Anda di halaman 1dari 51

CHONDROSARCOMA

y Chondrosarcoma of bone is a malignant tumor

of proliferating cartilage tissue. y It accounts for approximately 10% of malignant bone tumors and tends to occur in older adults. y Peak incidence is in the fifth to sixth decades of life y After myeloma and osteosarcoma it is the third most common primary malignancy of bone.

y More than 75% of these lesions are located in the

trunk or proximal portions of the femur or humerus. y The inner wall of the acetabulum is a particularly common site. y When it is found in the extremities, chondrosarcoma occurs centrally within the intramedullary portion of the bone.

y The lesions tend to grow slowly, and pain may be

experienced for months or years before a mass or swelling is detected. y Metastasis is generally to the lung but is rare in low-grade lesions and often occurs late in the course of the disease.

Classification of Chondrosarcoma
y Conventional chondrosarcoma y Periosteal chondrosarcoma y Mesenchymal chondrosarcoma y Clear cell chondrosarcoma y Dedifferentiated chondrosarcoma y Soft tissue chondrosarcoma y Secondary chondrosarcoma

Secondary chondrosarcoma arise in


y Solitary osteochondroma y Solitary enchondroma y Hereditary multiple osteochondromas y Ollier s disease y Maffucci s syndrome

Primary chondrosarcoma
y Malignant cartilage tumour arising in a previously y y y y

normal bone. Also known as central or conventional chondrosarcoma Age usually beyond 3rd decade ; peak incidence 5th to 7th decade. Males are affected twice as common. Illium is the most commonly involved bone ( followed by proximal femur and proximal humerus )

PATHOLOGIC FEATURES
y Chondrosarcoma tends to be lobular with a matrix

of varying consistencies y The center of the lobules often undergoes myxoid degeneration. y Cortical destruction with soft-tissue extension is common, and calcific densities are frequently found within the chondroid matrix.

y This tumor has been divided into three histologic

grades. y Approximately 90% are well-differentiated (grade 1 or 2) tumors. y In general, the grade of the tumor correlates with its cellularity y It is important to differentiate high-grade chondrosarcoma from chondroblastic osteosarcoma ( chondroblastic osteosarcoma has marked cytologic atypia and osteoid production )

Grade I chondrosarcoma .
The lesion is quite cellular, the nuclei are enlarged and irregular, and double nucleated cells are present

Grade 1
y Exclusive presence of small densely staining nuclei y Intracellulat matrix varies from chondroid to

myxoid y Multiple nuclei within one lacuna

Grade 2
y Increased cellularity and moderate sized nuclei y Mitotic rate is low ( < 2 mitosis per 10 high power

field ) y Matrix is myxoid y Nuclei have intranuclear detail

Findings suggestive of grade 2 chondrosarcoma may be limited to isolated areas of the tumour, so multiple sections must be studied.

Grade 3
y 2 or more mitosis per 10 high power field y High cellularity ( towerds periphery ) y Nuclear size is greater than grade 2

y It is also often difficult to differentiate a low-grade

chondrosarcoma from an enchondroma. Therefore, it is extremely important to correlate the clinical, radiographic, and histologic presentations before rendering a final diagnosis. Chondrosarcoma has more pleomorphic cells and may have myxoid changes within the chondroid matrix. Binucleation of the cells is not a distinguishing feature because it can be seen in both lesions.

y Differentiating a low-grade chondrosarcoma from

an enchondroma can be difficult (if not impossible) solely from a biopsy specimen. Factors that favor a malignant diagnosis include hypercellularity, plump nuclei, more than occasional binucleate cells, a permeative pattern, and entrapment of bony trabeculae.

RADIOGRAPHIC FEATURES
y Chondrosarcoma appears as a predominantly lytic

lesion that arises in the medullary cavity but usually involves the cortex as well. y On plain radiographs, the typical findings are expansion of medullary portion of bone and thickening of the cortex ; but periosteal reaction is scant or absent. y Eventually it extends through the cortex and forms a large soft-tissue mass.

Anteroposterior view of proximal humerus of 47year-old man with conventional chondrosarcoma

y The radiographic appearance of chondrosarcoma

frequently is diagnostic y The pattern of calcification has been described as punctate, popcorn, or comma-shaped. y Compared with enchondroma, however, chondrosarcoma has a more aggressive appearance with bone destruction, cortical erosions, periosteal reaction, and rarely a soft-tissue mass.

y A CT scan can be helpful to show endosteal erosions or

other evidence of a destructive lesion and to differentiate benign from malignant cartilage lesions. y The site of the lesion also must be considered because lesions in the hand (the most common site for an enchondroma and a rare site for a chondrosarcoma) may appear aggressive and still be diagnosed as benign. The same amount of cortical destruction shown in a pelvic or proximal femoral lesion would be diagnostic of a chondrosarcoma.

Plain radiographic and CT findings in low and high grade chondrosarcomas have been summed up Rosenthal as follows

Low grade features


y Dense calcification forming rings or spicules y Widespread or uniformly disrtibuted

calcifications y Eccentric lobular growth of a soft tissue mass

High grade features


y Faint amorphous calcification y Large non calcified areas y Concentric growth of a soft tissue mass

AP and lateral radiographs of the left distal femur in a 61-year-old woman with chondrosarcoma showing a calcified intramedullary lesion with destruction of the surrounding cortex.

Bone scan
y Murphey graded radionucleotide uptake in bone

into 3 grades y Grade 1 indicating uptake less than that in anterior superior iliac spine y Grade 2 similar to that in anterior superior iliac spine y Grade 3 uptake greater than that in anterior superior iliac spine

Bone scan s h ow i n g the e x te n t and a c t iv i t y of the tumor.

Coronal and axial MRI images further define the soft tissue and intramedullary extent to assist in preoperative planning.

Plain radiograph

Anteropost erior view of right shoulder showing an extensive chondrosar coma arising from the upper scapula

Biopsy
y It is important to establish the diagnosis before

any surgical intervention is planned. y Biopsy can be taken either as a minimally invasive procedure ( needle biopsy ) or as open procedure ( open biopsy ) y It is important to know that a single lesion may have areas of varying grades of disease, therefore it is important to sample different areas of the lesion from a single point of entry.

PERIOSTEAL CHONDROSARCOMA
y Rare malignant cartilage forming tumour arising

y y y y

from outer surface of bone and possibly of periosteal origin. Highest incidence in 4th decade Male predominance Mostly occurs in metaphysis of long bones ; especially distal femur Radiological and pathological features similar to conventional chondrosarcoma

y Grossly - a well differentiated cartilage mass with

stippled calcification. Tumour osteoid or bone is absent in disease mass. y Histopathologically cartilaginous lobular pattern limited to surface and rarely infiltrating the cortex. y Prognosis is excellent after adequate surgical excision and is better when compared to central chondrosarcoma of similar grade of malignancy

Mesenchymal chondrosarcoma
y This rare form of chondrosarcoma is a tumor of

undifferentiated mesenchymal cells mixed with areas of cartilage. y Highly malignant cartilage lesion with a bimorphic histologic appearance y Occur in patients who are in their second or third decade

y X-Ray Appearance and Advanced Imaging

Findings: The lesions are predominately lytic and often have stippled density characteristic of cartilage tumors. The border is poorly defined, but may be sharp with sclerosis. y Histopathology findings: Sheets of spindle cells, and/or ovoid/round cells which are markedly uniform in size. Multifocal areas of differentiation into cartilage.

y Treatment : Complete wide resection, with

aggressively wide margins. Long-term follow up is needed. Chemotherapy and radiation are used when complete resection is not possible, but the role of chemotherapy is not well defined. Multimodal treatment may be considered. y Chemotherapy appears to be linked to better survival.

Clear cell chondrosarcoma


y It is the least common of the chondrosarcoma

subtypes. y Low-grade malignant tumor with a predilection for the ends of long bones, particularly the proximal femur and humerus y Most common location is in epiphysis. The most common sites of involvement are the proximal femur, proximal humerus, distal femur and proximal tibia.

y The most common symptoms are pain and

swelling, that usually are of long duration. There may be limitation of the range of motion of the involved joint. y On radiographs, these tumors are wellcircumscribed mixed lucent and sclerotic lesions with an active appearance. y Treatment and prognosis: En bloc resection with a wide margin of normal bone and soft tissue is the procedure of choice.

Dedifferentiated chondrosarcoma
y Most malignant variant that accounts for

approximately 10% of chondrosarcomas y Contains 2 clearly distinct pathological tissue components ; one is a well differentiated cartilage tumour and the other is a high grade non cartilaginous sarcoma. y Average age of presentation is 50 to 60 yrs y Males and females are equally affected.

y Radiologically , tumour shows a poorly defined,

lytic, intraosseous lesion with associated cortical perforation and extra osseous extension into the soft tissues producing a large mass. y Histopathologically, the hallmark of this lesion is appearance of an aggressive sarcoma engrafted on an indolent appearing chondrosarcoma.

Anteroposterior view of right proximal humerus of 92-year-old woman with dedifferentiated chondrosarcoma shows aggressiveappearing area (arrows) adjacent to otherwise typical chondrosarcoma

Resected specimen from the same patient

Typical microscopic appearance of dedifferentiated chondrosarcoma High-grade spindle cell sarcoma is located adjacent to low-grade chondrosarcoma

TREATMENT
y Surgical resection remains the mainstay of therapy

for chondrosarcoma y A well-planned and adequate biopsy that is representative of the entire tumor is necessary. y A wide margin must be achieved at initial resection to ensure the best chance of cure for this tumor, which is notorious for local recurrence.

y Accurate preoperative staging with determination

of the histopathologic grade and regional tumor extent dictates the aggressiveness needed at operation. y For lesions of higher grade with extensive softtissue and neurovascular involvement, amputation may be required to ensure local control. y Even lower grade lesions that have recurred in the pelvis often involve the neurovascular bundle and necessitate external hemipelvectomy.

y For lesions involving the proximal femur, a custom

prosthetic replacement or an allograft prosthetic composite is an effective reconstructive option after proximal femoral resection.

Wide resection of the distal femur followed by reconstruction using a custom rotating hinge knee prosthesis

Anteroposterior radiograph after reconstruction with endoprosthesis

y Of the chondrosarcoma variants, clear cell

chondrosarcoma is curable after wide resection y Mesenchymal chondrosarcoma also necessitates an aggressive surgical approach to achieve a wide resection margin. y One report found radiation and chemotherapy to be beneficial in a subset of these tumors (Huvos AG, Rosen G, Dabska M, Marcove R. Mesenchymal Chondrosarcoma: A Clinicopathologic Analysis of 35 Patients with Emphasis on Treatment. Cancer 1983;51:1230.)

y Dedifferentiated chondrosarcoma has a dismal

prognosis, despite radical surgical resection and adjuvant therapy.

PROGNOSTIC FACTORS
y The single most predictor of local recurrence and

metastasis is the histological grade. y None of grade 1 chondrosarcomas metastatise while metastases is seen in 10% of grade 2 and 71% 0f grade 3 lesions.

Prognosis
y The overall survival rate for a patient with

conventional chondrosarcoma is highly dependent on the histologic grade y Because local recurrence affects survival adversely and late recurrences are common, 5-year survival rates are unreliable indicators of long-term results. y Local recurrence occurs in 20% and pulmonary metastases in 15%

y Patients treated with intralesional curettage have

an 80% risk of local recurrence, with metastases to the lung and other skeletal sites y Patients with mesenchymal chondrosarcoma have a survival rate of 50% at 5 years and 28% at 10 years y Results of treatment of highly malignant, dedifferentiated chondrosarcoma are poor despite radical surgery. The 5-year survival rate is approximately 10%

THANK YOU

Anda mungkin juga menyukai