Malignant bone tumours

malignant bone tumours

1. MALIGNANT BONE TUMOURS Dr.Archana Koshy

2. Primary malignant bone tumours are relatively rare . The determination of the nature and extent of the tumour is crucial . Conventional radiography , CT and MRI play an important role in the detection , establishing the nature , assessing the extent and in follow up in all suspected case of bone tumours .

3. Age in years Tumour <1 Neuroblastoma 1-10 Ewing sarcoma-tubular bones 10-30 Osteosarcoma, Ewing sarcoma-flat bones 30-40 Fibrosarcoma,malignant fibrous histiocytoma,lymphoma,Malignant GCT >40 Chondrosarcoma,chordoma,multiple myeloma AGE OF ONSET

4. SITE OF ORIGIN EPIPHYSIS MALIGNANT GCT METAPHYSIS OSTEOSARCOMA CHONDROSARCOMA FIBROSARCOMA DIAPHYSIS EWING’S SARCOMA MULTIPLA MYELOMA ADAMANTINOMA MFH

5. Radiographic features that may help differentiate benign from malignant lesions

6. STAGING OF BONE TUMOURS

7. IMAGING MODALITIES Conventional radiographic techniques still remain the mainstay in initial diagnosis and work up of a patient suspected to have any bony pathology . D emonstrate the location,pattern of bone destruction, size and shape of the lesion, presence and nature of the visible tumor matrix, trabeculation , periosteal response and the presence and characteristics of the adjacent soft tissue mass. Due to its excellent contrast resolution and multiplanar capabilities, MRI has now virtually replaced CT as the imaging modality for assessing the local extent . Non specific in differentiating benign from malignant tumours .

8. Dynamic contrast enhanced MRI – to determine response to chemotherapy . MR spectroscopy – Molecular characterisation of the tumours DWI – In assessing tumour response to therapy . -Effective therapy in bone tumours leads to breakdown of tumour cell membranes resulting in increased diffusion of water in the tumour on Diffusion weighted images . CT – Remains superior to MRI in the detection and characterisation of matrix mineralisation , in assessment of cortical involvement , bone trabeculation and periosteal reaction . -Also the investigation of choice for detecting pumonary metastases .

9. 99m Technetium methylene diphosphonate bone scan -first additional investigation in most instances to detect whether the lesion is monostotic or accompanied by other skeletal lesions. The major limitation of bone scanning is its lack of specificity as number of bone disorders may have similar scintigraphic appearances . FDG-PET - Evaluation of skeletal metastatic disease . FDG accumulation in skeletal lesions reflects direct uptake by tumor, in contrast to 99mTc MDP activity on conventional bone scans, which reflects increased new bone formation in response to destruction of bone by tumor.

10. OSTEOSARCOMA Commonest primary malignant bone tumour in the young . Characterised by the production of osteoid or bone by tumour cells . Malignant cells also retain the potential to form fibrous tissue or cartilage . Constituents of the tumour matrix are highly variable and can include osteoid tissue , calcification , fibrous tissue ,cartilaginous tissue or a combination .

11. CLASSIFICATION

12. Usually presents with localized pain or swelling , particularly around the knee May present with a pathological fracture . The lesion commonly arises eccentrically in the medullary cavity , with ill defined cortical destruction and soft tissue involvement . The pleomorphic nature of the histology may cause misleading biopsy results .

13. Conventional radiographs – Eccentric area of permeative bone destruction in the metaphysis adjacent to the knee joint Associated with a cortical erosion and a well defined soft tissue mass CODMAN’S TRIANGLE- Elevation of the periosteum when associated with new bone formation , formed at the margin of the lesion .

14. AP and lateral radiographs of the distal femur in a 14-year-old boy showing a classical osteocarcoma with mixed lytic and sclerotic areas, extraosseus mass with tumor bone formation, and Codman’s triangles at the upper and lower margins of the lesion

15. OSTEOSARCOMA OF THE METADIAPHYSEAL REGION OF TIBIA - MIXED LESION WITH A LARGE SOFT TISSUE COMPONENT AND THE CHARACTERISTIC “ SUNBURST” OR SPICULATED PERIOSTEAL REACTION

16. A coronal T1W MR image shows the large tumor as a predominantly hypointense mass with evidence of cortical destruction Axial T2W images show the cortical disruption with soft tissue extension of the tumor mass with loss of fat planes with the neurovascular bundle. Contrast enhanced coronal, (C) and axial, (D) MR images show heterogeneous enhancement of the tumor mass

17. AP radiograph showing a destructive lesion in the distal femur with extensive cloud like mineralization in the intra- and extraosseous component (B) A coronal T1W MR image shows the large tumor as a predominantly hypointense mass with evidence of cortical destruction (C) An axial T2W image shows a heterogenous mass which is completely encasing the neurovascular bundle. ( D) T2W (fat suppressed) coronal image shows the intra-articular extension of the tumor into the knee joint

18. OSTEOBLASTIC VARIETY . (A) AP and lateral radiographs show a purely osteosclerotic lesion of the proximal metaphysis of the tibia (B) CT shows mineralization of tumor matrix within the soft tissue component and a spiculated periosteal reaction

19. TELANGIECTATIC OSTEOSARCOMA. ( A) AP radiograph of the distal femur shows a predominantly lytic lesion in the distal femur with irregular cortical destruction and a large soft tissue component, (B) Sagittal T2W fat suppressed MR image shows the characteristic blood-fluid levels within the tumor, (C) On contrast enhancement, marked peripheral and septal enhancement is seen

20. Mestastatic spread occurs by the hematogenous route Pulmonary metastasis is associated with an unudually high incidence of pneumothorax . Any lung lesion arising in a patient with osteosarcoma should be regarded with supicion . Lymphatic spread is relatively rare .

21. JUXTACORTICAL/PAROSTEAL OSTEOSARCOMA Comprise 1% of all primary malignant bone tumours and about 4% of all osteosarcomas . The tumous is slow growing by comparison and has a much better prognosis . Parosteal sarcomas occur most commonly on the distal femur and proximal humerus .

22. Typically dense tumour surrounds a long bone , particularly a femur or tibia . The margins are sharply defined , but tend to undulate . The tumour is denser centrally and at the base than peripherally . Characteristically , there is a radiolucent zone between the ossified outer margins of the tumour and adjacent host bone .

23. CHONDROSARCOMA Malignant cartilaginous tumour > 40 yrs SITES—LONG BONES,PELVIS,RIBS,SPINE,STERNUM, skull base) Types Primary - Intramedullary Juxtacortical Clear cell,myxoid,extraskeletal Secondary -- Osteochondroma Solitary osteochondroma Hereditary multiple exostoses ) Enchondroma – Maffuci’s syndrome .

24. Plain film Lytic (50%) Intralesional calcification Rings and arcs / popcorn calcification) Endosteal scalloping: affecting more than two thirds of the cortical thickness ( c.F. Less than 2/3 in enchondromas )   Moth eaten / permeative appearance and periosteal reaction distinguishing between enchondroma and chondrosarcoma

25. Proximal tibia diaphysis. subtle calcifications,   Endosteal scalloping hallmark of chondrosarcoma .  MR better defines the extension of the lesion.  endosteal scalloping .

26. CHONDROSARCOMA ARISING FROM OSTEOCHONDROMA CALCIFIED MASS ARISING FROM THE PROXIMAL FIBULA. HIGH UPTAKE ON THE BONE SCAN

27. CONVENTIONAL CHONDROSARCOMA. (A) Plain radiograph shows an osteolytic , expansile lesion in the upper end of the fibula with faint calcification seen within (B) Coronal T2W Fat suppressed MR image shows the characteristic hyperintense , lobulated lesion with internal septations .

28. CHONDROSARCOMA. ( A) A large expansile lesion in the anterior end of the left 7th rib (B ) CT shows specks of calcification within the lesion

29. (A) Radiograph of the hemipelvis shows a mass extending from the left iliac blade with the characteristic ‘popcorn’ calcification of a cartilaginous matrix (B) T1W MR image shows the large soft tissue component of the chondrosarcoma as a low signal intensity lobulated mass (C ) On a coronal T2W MR image the tumor is seen as lobulated mass of high signal intensity with septations of low signal intensity (D) After intravenous administration of gadolinium, a coronal T1W MR image shows irregular peripheral and septal enhancement

30. FIBROSARCOMA Fibrosarcoma is a tumor of mesenchymal cell origin that is composed of malignant fibroblasts in a collagen background.. It can occur as a soft-tissue mass or as a primary or secondary bone tumor. Primary fibrosarcoma - central (arising within the medullary canal) or peripheral (arising from the periosteum ) Secondary fibrosarcoma of bone arises from a preexisting lesion or after radiotherapy to an area of bone or soft tissue. Fibrosarcomas of bone are typically seen between the third and sixth decades of life.

31. Plain Film   Highly destructive with a wide zone of transition and often expansile . Periosteal reaction is uncommon.  The lesion usually has not matrix mineralization, but may have areas of sequested bone. Often associated with a large soft tissue mass . CT   CT scanning is used to delineate bone involvement, bone destruction, or bone reaction. MRI best modality overall for examining soft-tissue masses and for detecting the intraosseous and extraosseous extent of many bony sarcomas. T1WI - Isointense T2WI - Hyperintense Shows strong enhancement on contrast images

32. Lateral radiographs of the knee show an osteolytic lesion with a short zone of transition in the distal femur. Note the absence of a periosteal reaction and tumor matrix mineralization

33. EWINGS SARCOMA Ewing sarcoma, a highly malignant primary bone tumor that is derived from red bone marrow and second most common primary bone tumour of childhood . This tumor is most frequently observed in children and adolescents aged 4-15 years and rarely develops in adults older than 30 years. Affected bones include, long bones: 50-60%, femur: 25%, tibia: 11%, humerus : 10%, flat bones: 40%, pelvis: 14%, scapula, ribs: 6% As far as location within long bones, the tumor is almost always metaphyseal or diaphyseal . It is the most lethal bone tumor. An association exists between Ewing sarcoma and primitive peripheral neuroectodermal tumor (PNET).

34. PLAIN RADIOGRAPHY Typical presentation: ill-defined osteolytic lesion with a moth-eaten or permeative type of bone destruction, irregular cortical destruction and aggressive periostitis . Reactive sclerosis, irregular periosteal reaction and soft tissue mass. Ewing sarcomas tend to be large poorly marginated tumours , with over 80% demonstrating extension into adjacent soft tissues. Laminated (onion skin) periosteal reaction: 57%

35. (A) CONVENTIONAL RADIOGRAPH SHOWS A PERMEATIVE LESION IN THE TIBIAL DIAPHYSIS WITH A SOFT TISSUE COMPONENT WITH SPICULATED PERIOSTEAL REACTION BETTER APPRECIATED ON THE AXIAL CT SCAN (B)

36. (A) Ewing’s sarcoma of the femur shows an illmarginated , lytic-sclerotic lesion with cortical irregularity , laminar periosteal reaction, with a large soft tissue mass in the metadiaphyseal region (B ) In another case involving the femoral diaphysis “ saucerization ” is well seen, with peripheral Codman’s triangles and a large soft tissue mass

37. Plain radiograph (B ) Axial CT section of left shoulder shows a dense sclerotic involvement of the scapula with a large soft tissue mass

38. A T1W coronal scan shows the intramedullary extent of the tumor as low signal intensity of the marrow with a small “skip” lesion proximally ( arrow) (B ) The extraosseous component is better appreciated on the STIR coronal image (C) The axial T2W image shows the tumor within the femur as high signal intensity with cortical discontinuity. (D) After radiotherapy, there is marked reduction in the soft tissue mass with well defined high signal intensity mass within the bone suggesting necrotic change.

39. Ewing’s sarcoma with skeletal metastases Pelvic radiograph shows the primary tumor as a large predominantly lytic lesion in the left iliac bone with multiple lytic bony metastases in the right iliac blade and both the femurs Humeral shaft (C) skull

40. PRIMITIVE NEUROECTODERMAL TUMOR OF BONE (PNET) These rare tumours of bone resemble Ewings tumours clinically except that they are commoner in the female sex Arise in the metaphyses and diaphyses of the lower limbs,humerus,pelvis and scapula . Radiologically , ill defined neoplasms which readily spread to soft tissues. ASKIN TUMOUR – rare PNET of the thoracopulmonary region occring in children and adults . A soft tissue mass involving the chest wall in seen in about half the patients and a subpleural pulmonary mass with pleural involvement in ¼ th .

41. NON-HODGKIN’S LYMPHOMA OF BONE (LYMPHOSARCOMA, RETICULUM CELL SARCOMA) Primary lymphoma of bone is a rare extranodal lymphoma, presenting intially as a localised solitary bone lesion . -20-50 years of age Usually presents with localised dull aching pain of long duration . A palpable mass is often felt . Common sites – diaphysis of long bones, femur , tibia , humerus Flat bones may also be involved .

42. The osseous destruction in this tumour is usually permeative or moth eaten in type with an associated soft tissue mass . Relative absence of cortical destruction is a characteristic feature . Proximal spread within the bone , sometimes with skip areas may be seen . MRI typically shows extensive involvement of the medullary canal with little cortical destruction but a large soft tissue mass. D/D – EWING’S SARCOMA -(older age group and lack of symptoms ) Good response to local radiotherapy and adjuvant chemotherapy.

43. Non-Hodgkin’s lymphoma . (A) AP radiograph of the humerus shows a mixed lytic and sclerotic lesion involving proximal half of the shaft with little cortical destruction and a large soft tissue component. (B) Sagittal T2W fat suppressed MR image through the shaft shows the extent of medullary involvement

44. HODGKIN’S LYMPHOMA OF BONE Occurs more commonly as a secondary manifestation of systemic hodgkin’s disease . Rarely as a orimary bone involvement Main site – Vertebral body of the lower thoracic and upper lumbar spine . Radiologically , most lesions are osteolytic . Occasionally , a marked sclerotic reaction can be seen as an ivory vertebra . In the lumbar vertebrae , lytic destruction is more common with exuberant periosteal reaction .

45. IVORY VERTEBRA SEEN AS A HOMOGENEOUS INCREASED DENSITY OF A SOLITARY THORACIC VERTEBRA

46. CHORDOMA Neoplasm which arises from ectopic cellular remnants of the notochord which have persisted within the nucleus pulposus of the intervertebral disk . Predilection for the sacrococcygeal and spheno occipital regions. 90% - occurs in the sacrococcygeal region Distant metastases are rare, mainly involving the lymph nodes , liver and lungs .

47. Sacral lesions usually show a large area of bone destruction with well defined , scalloped margins and a large soft tissue mass . Amorphous calcification may occur within the mass . In the spheno occipital region , the dorsal aspect of the sella and clivus may be destroyed with a soft tissue mass indenting the nasopharyngeal air space . In the sacrum , the possibility of plasmacytoma or giant cell tumour should be considered .

48. CHORDOMA OF THE SACRUM. PLAIN RADIOGRAPH (A) AND CT (B) SHOWING A LARGE PELVIC MASS WITH CALCIFICATION

49. CHORDOMA OF THE SACRUM. (A) PLAIN RADIOGRAPH SHOWS A LYTIC LESION DESTROYING THE DISTAL HALF OF THE SACRUM (B) AND (C) T1W SAGITTAL AND T2W AXIAL MR IMAGES SHOW THE LARGE TUMOR WITH INHOMOGENEOUS SIGNAL INTENSITY ARISING FROM THE DISTAL SACRUM

50. VASCULAR TUMORS OF THE BONE ANGIOSARCOMA Rare vascular tumour of bone , affecting all ages Any bone may be affected, but the vertebrae and femur are most commonly involved . A multilocular or “soap bubble “ appearance may be seen . Reactive sclerosis may be seen at the margins of some tumours . Highly malignant tumour usually treated by surgery and radiotherapy with a 5 year survival rate under 10 % .

51. Adamantinoma Rare primary malignant bony tumour , Occurs almost exclusively in the long bones. The diaphyseal region is the area most commonly affected. May present as a solitary focus or multicentric lucencies or slightly expansile osteolytic lesion May extend into the marrow cavity An eccentric epicenter and a lack of periosteal reaction. No periosteal reaction

52. MULTIPLE MYELOMA Most common primary malignant neoplasm of bone . Production of an abnormal paraprotein leading to a wide M band on plasma electrophoresis . Hypercalcemia , hypercalciuria and amyloidosis can occur , but serum ALK and serum Phosphorus are normal . CLASSICAL APPEARANCE - Well defined, osteolytic , punched out lesions throughout the skeleton, most characteristic in the skull . Generalised osteopenia A pathological fracture . Involvement of the spine Is usually in the lower thoracic and upper lumbar regions and is seen as diffuse osteopenia .

53. Multiple myeloma. (A) AP and (B) Lateral skull radiographs showing small, well defined, ‘punched out’ lesions in the calvarium

54. RADIOGRAPHS OF THE DORSAL SPINE SHOW OSTEOPOROSIS WITH SMALL DISCRETE LESIONS IN THE FEMUR .

55. Multiple myeloma. T1W (A) and T2W (B) sagittal MR images of the dorsal spine show complete alteration of the marrow signal in the seventh thoracic vertebra, hypointense on T1W and hyperintense on T2W images , with evidence of collapse.

56. Metastases Metastatic bone tumors are the most common malignant tumors of the skeleton. Approximately 70% of all malignant tumors are metastatic in origin . Metastases are usually found in: Vertebrae - especially posterior vertebral body, extending into pedicle, pelvis, proximal femur, proximal  humerus and skull. Metastases distal to the elbow and knee are distinctly uncommon. Types : - osteo lytic metastases - sclerotic / osteoblastic metastases - mixed lytic and sclerotic metastases

57. Bone involvement in metastases occurs by means of 3 main mechanisms : ( 1) direct extension (2 ) retrograde venous flow (3) seeding with tumor emboli via the blood circulation which is the most common route of spread.

58. Usually no periosteal reaction May appear as moth-eaten, permeative or geographic lesions Indistinct zones of transition, no sclerotic margins and may be sharply circumscribed or have indistinct borders Lesions distal to elbows and knees - 50% are from lung and breast Diffuse skeletal sclerosis or multiple round, well-circumscribed sclerotic lesions - Prostate & Breast Expansile and lytic (soap-bubbly) – R CC Cookie-bite lesions of the cortices of long bones – Lung Bone scans are extremely sensitive but not very specific 10-40% of lesions will not be visible on plain film but will be positive on bone scans CT or MRI can be used to show findings in patients with negative conventional radiographs and positive bone scans

59. SKELETAL METASTASES. LATERAL RADIOGRAPHS OF THE SKULL OF TWO DIFFERENT PATIENTS SHOW MULTIPLE ILL-DEFINED LYTIC LESIONS OF VARYING SIZES IN THE CALVARIUM

60. Metastasis from carcinoma prostate. AP radiograph of the femur shows OSTEOBLASTIC METASTASIS WITH EXTENSIVE ‘SUNBURST’ PERIOSTEAL REACTION resembling osteosarcoma

61. SKELETAL SCINTIGRAPHY IN METASTATIC DISEASE OF THE SKELETON SHOWS MULTIPLE RANDOMLY DISTRIBUTED FOCAL LESIONS SCATTERED THROUGHOUT THE SKELETON, PARTICULARLY THE SPINE, RIBS, AND PELVIS

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