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  • Normal cartilage is seen above ,presense of both malignant and benign components in one tumor is diagnostic of secondary chondrosarcoma1 chondrosarcoma with cartilaginous cap,bi and trinucleatechondrocytes with nuclear atypia is seen
  • Chondrosarcoma

    1. 1. Dr Amruth ram reddy Ortho PG Gandhi medical college
    2. 2. CHONDROSARCOMA •It is a malignant ,relatively slow growing cartilage producing tumor. •it has a long natural history and a better prognosis compared to osteosarcoma. •It forms approximately 10% of malignant primary bone tumors. •It is third most common primary malignant tumors of bone after multiple myeloma and osteosarcoma.
    3. 3. • TYPES OF CHONDROSARCOMAS • Central or conventional • Peripheral or periosteal • Clear cell • Mesenchymal • Dedifferentiated • Chondrosarcomas of small bones
    4. 4. • Primary chondrosarcomas arises denovo from bone with out any preexisting lesion. • Central chondrosarcomas are generally primary chondrosarcomas. • Secondary chondrosarcomas. when tumor arises from preexisting benign lesions of bone. • Peripheral chondrosarcomas are usually secondary chondrosarcomas
    5. 5. • ETIOLOGY AND PREDISPOSING FACTORS • AGE:primary tumor is generally seen between 20 to 60 years of age. • Secondary chondrosarcomas are more common in young adults. • They are extremely rare under 20 years of age. • SEX:more common among males. • LOCATION:common sites include
    6. 6. • pelvis (ilium) • Proximal humeral shaft. • Proximal femoral shaft. • Distal femoral shaft. • Innominate bone. • Ribs • Scapula. • ANTECEDENT LESIONS:An enchondroma which is a part of multiple enchondromatosis(olliers disease) and if situated in a long tubular bone may change in to secondary chondrosarcomas.
    7. 7. • An osteochondroma(exostosis) when part of heriditary multiple exostosis is also vulnarable to malignant change. • Pagets disease and synovial chondromatosis also predispose to chondro sarcoma. • It can also develop as a consequence of radiation.exposure to x rays may very well have been in safer range.
    8. 8. • PATHOLOGY OF CHONDROSARCOMAS • CENTRAL TYPE • lobulated., translucent, bluish whitish cartilaginous mass with in medullary cavity. • Endosteal aspect of cortex appears eroded and scalloped. • Tumor spreads widely through medullary cavity, its extent is greater than it appears on x rays. • As tumor penetrates the cortex it is temporarily delimited by periosteum.
    9. 9. • New reactive new bone is formed at the periphery of sub periostealmass there by thickning the cortex. • This is seen in slow growing and less malignant tumors. • In rapidly growing tumors periosteum is penetrated easily, no no reactive new bone formation. • When a firm greyish tanned tissue with out lobular configuration is found, it represents most malignant type.
    10. 10. • On removing the closely investing periosteum, the tumor appears lobulated,bluish white, shiny, opalescent or semitranslucent mass of firm texture or rubbery consistency. • The cut surface exhibits specks of calcification that impart a gritty sensation on palpation, gelatinous or myxoid areas and ragged cavities. • The degree of calcification is greater in slower growing tumors.
    11. 11. • MICROSCOPIC APPEARANCE • Histological features of cartilage should be noted to diagnose presence of a cartilaginous tumor • A normal chondrocyte is ellipsoid, ovoid or round in shape, has slightly scalloped surfaces. • contain a central, oval, sometimes indented nucleus with regularly distributed abundant chromatin and indistinct nucleolus.
    12. 12. • Sarcomatous chondrocytes and their nuclei vary greatly in size, shape, number of nuclei and nucleoli chromatin content. • Histology shows signs of malignancy including hyper cellularity, plumpness of nuclei, double nuclei in more than occasional cells, pleomorphism of cells and nuclei, hyperchromatism and mitotic figures. • Mitotic figures are rare in low and medium grade tumors but are readily found in high grade tumors.
    13. 13. • HISTOLOGICAL DIFFERENTIATION FROM AN OSTEOSARCOMA • It is essential to distinguish a chondrosarcomas from an osteosarcoma, in which a chondroid tissue is often found but has a more serious out look. • In osteosarcoma the malignant osteoblasts contain alkaline phosphatase and lay down osteoid. • In a chondrosarcomas, both osteoid and alkaline phosphatase are lacking.
    14. 14. • CLINICAL PRESENTATION • PRIMARY CHONDROSARCOMAS • SWELLING AND PAIN • A central tumor of a long tubular bone usually remains entire asympotomatic until it has eroded and penetrated the cortex or caused a pathological fracture. • It can present as an swelling over the affected bone. • Pain is not a prominent feature of primary chondrosarcomas. • Depending upon the grade of malignancy the swelling will be fast or slow growing. • Later on patient may complain of rapid onset of severe pain that generally reflects the rate of growth and severe grade of malignancy.
    15. 15. • PATHOLOGICAL FRACTURES • Occasionally patients with chondrosarcomas can present with pathological fractures and synovial effusion, when tumor is near a joint. • NEUROLOGICAL INVOLVEMENT • Tumors of pelvis and innominate bones can present with nerve dysfunction of either sciatic, femoral or lumbosacral plexus.
    16. 16. • ON CLINICAL EXAMINATION • These will be presence of a enlarged irregular or swelling, hard in consistency, arising from bone, and firmly fixed to the underlying bone. • Overlying skin will be stretched and may show engrorged veins. • Other general features of malignancy with weight loss, loss of appetice and general ill health will be present.
    17. 17. • RADIOLOGICAL FEATURES • CENTRAL TUMORS • The lesion is seen in metaphysic or diaphysis of a long tubular bone. • Rarely it may occupy epiphysis at juxtraarticular area, where it may be mistaken for a gaint cell tumor or chondroblastoma. • The lesion is located in medullary cavity, it is large and ill defined. Countour of bone is enlarged or expanded. • The cortex is focally or extensively eroded and thinned out. The inner cortex may have scalloped borders.
    18. 18. • The interior of lesson is radiolucent and may show blochy radioopactiies radiolucencies represents large lobules of cartilage which may give a bubbly appearance. • Tumors will have specks of calcification and cotton whorl appearance. • Slow growing tumors will show a greater of calcification where as highly malignant rapidly growing tumors will show little or no calcification and often destroys the cortex without expanding shaft. • There is a associated soft tissue mass.
    19. 19. • The extent and degree of endosteal scalloping correlate with likelihood of lesion being a chondrosarcoma • Cortical expansion and thickning are adaptive changes and cortical disruption and soft tissue masses are aggressive radiological changes.
    20. 20. • LOW GRADE FEATURES: • Dense calcification forming rings or specules • Eccentric lobular growth of a soft tissue mass • HIGH GRADE FEATURES: • Faint amorphous calcifications • large non calcified areas • Concentric growth of soft tissue mass
    21. 21. • TREATMENT: • These tumors are not generally sensitive tochemotherapy and radio therapy.surgeryis the only reliable treatment for these tumors. • CHONDROSARCOMA IN SITU: • These patients can be treated with intra lesional excision coupled with adjuvants like phenol or cryotherapy instead of wide excision.
    22. 22. • CHONDROSARCOMAS WITH ADAPTIVE OR AGGRESSIVE RADIOLOGICAL CHANGES:a wide resection is recommended for grade2 and grade 3 chondrosarcomas of long bones. • grade 1 chondrosarcomas in long bones with adaptive or aggressive radiological findings a wide resection is preferable for adequate margins. • A report at the musculoskeletal society tumor meeting showed that proiliferation of chondrocytes was inhibited by ciprofloxacin. • It inhibits apoptosis and cause magnesium deficiency in tumor cells
    23. 23. • It enhances production of apoptogenic interleukin1. • It also interferes with enzyme topoisomerase 2 inhibiting dna synthesis. • Poorly differentiated chondrocytes appear to be susepteble to effects of ciprofloxacin. • Clinical relavance of this finding is not known.
    24. 24. • Single most important predictor of local reccurance and metastasis is histological Grade. • Prognosis: • Most of pulmonary metastasis or local reccurences occur with in first five years of presentation. • Survival rates at the end of ten years were 89% for grade 1 ,53% for grade2and 38% for grade3 chondro sarcomas.
    25. 25. • SECONDARY CHONDROSARCOMAS • Chondrosarcoma occuring in a known benign precursor lesion is known as secondary chondrosarcoma.risk is less than 1 % in solitary osteo chondroma,however risk increases to 1 to 5 % in osteochondromatosis. • Patients with olliers disease and maffuccis syndrome have 25 to 30 % risk. • They develop at a slightly earlier age,they are of low grade and have favourable prognosis
    26. 26. • Chondrosarcoma developing on the surface of bone as a result of malignant transformation of cartilage cap of pre existing osteochondroma is called as peripheral chondrosarcoma. • Sites: • Any part of skeleton may be involved,pelvic and shoulder girdle bones are commonly effected. • Clinical features: • Sudden on set of pain or increase in size of swelling in a known case of precursor lesion is usual clinical presentation.
    27. 27. • Imaging: • Plain x rays show irregular Mineralization and increased Thickness of cartilage cap in Osteochondromas. In preexisting enchondromas there is destructive permeation of bone and development of soft tissue mass. A cartilage cap of greater than 2 cms is suspicious of malignancy.
    28. 28. • This cartilage usually shows cystic cavities. • Secondary chondrosarcomas arising in enchondromas are usually very myxoid unlike solid blue areas of enchondroma. • HISTOPATHOLOGY: these are usually grade 1 tumors • Invasion of surrounding tissues and marked myxoid changes in the matrix are features useful in making diagnosis.
    29. 29. Normal cartlage is seen in above slide, Presence of both normal and malignant Changes ina single tumor is diagnostic Of secondary chondrosarcoma Chondro sarcoma with cartilaginous cap ,bi and trinucleate chondrocytes with Nuclear atypia is seen
    30. 30. • TREATMENT: • Complete wide excision of the lesion • Cartilage cap should not be violated during resection of chondrosarcoma arising out of osteochondroma as it will increase the risk of local reccurence. • PROGNOSIS:tumor arising in osteochondromas have excellent prognosis. • Secondary chondrosarcomas arising in enchondramatosis have same prognosis as that of conventional chondrosarcomas.
    31. 31. PERIOSTEAL CHONDROSARCOMA It is a rare tumour arising fromouter surface of bone possibly periosteal in origin. • Also known as juxtacortical chondrosarcoma. • Occurs in adults,peak incidence is 4th decade • More common in males • Occurs in metaphysis of long bones,especially distal femur • Presents usually with pain or swelling.
    32. 32. IMAGING; Round ,lies on surface of bone Indistinct irregular margins Radiolucent lobulated lesion With stippled calcification. Covered by an elevated Periosteum. Shows variable erosion of Cortical bone underneath. It rarely involves medullary canal
    33. 33. • GROSS:it is a well defined lobulated cartilage mass with stippled calcification and endochondral ossification. • Tumor osteoid or bone is absent in disease mass. • HISTOPATHOLOGY:it shows a cartilaginous lobular pattern limited to surface and rarely infiltrating the cortex of the bone. • Areas of spotty calcification and endochondral ossification is seen. • These are generally low to intermediate grade tumors.
    34. 34. • DIFFERENTIAL DIAGNOSIS: • Secondary peripheral chondrosarcoma • Periosteal osteosarcoma:on plain xray it is often round in shape,containing granular or pop corn more commonly involves diaphyseal bone • It is elongated along the long axis of the bone and shows multiple calcified spicules perpendicular to long axis of underlying bone. • PERIOSTEAL CHONDROMAS:small 2 to 3cms,longer than they are wide and have solid periosteal buttresses on x ray.
    35. 35. DEDIFFERENTIATED CHONDROSARCOMA • Most malignant of all chondrosarcomas and has very poor prognosis. • It has an additional mesenchymal component • It has two clearly distinct pathological tissue components. 1)well differentiated cartilage tumor either enchondroma or low grade chondrosarcoma • 2)a high grade non cartilaginous sarcoma,with a remarkably sharp junction between both components.
    36. 36. • The non cartilaginous component may express features of malignant fibrous histiocytoma,osteosarcoma,fibrosarcoma,rhabdomyo sarcoma. • The sharp interface between two varieties is important in diagnosis and its distinction from grade 3 conventional chondrosarcoma. • Around 10%of all reported chondro sarcomas are of this type. • AGE:average age of presentation is between 50 and 60 years. • Males and females are equally effected.
    37. 37. • Most common sites of involvement are pelvis,proximal femur,proximal humerus distal femur and ribs. • CLINICAL FEATURES: • Pain is most common presenting symptom,parasthesias and pathological fractures are also common presentations
    38. 38. • IMAGING:on plain xray tumor usually shows poorly defined,lytic,intraosseous lesion with associated cortical perforation and extension in to soft tissues producing a large mass. • The cartilaginous component of the disease is sharply distinct from lytic,permeative and destructive components. • The large size of soft tissue mass and presence of metastasus at presentation are usefull clues topossible diagnosis.
    39. 39. • Left arrow low grade cartilaginous area which is heavily calcified. Right arrow :aggressive Lytic area with Dedifferentiated Component,cortical Destruction with out Calcification.
    40. 40. • GROSS:on cut surface both tumor components are grossly apparent in varyingproportions. • The low grade cartilaginous portion is blue grey in color and is usually central in locationwhile hemorrhagic high grade component is predominantly extraosseous.
    41. 41. •De differentiated chondrosarcoma Of humerus •Top arrow low grade cartilage •Bottom arrowhigh grade dedifferentiated Component.
    42. 42. • HISTOPATHOLOGY:hall mark of the lesion is aggressive sarcoma engrafted on indolent appearing chondrosarcoma. • Cartilaginous component is usually a low grade chondrosarcoma • The high grade component is usually malignant fibrous histiocytoma.
    43. 43. Left arrow low grade cartilage component Right arrow dedifferentiated component.
    44. 44. • DEDIFFERENTIATED CHONDROSARCOMA WITH PSEUDO-GAINT CELL TUMOUR VARIANT:In rare cases of chondrosarcoma the highly malignant dedifferentiated component attact myriads of bening osteoclast-like giant cells, do: itomg the anaplastic cells by hundreds is to one. • The tumour will resemble a conventional GCT in term of cellular constituent. Nevertheless, the tumor will behave as a fully malignant dedifferentiated sarcoma with metastasis and lethality in 80-90% of cases.
    45. 45. • These have very poor prognosis. • Despite aggressuve treatment 90% of patients die with in 2 years with distant metastasis. • Certain reports advocate the use of chemotherapy in imprtoving the out comes but efficacy is debatable.
    46. 46. MESENCHYMAL CHONDROSARCOMAS • IT is a malignant tumor charecterized by bimorphic pattern that is composed of highly un differentiated small round cells and islands of well differentiated cartilage. • Accounts for less thanm 3 to 10% of all primary chondrosarcomas • Occurs at any age,peak incidence at 2nd or 3rd decades. • Males and females are equally effected.
    47. 47. • The craniofacial bones a,ribs,ilium,vertebrae are most common sites. • Approximately1/3 to 1/5 of lesions primarily affect somatic soft tissues. • Meninges are most common sites of extra skeletal involvement followed by leg or thigh. • METASTASIS TO REGIONAL LYMPHNODES AND OTHER BONES ARE COMMON unlike conventional chondrosarcomas.
    48. 48. • CLINICAL FEATURES: • Chief presenting symptoms are pain and swelling which range in duration from days to several years. • IMAGING:it resembles conventional chondrosarcoma • Bony lesions are lytic and destructive with motteled calcification and in distinct margins. • May have charecteristic permeative growth pattern similar to round cell tumors • Some have sclerotic rim with clearly defined margins • Cortical destruction with cortical breakthrough with extension in to soft tissue is common finding.
    49. 49. • HISTOPATHOLOGY:highly malignant tumor and has biphasic pattern. • It is composed of scattered areas of cartilage,together with highly vascular mesenchymal tissue composed of undifferentiated spindle cells or round cells with cytoplasm. • The cartilaginous areas are usually small,sharply demacated and cytologically benign or of low grade. • When recognisable cartilage is absent it may be confused with ewings sarcoma or malignant vascular tumor
    50. 50. • Differential dagnosis includesewings sarcoma,small cell osteosarcoma and Dedifferentiated chondrosarcoma. PROGNOSIS: It is a rare but highly malignant tumor with a strong tendency for local reccurence and distant metastasis. It has a poor ten year survival rate of 28%. Certain reports advocate the use of chemotherapy similar to ewings sarcoma but efficacy is debatable.
    51. 51. CLEAR CELL CHONDRO SARCOMA • This is a low grade chondrosarcoma. • It has a prediliction for epiphyseal end of long bones. • It is highly uncommon type comprising less than 4% of all chondrosarcomas. • Most patients are between 25 to 50 years. • 2/3 of the lesions occur in femoral head or humeral head • Pain is most common presenting symptom.
    52. 52. IMAGING:it presents as a well defined slightly expansile lytic Lesion often with sharp margin,in epiphysis of long bone. Some lesions may have stippled radiodensites charecteristic Of a cartilaginous tumor. It may resemble chondro blastoma.
    53. 53. • HISTOPATHOLOGY: • It is a low grade malignant tumor consisting primarily of lobular group of cells which are charecteristically large and rounded with centrally located nuclei and clear cytoplasmic membranes • Clarity of the cells is due to abundant intra cellular glycogen and paucity of cellular organelles. • A chondroid and osseous matrix,trabeculae of reactive bone and numerous osteoclast like giant cells are unique features seen in this tumor.
    54. 54. • Recurrence after intralesional curettage is common. • Wide resection is treatment of choice. • Marginal excision or curettage has 86% recurrence. • Enbloc excision with clear margins usually results in cure. • Prognosis is usually poor with metastasis developing in to lungs and other bones.
    55. 55. EXTRASKLETAL MYXOID CHONDROSARCOMA • It is a rare tumor usually developing in deep soft tissues of extremities. • It has also been reffered as chondroid sarcoma • Repoted age is from 6 to 89 years. • Male to female ratio is 2:1 • Almost 4/5 cases occur in proximal part of extremities or limb girdles
    56. 56. • HISTOPATHOLOGY: • Tumor cells are allinged in cords and strands and are seperated by large amounts of myxoid matrix. • PROGNOSIS:there is high rate of local reccurence following surgery • 46% of cases develop metastasis.deathrate is high. • Histological grading has no prognostic value.
    57. 57. Thank you
    58. 58. • PERIPHERAL TUMORS • Are often large. • Central portion of tumor is usually heavily calcified and from this central dense shadow, blotches and streaks of calcification extends towards periphery of tumor which is lobulated translucent and usually not calcified. • In some tumors, the base of the original osteocartilagenous exoxtosis can be identified, where it is continuous with medullary cavity.
    59. 59. • DIAGNOSTIC FEATURES OF SECONDARY MALIGANT CHANGE OF A BENING CARTILAGE TUMOR INDUCES • Evidence of soft tissue calcification or a soft tissue mass. • Endosteal erosion and permeative features in an enchondroma including areas of lysis. • Growth in a previously stable exostosis • Expansion of the cartilage cap of an exoxtosis
    60. 60. • PROGNOSIS OF CHONDROSARCOMAS • Depends on correlating following points • AGE: Chondrosarcomas is not commonly seen in younger age group, but when present, it has been repoted to pursue a more aggressive course. • LOCATION: Cartilaginous tumors of hands and feet rarely become malignant. • Cartilaginous tumors of axial skeleton and proximal portion of long limbs bones are highly succeptible to sarcomatous change tumor of pelvic and thorasic regions are difficult to control. • Direct extension into pelvic cavity and thorax and recurrence accounts for higher mortality.
    61. 61. • On other hand tumors of limbs are more amenable to repeated surgical control. • So axial chondrosarcomas have poor prognosis as compared to appendicular chondrosarcomas. • SIZE OF TUMOR: A large chondroma is more likely to become sarcomatous than a smaller one. • CYTOLOGICAL FEATURES: High grade chondrosarcomas form a relatively small proportion of cases, but they are highly lethal with approximately 75% risk of metastasis and eventual mortality of about 90%. • None of the low grade tumors metastasize and metastasis occurs in 10% of medium grade tumors.
    62. 62. • Heavily calcified tissue are generally of low grade malignancy. • Such changes are minimal in rapid growing tumors. • LOCAL BEHAVIOUR: In low grade and medium grade tumors, despite an adequate surgical resection, multiple recurrence can occur over many years and tumor may progress towards a high grade and such recurrent tumors can eventually metastasize to lungs. • Central tumors tend to be more aggressive than peripheral ones.
    63. 63. • ANTECEDENT LESIONS: Secondary chondrosarcomas arising from a osteocartilaginous exoxtosis or an enchondroma are mostly low grade tumors. • SURVIVAL TIME: Survival of 10 years in usual in approximately 70% of low grade tumors, 40% in medium grade tumors and 30% in high grade tumors after adequate surgical treatment. • After 10 years, death from tumor is extremely rare. • A first recurrence is highly unusual after 5 years following adequate surgical resection.
    64. 64. • DIFFERENTIAL DIAGNOSIS: • Enchondroma • Osteochondromas • Osteosarcoma • Fibrosarcoma • Malignant fibrous histiocytoma • Metastasis • Chordomas
    65. 65. • Giant cell rich osteosarcoma by absence of tumour bone, from malignant fibrous histocytoma – by the absence of storiform pattern – and histocytic cytoplasmic features. • MALIGNANT FIBROUS HISTOCYTOMA RICH IN BENING GIANT CELL: Infrequently a malignant fibrous histocytoma may be premeated by large number of benign osteoclast – like giant cells. • The tumour should show feature of MFH plus bening osteoclast like giant cells.
    66. 66. • DEDIFFERENTIATED CHONDROSARCOMA WITH PSEUDO-GAINT CELL TUMOUR VARIANT:In rare cases of chondrosarcoma the highly malignant dedifferentiated component attact myriads of bening osteoclast-like giant cells, do: itomg the anaplastic cells by hundreds is to one. • The tumour will resemble a conventional GCT in term of cellular constituent. Nevertheless, the tumor will behave as a fully malignant dedifferentiated sarcoma with metastasis and lethality in 80-90% of cases.
    67. 67. • MANAGEMENT: Is based on enneking system for staging bening and malignant musculaoskeletal system. • TUMOR TYPE: • Stage-1A - Local wide resection • Stage-1B - Wide amputation • Stage-11B - Local radical resection • Stage-11B - Radical amputation • When local wide resection – may result in significant functional impairment the defect created by excision can be made by some reconstructive procedure.
    68. 68. • Resection arthrodosis of knee is an extensive procedure, requiring prolonged recovery and is best reserved for vigorous young adults. • Reconstruction with custom total knee arthroplasty or above knee amputation preferred for middle aged / elderly patient. • ARTHRODESOIS BY TURN ‘O’ PLASTY: • In this technique the required length of tibia is split into two halves, one half is turned upside down and fixed with stump of femur left after excising the tumor. • A similar procedure can be used for tibial lesion after taking half of femur.
    69. 69. • TECHNIQUE OF ARTHRODESIS AFTER RESECTION OF FEMORAL LESION • Rod inserted retrograde into femur. • Posterior fibular graft with medullary pin in place is advanced 1 cm into cancellous bone of tibia. • Spacer block in place, rod is driven down the tibia. • Tibial cortical graft removed leaving tibial metaphyseal bridge. • Anterior graft from tibia is inserted into slot in femur proximally, countersunk into tibial bridge distally and fixed with two screws.
    70. 70. • Arthroplasty tumour is excisied and attempt made to reconstruct the joint by an autograft, allograft and artifical joint. • Radical amputation. • IN SUMMARY KEY TO DIFFERENTITING GCT FROM OTHER VARIANTS IS – • Age – Openepiphysis – Chondroblastoma / Osteosarcoma closed – GCT. • Location – Lesion is epiphyseal – GCT, - if metaphyseal / diaphyseal – Non-ossifying fibroma, osteosarcoma, fibrosarcoma, MFH/ GCT of hyperparathyroidism.
    71. 71. • Radiograph : GCT – Purely lytic lesion with geographic / moth eaten appearance. • Correct interpretation of histological picture suspecting GCT. • The key is first to eliminate gaint cells from consideration and concentrate on stromal cells.