Cartilage Forming Tumors Cartilage Forming Tumors

8/3/2019 Cartilage Forming Tumors Cartilage Forming Tumors

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CCARTILAGEARTILAGEFFORMINGORMING

TTUMORSUMORS


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CCARTILAGEARTILAGE FFORMINGORMING TTUMORSUMORS

Benign:

y Chondroma

y Osteochondroma

Maligna:y Chondrosarcoma


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CCHONDROMAHONDROMA


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CCHONDROMAHONDROMA

Benign and asymptomatic cartilaginous tumor ofbone which most often occurs in adolescents oryoung adults;

It is an intramedullary cartilage lesion located incentral metaphysis;

Intramedullary tumor develops in adjacentmetaphysis and may eventually penetrates thediaphysis;


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Results from failure of normal endochondral

ossification below growth plate & represents a

dysplasia of the central growth plate;

y

If dysplastic process occurs in lateral growth plate,resulting tumor is called osteochondroma;

y Dysplastic cartilaginous proliferation beneath the

perichondrium results in periosteal chondroma;


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SSITESITES OFOF INVOLVEMENTINVOLVEMENT::

Hand:

y The most common benign skeletal lesions of the hand

y Most often affects small tubular bones of hands or

feet (40-65%);

y It is a frequent cause of pathologic fracture;

May also affect proximal humerus and femoral

shaft;


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RRISKISKOFOF TRANSFORMATIONTRANSFORMATION::

Enchondroma usually becomes latent in adulthood,

& < 2% of asymptomatic solitary enchondromas

will transform to chondrosarcoma

Enchondromas of long bones have higher chance

for malignant transformation;

Risk of malignant transformation is about 10-25%;

Maffucci disease (multiple enchondromas andhemangiomas) risk is near 100%;


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EXAMINATION:

Enchondroma does not cause symptoms unless

there is pathologic frx;


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HISTOLOGY:

Lobules of different sizes separated by small

septa of fibrous tissue;

May see uniform cartilage cells with dark round

nuclei & some binucleate nuclei (which isnormal);

Often it is difficult to differentiate an

enchondroma from a low grade chondrosarcoma


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RADIOGRAPHS:

Central radiolucent lesion w/ well defined miminallythickened bony margin;

During active phase in adolesence, the lesion mayslowly enlarge.

In children, cortex is usually thin and the lesion isradiolucent;

Later, intralesional calcifications appear;

There is no periosteal reaction;

In latent phase, cartilaginous tissue may calcify w/diffuse punctate pattern;

As lesion matures, it deveolops a reactive margin;


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RADIOGRAPHIC FINDING


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OLLIERS DISEASE:

MULTIPLE

ENCHONDROMA.


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BONE SCANS:

Demonstrate radioisotope uptake in margin,

related to activity of lesion;

While there is moderate uptake in the acitive

phase there will also be some activity in latentphase;


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TREATMENT:

Asymptomatic solitary enchondromas may be

followed non operatively

Prognosis for benign enchondroma is excellent.

If solitary or multiple enchondromas becomesymptomatic or begin to enlarge, they may

require biopsy to r/o malignancy (the terrible

triad: pain, increase radioisotope uptake on bone

scan, & destructive changes on x-ray);


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PPATHOLOGICATHOLOGIC HANDHAND ANDAND

FINGERFINGER FRACTUREFRACTURE::

Most cases the fracture is allowed to heal with

closed treatment;

Curettage and bone grafting is then required

after fracture healing;


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EXCISION:

Tumor is excised with a wide margin to reduce

recurrance


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OOSTEOCHONDROMASTEOCHONDROMA(C(CARTILAGEARTILAGE--CCAPPEDAPPED EEXOSTOSISXOSTOSIS))


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OOSTEOCHONDROMASTEOCHONDROMA

Adevelopmental dysplasia of peripheral growth

plate which forms a cartilage capped projection of

bone found near metaphyses of long bones;


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Peripheral chondroblast grows outward from the

metaphysis, acting as an ectopic growth plate,

which ceases growth at skeletal maturation;

An excrescene of trabecular bone capped by athin zone of proliferating cartilage;

The most common benign bone tumor;

Usually occurs in long bones, but may occur any

bone that is preformed in cartilage;


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DDIFFERENTIALIFFERENTIAL DDIAGNOSISIAGNOSIS::

Multiple cartilaginous exostoses

y Patients have polyostotic tumors

y Look for short stature, clubbing of radius, & angular

deformity of the lower limbs;

y Have an increased risk for secondary chondrosarcoma

after the age of 30 years;

Parosteal osteosarcoma

y May present as a symptomatic "exostosis" that

increases in size in adults;


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TTUMORUMOR GGROWTHROWTH::

Lesion growths by enchondral ossification of proliferatingcartilage cells in its cap;

Tumor will continue to enlarge during skeletal growth, butwill become latent at skeletal maturity;

y However, the lesion may continue to grow into the 3rd decade;

y Occcassionally a lesion grows more rapidly than expected;

Most common locations are proximal or distal femur,proximal humerus, proximal tibia, pelvis, and scapula;

y In areas other than the knee, more likely to undergoemalignant degeneration;

y May occur in the spine and cause neurologic damage;


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MMALIGNANTALIGNANT TTRANSFORMATIONRANSFORMATION::

Risk of sarcomatous transformation in solitary

exostosis is about 1%, but in MHE (Multiple

Hereditary Exostosis), risk approaches 10%;

Evidence for transformation: (to chondrosarcoma)y Cartilaginous cap thicker than 1 cm in an adult (in child

may be 2-3 cm thick) as seen by MRI;

y Cudden or marked increase in uptake on bone scan in

an adult

y

Confirmation by CT or MRI imaging of a soft tissuemass or displacement of a major neurovascular bundle;


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CCLINICALLINICAL PPRESENTATIONRESENTATION::

Look for a firm, nontender, immovable mass

arising near end of the long bone;

Asymptomatic lesion, may be caused by

irritation of overlying soft tissues which may goon to form a fluid filled bursa;

Bursal fluid may be mistaken for a soft tissue

mass;


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DDIAGNOSTICIAGNOSTIC STUDIESSTUDIES::

X-ray appearance of an exostosis is either flat,sessile lesion or a peduculated (stalk like)process;

Peduncultaed osteochondromas are oriented in

proximal direction; X-ray hallmark is blending of tumor into

underlying metaphysis;

Look for a well defined metaphyseal excrescenceof bone w/ a mottled density;

Calcification:y Cartilaginous cap displays irregular areas of

calcification;

y Amount of calcification and bone formation increase w/age


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RADIOGRAPHIC FINDING


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OsteochondromaOsteochondroma(4D USG)(4D USG)


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MMICROSCOPICICROSCOPIC EEXAMXAM::

Cartilaginous cap is seen to have same pattern asnormal growth plate but it will be less organized;

Underlying trabeculae form by endochondralossification of cap and contain central cores of calcifiedcartilage.

Cartilage cells w/ small round or elongated nucleiwhich may be positioned in rows similar to a physis;

Polymorphy and hyperchomasy of cartilage cells is anexpected finding in young children;

Cartilagenous cap may be upto 1 cm in width inadolescence and that a cap greater than 3 cm is

consistent w/ low grade chondrosarcoma;


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OsteochondromaOsteochondroma microscopy:microscopy:


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TTREATMENTREATMENT::

No treatment is required if the diagnosis is not in

doubt and if the patient is relatively

asymptomatic;

Surgical resection is indicated forpersistantirritation (from bursitis) or for neurovascular

comprimise;

Surgical resection is also indicated for continued

osteochondroma growth after skeletal maturity

(in which case malignancy is suspected);


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TTREATMENTREATMENT::

Definitive treatment includes marginal excisionof an active exostosis, including the cartilaginouscap & overlying perichondrium;

Deep bony base has minimal activity and may be

removed piecemeal The cartilaginous cap should not be traumatized

during its removal;

Prognosis for a solitary exostosis is excellent (

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