimelica – Trevors Disease
(DD in distal femur and tibia) In Trevors disease isolated irregular
centers of ossification form on one side of epiphysis of involved articular
segment. In later stages these separate ossification centers fuse together,
resulting in symmetrical epiphyseal enlargement causing limb length
discrepancy and deformities.
In any case of the cartilage cap is irregular and more than few millimeters
thick, it should be suspected as a Chondrosarcoma. Lichtenstein considered
1 cm as the upper limit.

X-rays: flat sessile / plateau like / with a stalk / Kleiderhaken appearance

(tubular appearance ending in a hook). Malignant transformation is
suspected when the demarcation is interrupted and a soft tissue mass is
present with streaky and blotchy radio-opacity is seen. Calcifications are
seen as fluffy amorphous opacities. If there is an end-on view, they can be
mistaken for enchondromas.

Multiple Osteocartilaginous Exostosis ( Hereditary

multiple Exostosis, Diaphyseal Aclasis):

Boyer described this condition originally in 1814. It is inherited by single

autosomal dominant gene. Half the progeny of affected parent express the
trait. Keith called it diaphyseal aclasis. Many divergent theories are
postulated in the formation. Virchow and Von Recklinghausen postulated
that both exostosis and enchondromas are derived from the cartilaginous
growth plates. Thus, small portions of cartilage may become separated from
the lateral aspect of the epiphyseal plate and make a right angle turn shaft
wards, establishing the onset of an exostosis.
Miller postulated that exostosis originated in nests of cartilage arising from
the disordered osteogenic layer of the periosteum. Keith postulated that it is
related to the defective development of periosteal ring surrounding the
growth plate. He said that defective deposition of bone in this area permits
the expansion of cartilage preformed bone to grow in anomalous directions
precluding normal osseous modeling. This unrestrained growth of bone at
the epiphyseal plate ‘runneth over’ onto the outer surface of the bone
constituting the exostosis.
This condition manifests during childhood. New exostoses do not form in
adults. Shortness of stature is manifested. Unequal growth, of bones causes
deformities in forearm. Tibio fibular growth discrepancies cause deformities

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of knee and ankle. Ribs, pelvis and scapula are most frequently affected
areas. In multiple variety, they are common in distal ends of proximal and
middle phalanges.

Solitary Enchondroma:

Def: Solitary enchondroma, also known as central chondroma is a benign

hyaline cartilage growth that develops within the medullary cavity of a single
bone.

Pathogenesis: It evolves from heterotrophic cartilage rests found frequently

in otherwise normal metaphyses. These cartilage cells giving raise to the
lesion may be embryonic rests derived from epiphyseal cartilage plate. Thus
an enchondroma is produced by disordered endochondral bone formation
that results in occurrence of a cartilage tumor in the metaphyseal and
diaphyseal regions of the shaft. They arise during the growth period and
manifest in third or fourth decade.

They are usually asymptomatic, cause fusiform shape of the phalanx. Local
trauma can cause a pathological fracture and cause severe pain. If there is
pain without trauma and pathological fracture, we have to suspect malignant
transformation. Lesions at the base of skull or vertebrae may cause
neurologic symptoms. In the vertebral body it has to be differentiated from a
Schmorl’s node. 35% arise in the hand. If it is central, it is called an
Enchondroma. If it is peripheral it is cammed a ‘periosteal’ of ‘juxtacortical’
enchondroma. Caballes called these peripheral lesions as “Enchondroma
Protruberans”. Lesions in long and flat bones are rare. If present in long or
flat bones they have increased propensity for malignant transformation. This
is the commonest tumor in the patella, nasal cavity and sinuses.

They are easy to diagnose on x-rays with a well demarcated edge, central
lesion, no periosteal reaction, paper thin cortex and clear center. There can
be a calcifying enchondroma in large lesions with central calcifications.
Grossly, they are coarsely lobular or nodular myxomatous structure with
bluish opalescence due to the hyaline cartilage. Calcification causes
grittiness of the lesion.
The transformation of a benign enchondroma to a Chondrosarcoma has been
traced and well documented histologically. The pathologist’s tendency to
under diagnose chondrosarcomas, especially on limited biopsy material,
makes evaluation of this contention rather difficult in most cases. The
beginning of persistent unrelenting pain or cortical penetration by the tumor
withour apparent trauma are foreboding signs.

Indications for surgical removal of enchondromas as per Memorial Solan-

Kettering Cancer Institute – all enchondromas arising in the long bones or in
the pelvis and shoulder girdles showing  renewed, previously stable

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growth; persistent unrelenting pain; x-ray signs of cortical destruction
without trauma, poorly defined margin and focal periosteal reaction.

Juxtacortical Enchondroma:

This is a benign cartilage growth beneath the periosteum and external to the
bone. These are common in long bones like humerus and ribs.
Pathogenesis: Unlike enchondromas, these lesions are not related to areas of
cartilaginous growth plates, but are probable produces by sub-periosteal
cartilage formation. They form and continue to grow past skeletal maturity.
They are rarely seen in the hyoid bone.
X-rays show clear bubble like lesion on the cortex with a rim of reactive new
bone and bone deposition beneath the saucer like cortical erosion. They
have to be differentiated from Pigmented villonodular tenosynovitis,
nonspecific tenosynovitis, ganglion, glomus tumor, osteochondroma and
Chondrosarcoma on the x-rays.

Multiple Enchondromatosis (Ollier’s Disease):

(Ollier – French physician from Lyon). Skeletal enchondromatosis is a

cartilage dysplasia of one representing an inborn anomaly of osseous
development, a developmental error in endochondral calcification.

Pathogenesis: Jaffe postulated that cartilage derived from cambium layer of

periosteum penetrated the shaft of involved long bone and is distributed
throughout. These unossified remnants of cartilage in metaphysis and
diaphysis may evolve to form large tumors. There is no evidence of generic
factor. The association of Ollier’s disease with a functioning ovarian tumor
has been reported.

Bones of hands are most commonly involved. They also involve the flat
bones, skull, femur and tibia. They cause deviation of proper growth,
deformities with eventual brachydactyly. This condition is compatible with
long life. Transformation into Chondrosarcoma is very common. Incidence of
malignant transformation according to Mayo clinic data is 30% and 50%
according to Jaffee. Secondary malignance can also be a chordoma and
ostoegenic srcoma.

Differential diagnosis – Metachondromatosis (described by Maroteaux in

1971) mimics multiple enchondromatosis. It is differentiated as it is
charecterised by multiple enchondromas in iliac crests and metaphyses of
long bones along with periarticular multiple joint calcification and
ossification. This is an autosomal dominant condition and there are multiple
osteocartilagenous exostoses in digits and long bones which point towards
the joint and disappear spontaneously.

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 Maffucci’s Disease:

Described by Maffuci in 1881, also called Maffuci-Kast syndrome.

Enchondromas are associated with soft tissue hemangiomas. 50% lesions
are unilateral. Malignant transformation rage is very high upto 19%.
Although a low grade Chondrosarcoma is the likeliest outcome in a patient
with Maffuci’s syndrome, occasionally some patients may have a fully
malignant osteogenic sarcoma or other non-osseous malignant tumors like
pancreatic or biliary adenocarinoma or an astrocytoma. It is noted that a
Chondrosarcoma arising within the complex of Maffuci’s located in the
craniofascial bones may be associated with a carotid body tumor in the same
patient. Bilateral fibroadenomas are also seen in females affected with
Maffuci’s.

The radiographic features are identical with the lesions in Olliers disease. In
some cases, numerous phleboliths can be seen in the accompanying
hemangiomas. On arteriographic study, multiple arteriovenous
malformations may be noted.

Chondroblastoma:

Chondroblastoma is a rare, primary, usually benign bone tumor of immature

cartilage cell derivation with preferential localization in the epiphysis.

Kolondy in 1927 called it – giant cell tumor variant, a cartilage containing

giant cell tumor.
Ewing in 1928 called it – calcifying giant cell tumor.
Codman in 1931 described it in detail – epiphyseal chondromatous giant cell
tumor.
Jaffe and Lichtenstein called it – benign chondroblastoma.

Also known as Codman tumor, representing, less than 1% of all primary bone
tumors, is a benign lesion occurring before skeletal maturity,
characteristically presenting in the epiphyses of long bones such as the
humerus, tibia, and femur. Although secondary involvement of the
metaphysis after skeletal maturity is recognised, a predominantly
metaphyseal or diaphyseal location is exceedingly rare. Equally unusual is
involvement of the vertebra or intracortical location in the long bones.
Occasionally patella, which is considered equivalent to an epiphysis, is
affected. Buttress type of periosteal reaction is seen.
Histogenesis: Proposed by Jaffe and Lichtenstein – it is a primary tumor of
bone that developed from cartilage germ cells. Tissue culture studies showed
that the cell of origin was of histiocytic derivation.
Non specific pain and wasting of muscles are common. Pathological fracture
is rare. May present like early arthritis. Secondary synovial reaction with

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synovitis and effusion is common. They often straddle the adjacent epiphysis
and metaphysis. Exclusive epiphyseal lesions are smaller than the ones
extending on to the metaphysis. Axial and appendicular skeleton is equal not
sparing the tarsal, Innominate or the sacrum. Lesions can also occur in the
carpal and tarsal bones, bones of skull, mandible and are common in the
calcaneum.

On gross examination the curetted tissue shows soft or friable mottled

grayish material that appears to be focally gritty, grey yellow to grey brown.
Mictoscopically the pericellular lattice like fine calcification attanged in the
characteristic “chicken wire” or “picket fence” pattern is the hall mark of this
lesion. The evolutionary progression in chondroblastoma as studied by light
microscopy is depicted as –
Earliest Phase – compact round or polyhedral cells of moderate size. Large
nuclei, occasionally more than one nucleus with a sprinkling of
multinucleated giant cells.
Intermediate Phase – focal areas of calcification. The heavier the
calcification, the heavier the degeneration and necrosis of tumor cells.
Late Phase – connective tissue replacement of necrotic tissue. Chondroid or
even osseous matrix formation. S-100 protein immunostaining provides help
in histologic differential diagnosis of chondroblastoma.
Curettage with intra-operative implantation often results in clinical soft tissue
recurrence or tumor invasion of adjacent bones. Synovial membrane and
articular recurrences follow transarticular curettage, thereby emphasizing
the importance of avoiding entering the adjacent joints. Long standing,
neglected lesions may extend spontaneously into adjacent joints, articular
surfaces and soft tissues, necessitating major ablation. All these lesions,
although histologically benign, did indeed exhibit aggressive but self-limited
clinical behavior.

Chondromyxoid fibroma:

This is a benign tumor of bone characterized by chondroid and myxoid

differentiaton of its basic tissue growing in a lobular pattern.

Radiologically it is an eccentric round or oval lesion in the metaphysis of a

long bone. It has a well defined sclerotic margin and hemispheric “bite-like”
cortical destruction. If it appears after cessation of skeletal growth, sub-
articular location is a rule. In some planes the external shell is difficult to
outline if there is no sup-periosteal new bone formation. There are lot of
pseudo-trabeculations. Thus the differential diagnoses will be a giant cell
tumor, eccentric aneurismal bone cyst, unicameral bone cyst, non-ossifying
fibroma, chondroblastoma and fibrous dysplasia.
Curettage without grafting has a recurrence rate of 15%. With curettage with
grafting the recurrence rates dip steeply.

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 Malignant cartilage tumor – Chondrosarcoma:

Chondrosarcoma is a malignant tumor in which the basic neoplastic tissue is

fully developed cartilage without tumor osteoid being directly formed by a
sarcomatous stroma. Myxoid changes, calcification, or ossification may be
present.

Excluding multiple myeloma, chondrosarcoma is second to osteogenic

sarcoma in frequency as a malignant tumor of bone.
Types – Nomenclature –
Primary chondrosarcoma arises de novo in bone.
Secondary chondrosarcoma develops from a pre-existing benign cartilage
tumor like – enchondroma, cap of osteocartelagenous exostosis, juxtacortical
chondroma, Ollier’s disease and Maffuci’s disease.
These can be central, peripheral, juxta-cortical and rarely periosteal.
Pain, tenderness are vital symptoms but absent in pelvic lesions. These
present with symptoms of pressure effects on nerves or vessels.
On x-rays, central lesions demonstrate large thick walled areas of
radiolucency with trabeculation and central areas of multilocular medullary
bone destruction. Foci of irregularly scattered spotty or blotchy calcification
represented by densities throughout the lesion are noted. These
calcifications have also been described as appearing as ‘fluffy’, ‘cotton wool’,
‘popcorn’, ‘breadcrumb’ configuration. Some lesions show thinning and
bulging expansion of cortex, of a cystic radiolucent appearance may even be
presented. Sometimes if the lesion is extending all along the shaft, with
periosteal new bone formation it mimics Pagets disease.
Peripheral lesions are faintly visible, with sparsely calcified shadows in the
soft tissues next to the lesion, with radiating spicules or large speckles
arranged at fight angles to the cortex. Adjacent periosteum may be elevated.
No medullary involvement is demonstrated and the cortex is rarely affected.
Codman’s triangle is seen in early stages due to the elevation of the
periosteum.
Secondary chondrosarcomas from central lesions (enchondroma) show the
original lesion and the superimposed malignant change is characterized by a
fuzzy infiltrating periosteal activity. Once the lesion is in an advanced stage,
the entire lesion becomes irregularly and coarsely granular, and infiltration
with scattered foci of ossification is noted. The adjacent soft tissues show
evidence of stippled calcification. Such central lesion should be differentiated
from bone infract before jumping into this malignant diagnosis. In peripheral
lesions (from exostosis) the progressive gradual erasure of the smooth
outlines of a cartilaginous cap from the periphery inward toward the cortex
and a soft tissue mass with foci of irregular calcific speckles represent fairly
good evidence that malignant transformation had taken place.
Histologic grading of chondrosarcoma –

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Conventional chondrosarcoma is separated into three histological grades:
low (grade 1), intermediate (grade 2), and high (grade 3). Grading is highly
subjective and based on relative degrees of cellularity, atypia, and
pleomorphism.
Low-grade tumors have mild hypercellularity and mild cellular atypia. Nuclei
are small and dense. The cartilage cells are present in lacunae, which may
be enlarged, and binucleate cells are sparse. Only rarely is there myxoid
material; the material is abundant and obviously chondroid.
Intermediate chondrosarcomas have significant atypical, increased cell
numbers, correspondingly less matrix, and the nuclei often enlarged. The
incidence of bi-nucleate cells is increased, and multinucleated cells are seen.
Rare mitoses may be found. These features usually are especially prominent
at the periphery of tumor lobules. Occasional microscopic areas of necrosis
may be found.
Grade 3 lesions are characterized by marked hypercellularity and extreme
plemorphism with bizarre giant-cell tumors and mitoses. The cartilage cells
usually are not located in lacunae. Myxomatous change often is prominent,
and foci of necrosis almost are invariably present. Evans et al reported that if
there are >2 mitoses per 10 high-power fields, the tumor is most likely high
grade.
Subtypes of chondrosarcoma such as dedifferentiated or mesenchymal have
a poor prognosis and are beyond the scope of this review. Clear-cell
chondrosarcoma is another rare variant that resembles chondroblastoma in
its presentation, and is much less biologically aggressive than the other
mentioned subtypes.
Lee et al monitored 227 patients with chondrosarcoma for an average of 6
years and reported predictive factors for metastasis to be local recurrence;
pelvic location, tumor volume greater than 100 cc, aneuploidy of the tumor
coupled with a high mean DNA index, histologic grade 3, and
dedifferentiated chondrosarcomas. The reported recurrence rate ranges from
4-12%, depending on initial treatment modality and tumor grade
Classically, because chondrosarcomas are known to be resistant to
radiotherapy and chemotherapy, wide resection has generally been accepted
as recommended treatment for low-grade chondrosarcoma. Borderline
cartilage tumors have an even lower metastatic potential than the true low-
grade (grade 1) malignant chondrosarcoma. Thus, recommended treatment
of these borderline tumors has ranged from marginal curettage and grafting
to limited local resection. There has been a push for more conservative
treatment of borderline and low-grade malignant chondrosarcomas, with
extended intralesional curettage being considered a viable alternative to
wide resection.

I encourage the postgraduate to build up his answers based on these finer

points by filling up details like incidence, common bones, and treatment
options and latest surgical, chemo and radio-theraputic options for each
tumor.

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