CARTILAGE FORMING TUMORS

CARTILAGE FORMING TUMORS

Benign:
Chondroma y Osteochondroma
y

Maligna:
y

Chondrosarcoma

CHONDROMA

CHONDROMA
Benign and asymptomatic cartilaginous tumor of bone which most often occurs in adolescents or young adults; It is an intramedullary cartilage lesion located in central metaphysis; Intramedullary tumor develops in adjacent metaphysis and may eventually penetrates the diaphysis;

Results from failure of normal endochondral ossification below growth plate & represents a dysplasia of the central growth plate;
If dysplastic process occurs in lateral growth plate, resulting tumor is called osteochondroma; y Dysplastic cartilaginous proliferation beneath the perichondrium results in periosteal chondroma;
y

SITES OF INVOLVEMENT:

Hand:
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;
y

May also affect proximal humerus and femoral shaft;

RISK OF TRANSFORMATION:

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 and hemangiomas) risk is near 100%;

EXAMINATION:

Enchondroma does not cause symptoms unless there is pathologic frx;

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 is normal); Often it is difficult to differentiate an enchondroma from a low grade chondrosarcoma

RADIOGRAPHS:

Central radiolucent lesion w/ well defined miminally thickened bony margin; During active phase in adolesence, the lesion may slowly enlarge. In children, cortex is usually thin and the lesion is radiolucent; 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;

RADIOGRAPHIC FINDING

OLLIERS DISEASE: MULTIPLE ENCHONDROMA.

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 latent phase;

TREATMENT:
Asymptomatic solitary enchondromas may be followed non operatively Prognosis for benign enchondroma is excellent. If solitary or multiple enchondromas become symptomatic 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);

PATHOLOGIC HAND AND FINGER FRACTURE:

Most cases the fracture is allowed to heal with closed treatment; Curettage and bone grafting is then required after fracture healing;

EXCISION:

Tumor is excised with a wide margin to reduce recurrance

OSTEOCHONDROMA
(CARTILAGE-CAPPED EXOSTOSIS)

OSTEOCHONDROMA

A developmental dysplasia of peripheral growth plate which forms a cartilage capped projection of bone found near metaphyses of long bones;

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 a thin 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;

DIFFERENTIAL DIAGNOSIS:

Multiple cartilaginous exostoses

y y y

Patients have polyostotic tumors Look for short stature, clubbing of radius, & angular deformity of the lower limbs; Have an increased risk for secondary chondrosarcoma after the age of 30 years; May present as a symptomatic "exostosis" that increases in size in adults;

Parosteal osteosarcoma
y

TUMOR GROWTH:

Lesion growths by enchondral ossification of proliferating cartilage cells in its cap; Tumor will continue to enlarge during skeletal growth, but will become latent at skeletal maturity;
y y

However, the lesion may continue to grow into the 3rd decade; Occcassionally a lesion grows more rapidly than expected;

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

In areas other than the knee, more likely to undergoe malignant degeneration; May occur in the spine and cause neurologic damage;

MALIGNANT TRANSFORMATION:
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 y y

Cartilaginous cap thicker than 1 cm in an adult (in child may be 2-3 cm thick) as seen by MRI; Cudden or marked increase in uptake on bone scan in an adult Confirmation by CT or MRI imaging of a soft tissue mass or displacement of a major neurovascular bundle;

CLINICAL PRESENTATION:
Look for a firm, nontender, immovable mass arising near end of the long bone; A symptomatic lesion, may be caused by irritation of overlying soft tissues which may go on to form a fluid filled bursa; Bursal fluid may be mistaken for a soft tissue mass;

DIAGNOSTIC STUDIES:
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 excrescence of bone w/ a mottled density; Calcification:

y y

Cartilaginous cap displays irregular areas of calcification; Amount of calcification and bone formation increase w/ age

RADIOGRAPHIC FINDING

Osteochondroma (4D USG)

MICROSCOPIC EXAM:

Cartilaginous cap is seen to have same pattern as normal growth plate but it will be less organized; Underlying trabeculae form by endochondral ossification of cap and contain central cores of calcified cartilage. Cartilage cells w/ small round or elongated nuclei which may be positioned in rows similar to a physis; Polymorphy and hyperchomasy of cartilage cells is an expected finding in young children; Cartilagenous cap may be upto 1 cm in width in adolescence and that a cap greater than 3 cm is consistent w/ low grade chondrosarcoma;

Osteochondroma microscopy:

TREATMENT:
No treatment is required if the diagnosis is not in doubt and if the patient is relatively asymptomatic; Surgical resection is indicated for persistant irritation (from bursitis) or for neurovascular comprimise; Surgical resection is also indicated for continued osteochondroma growth after skeletal maturity (in which case malignancy is suspected);

TREATMENT:
Definitive treatment includes marginal excision of an active exostosis, including the cartilaginous cap & 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 (< 5% recurrence following marginal excision);

OSTEOCHONDROMA:

OSTEOCHONDROMA:

CHONDROSARCOMA

CHONDROSARCOMA
Malignant cartilaginous tumor of bone; It is less common and less aggressive than osteosarcoma

STAGING:
Most often presents as a low grade intracompartmental lesion; They are slow to metastasize and may grow to immense proportions; Tumor tends to destroy bone and to extend into the soft tissues; Tumor may invade blood vessels and produce long intravascular tumor bungs that may extend into heart and pulmonary arteries; Long-term survival rate of patients w/ treated chondrosarcoma is between 50-75%.

Primary form
y

Occurs most often in men between 50-60 yrs of age (where as osteosarcoma occurs between 20-30 yrs of age);

Secondary chondrosarcoma:
Occurs in about 25% of cases; y It is a malignant transformation from preexisting enchondroma or osteochondroma
y

LOCATION
Pelvis, proximal femur, & shoulder girdle (where as osteosarcoma occurs most often around the knee); When chondrosarcoma occurs in the pelvis, it is often large, high grade, diagnosed late, and has a poor prognosis;

VARIANTS OF CHONDROSARCOMA: (BASED ON HISTOLOGY & LOCATION)

Dedifferentiated (high grade)

y y y y

Rare type of high grade chondrosarcoma which arises from a low grade chondrosarcoma; A high-grade spindle-cell sarcoma coexists with a lowergrade chondroid tumour; Histologically there will be areas c/w MFH, osteosarcoma, or fibrosarcoma; High risk for metatastasis, only 5% of patients will survive more than 5 years;

J Bone Joint Surg [Br] 2000

Extraosseous Chondrosarcoma:
y y y y y y

Known as extraskeletal myxoid chondrosarcoma; Known also as chordoid sarcoma because of its resemblance to chordoma Accounts for < 2 % of all soft-tissue sarcomas; Tumor usually affects men, in fifth decade of life; Tumor frequently arises in deep soft tissues, esp of lower extremity and frequently the foot Usually present w/ gradually enlarging mass, with or without pain;

Prognosis:
Typically behaves as a low-grade sarcoma; Five-year survival rate has ranged from 65 to 85 %;

y y

Radiographs:

neoplasm appears as nodular, radiolucent mass;

Histology:
Proliferation of ovoid and bipolar cells w/ in prominent myxoid matrix; Individual tumor cells are surrounded by lacunar spaces, but formation of well-developed hyaline cartilage is uncommon;

Clear Cell (Intermediate Grade)

y

Rare, slow growing, locally recurrent tumor easily confused w/ chondroblastoma but malignant;
Tumor is especially rare in a child or adolescent; May invade epiphysis; Most common in proximal femur (over 50%) followed by proximal humerus;

y y

Microscopically, sheets of cartilaginous cells in a lobular arrangement are mixed with scattered giant cells; This radiolucent lesion is often misdiagnosed and undertreated;

TJ Gilbert et al. Orthopedics Vol 18. 1995.

Atypical Enchondroma (Juxtacortical Chondroma)

y

Histologic exam of a low grade chondrosarcoma may show nodular cartilage tissue with mostly isomorphic tumor cells;
More cellularity than is seen w/ enchondroma; Differentiating between this malignant tumor growth and an enchondroma can be extremely difficult; Well circumscribed calcified lesion which has not changed in size is most consistent w/ an enchondroma; More peripherally located lesions are also more likely to represent an enchondroma;

HC Bauer et al. Acta Orthop. Scand. Vol 66

CLINICAL FINDINGS
Persistent, dull, aching pain Found in an older age group, peaking at age 45 years; Majority of chondrosarcomas occur about the hip and pelvis

HISTOLOGY
May be identical to benign enchondroma Where as a low grade malignant appearing cartilagenous tumor of extremities may actually be an enchondroma, a centrally occurring malignant appearing enchondroma in an adult is more likely represents a chondrosarcoma;

CARTILAGE IN CHONDROSARCOMA:

CARTILAGE IN CHONDROSARCOMA:

CHONDROSARCOMA - GROSS

CHONDROSARCOMA - GROSS

CHONDROSARCOMA OF PELVIS

nodules of white to bluish-white cartilagenous tumor tissue eroding and extending outward from the bone at the lower right.

RADIOGRAPHIC STUDIES:
Subtle, radiolucent, permeative lesion; May have hazy or speckled calcifications w/ either a diffuse "salt & pepper" pattern or a more discrete "pop corn" pattern; Pattern of calcification is usually diagnostic of cartilaginous tumor;

RADIOGRAPHIC FINDING

TREATMENT:
Tumor does not respond to x-ray therapy nor to currently available antineoplastic drugs; Low grade tumors:

y y y

Rarely metastasize; Rarely recurs after wide limb salvaging excision; The involved bone is resected along w/ a small cuff of surrounding muscle; Have higher rate of recurrence after limb salvage (requires amputation) Prone to pulmonary metastases.

High grade tumors

y y

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