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Malignant Bone and Soft-Tissue Tumors

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  • 1. Malignant Bone Tumors Aaron Kabb Chicago Medical School August 2004
  • 2. Topics
    • Introduction
    • Imaging Modalities
    • Plain Film Radiographic Findings
    • Malignant Tumors
  • 3. Introduction
    • X-ray examination of the skeletal system is very common.
    • Skeletal radiographs constitute the second largest group of films seen in a busy radiology practice.
    • Primary malignant bone tumors are fortunately very rare, however, it is important for the radiologist to recognize bone tumors and provide a differential diagnosis.
  • 4. Introduction cont.
    • Approximately 2000 cases of primary malignant tumors are diagnosed each year in the US (excludes Multiple Myeloma).
    • (Contrast with the estimated 165,000 cases of Lung CA, 185,000 cases of Breast CA and 130,000 cases of Colon CA).
  • 5. Imaging Modalities
    • Plain Radiograph-
      • Best modality for characterizing a bony lesion as benign or malignant.
      • Many lesions have characteristic appearances that allow for accurate diagnosis.
      • Provides the road map for further investigation and diagnosis.
    • CT scan-
      • Provides diagnostic information of bones and soft tissue in another plane.
  • 6. Imaging Modalities
    • CT scan cont.
      • Mainstay for safe and accurate biopsy procedures.
    • MRI
      • Imaging procedure of choice for determining the extent of a lesion, both in the skeleton and soft tissues.
      • If resection of a tumor is contemplated, MRI should be performed.
  • 7. Radiographic Findings
    • Cortical Destruction
    • Periostitis
    • Orientation or Axis of the Lesion
    • Zone of transition
    • -the above criteria are used to differentiate an aggressive process i.e., malignant tumor from a benign process, but with varying accuracy
    • -the above criteria apply to plain films and
    • do not apply to CT or MRI in many instances
  • 8. Cortical Destruction
    • Cortical destruction usually makes one think of a malignant lesion when using the “gestalt approach”.
    • However, there are benign processes that can cause cortical destruction and mimic a malignant tumor.
    • These include:
    • - infection
    • -eosinophilic granuloma
    • -benign fibro-osseous lesions (radiolucent fibrous matrix replaces cortical bone)
    • -aneurysmal bone cyst (thinning of the cortex makes cortex radiographically undetectable
  • 9. Cortical Destruction
    • Notice in this benign
    • Aneurysmal Bone Cyst
    • how the thinned cortex
    • could be mistaken for
    • cortical destruction
  • 10. Cortical Destruction
    • Notice again in this benign chondroblastoma the noncalcified chondroid tissue replacing cortical bone. There is no cortical destruction but rather replacement
  • 11. Cortical Destruction
    • This radiograph illustrates true cortical destruction seen in an osteosarcoma affecting the lateral femur
  • 12. Cortical Destruction
    • Therefore, the presence of cortical destruction is not a reliable indicator of whether the lesion is a malignant process or a benign process.
    • Other radiographic findings must also be examined.
  • 13. Periostitis
    • A periosteal reaction will occur whenever the periosteum is irritated.
    • This may occur due to a malignant tumor, benign tumor, infection or trauma.
    • There are two types of periosteal reaction, Benign or Aggressive.
  • 14. Periostitis cont.
    • Benign processes such as a slow growing tumor will cause thick, wavy, uniform or dense periostitis. Since it is a low grade, chronic irritation, the periosteum has time to lay down thick new bone and remodel into a more normal appearing cortex.
    • Aggressive processes such as a malignant tumor cause a periosteal reaction that is more acute and high-grade. The periosteum does not have time to consolidate. It appears lamellated, “onion skinned”, amorphous or “sun-burst”.
  • 15. Periostitis cont.
    • When periostitis is seen the radiologist should therefore try to classify it into benign or aggressive.
    • This classification also has its limitations.
    • These limitations are similar to those seen with cortical destruction, such that benign processes such as infection or eosinophilic granuloma can cause an aggressive periostitis.
  • 16. Periostitis
    • However, seeing benign periostitis can be very helpful, because malignant lesions CAN NOT cause benign periostitis.
  • 17. Periostitis cont.
    • Notice the “onion skin” appearance of this aggressive periostitis seen in Ewing’s sarcoma
  • 18. Periostitis cont.
    • Notice the “sunburst” pattern seen in this aggressive Osteosarcoma of the distal femur.
  • 19. Periostitis cont.
    • Notice the “thick” periostitis seen in Eosinophilic Granuloma, a non-neoplastic proliferation of histiocytes. Remember, EG may also cause an aggressive periostitis
  • 20. Periostitis cont.
    • Therefore, the characterization of periostitis as Benign or Aggressive is helpful in determining whether a lesion is benign or malignant, but it is not absolute.
  • 21. Orientation or Axis of lesion
    • Lesions growing in the long axis of long bones are said to be benign.
    • Lesions growing in a circular orientation are said to be malignant.
    • Poor determinant of benign versus aggressive lesions.
    • Too many exceptions to each to be useful.
    • For example, Ewing’s sarcoma, a malignant lesion, usually has its axis along the shaft of a long bone.
  • 22. Zone of Transition
    • Describes the border of the lesion with the normal bone.
    • Most reliable plain film indicator for benign versus malignant lesions.
    • “ Narrow” , if it is so well defined that it can be drawn with a fine-point pen. A benign process should be considered as the most likely possibility.
    • “ Wide” , if it is imperceptible and can not be drawn at all. An aggressive process should be considered, although not necessarily a malignant lesion.
  • 23. Zone of Transition
    • Zone of transition is always present to evaluate, whereas many lesions, whether benign or malignant, will not necessarily show evidence of periostitis.
    • Therefore Zone of Transition is the most useful indicator of benign versus malignant lesion.
  • 24. Zone of Transition
    • “ Narrow” Zone of Transition seen in a benign nonossifying fibroma. The margins of this lesion can be drawn with a fine-point pen.
  • 25. Zone of Transition
    • Permeative lesions seen in this primary lymphoma have a “Wide” Zone of Transition and are therefore aggressive. Notice also the pathologic fracture seen in the proximal humeral diaphysis
  • 26. Summary of Radiographic Findings
    • Cortical Destruction is less helpful than Periostitis in assessing whether a lesion is benign or aggressive on plain film.
    • Axis of a lesion is not helpful.
    • Zone of Transition is the most reliable plain film indicator of benign versus aggressive processes.
    • If a lesion is aggressive, it is not necessarily malignant!
  • 27. Malignant Tumors
    • Once it is decided that a lesion is malignant the differential diagnosis should take into account the age of the patient.
    • Jack Edeiken, a bone radiologist evaluated 4000 malignant bone tumors and found that they could be diagnosed correctly 80% of the time using the patient’s age.
  • 28. Malignant Tumors
    • Edeiken classified malignant tumors into the following age groups:
      • 1-30 : Ewing’s sarcoma, osteosarcoma.
      • 30-40 : Giant cell tumor, parosteal sarcoma, fibrosarcoma, malignant fibrous histiocytoma, and reticulum cell sarcoma.
      • Over 40 : Chondrosarcoma, metastatic disease, myeloma.
  • 29. Malignant Tumors
    • The following malignant tumors will be discussed:
      • Ewing’s Sarcoma
      • Osteosarcoma
      • Malignant Fibrous Histiocytoma
      • Chondrosarcoma
      • Metastatic Disease
      • Multiple Myeloma
  • 30. Ewing’s Sarcoma
    • Primitive small and round blue cell tumor possibly related to primitive neuroectodermal cells.
    • Tend to occur in children and adolescents ( 1-30 age group ).
    • Presentation of pain and a mass at the site of tumor with constitutional symptoms including fever, anemia, leukocytosis, and an increased erythrocyte sedimentation rate.
  • 31. Ewing’s Sarcoma
    • Most often permeative in appearance ( multiple small holes ).
    • Often have an “onion skin” type of periostitis.
    • 40% of lesions occur in the diaphysis.
    • Most commonly affects the femur.
    • Differential diagnosis should also include infection and eosinophilic granuloma.
    • Treatment is in evolution and includes neoadjuvant chemotherapy followed by wide resection and further chemotherapy.
  • 32. Ewing’s Sarcoma
  • 33. Ewing’s Sarcoma
  • 34. Osteosarcoma
    • Most common primary malignant bone tumor.
    • Typically metaphyseal in location.
    • More than half occur around the knee.
    • Typically affects patients in their second or third decade, however, there is a second peak in patients >60 years old ( 1-30 age group ).
    • Patients present with pain, a mass or occasionally a pathologic fracture.
  • 35. Osteosarcoma
    • Lesions are destructive in nature.
    • Sclerosis is present from either tumor new bone formation or reactive sclerosis.
    • Plain films reveal permeative lesion with cortical destruction.
  • 36. Osteosarcoma
    • “ Codman’s triangle” of bone appears as tumor elevates periosteum from underlying bone.
    • Cortical soft tissue extension may produce radiating spicules of bone called “sunray” appearance.
    • Treatment includes chemotherapy and resection.
  • 37. Osteosarcoma
    • Mixed sclerotic and lytic lesion of the proximal humerus
  • 38. Osteosarcoma
    • Sclerotic lesion of the proximal tibia
  • 39. Osteosarcoma
    • “ Sunburst” pattern of distal femur
  • 40. Osteosarcoma
    • Elevated periosteum described as “Codman’s triangle”
  • 41. Malignant Fibrous Histiocytoma
    • Pleomorphic high grade tumor composed of fibroblast, myofibroblasts and histiocytes.
    • May also be considered a soft tissue tumor.
    • Found in extremities 70-75% of the time.
    • Common in 30-40 age group.
    • Patients present with a painless mass of several months’ duration.
  • 42. Malignant Fibrous Histiocytoma
    • Radiologically, they appear as lytic lesions that may be permeative or fairly well defined.
    • Periosteal reaction is not usually seen.
    • Treatment is variable and includes chemotherapy and surgery.
  • 43. Malignant Fibrous Histiocytoma
    • Soft tissue sarcoma invading cortical bone
  • 44. Chondrosarcoma
    • Malignant cartilage forming tumor.
    • Occurs in patients in the Over 40 age group.
    • Affects men twice as common as women.
    • Common sites include pelvis (30%), proximal and distal femur, ribs, proximal humerus, and proximal tibia.
    • Patients present with pain or mass.
    • Treatment is excision with a wide margin, as these lesions are resistant to chemotherapy and radiation.
  • 45. Chondrosarcoma
    • Plain film may show typical snowflake, or popcorn-like, amorphous calcification.
    • Plain films may also show large osteolytic lesions.
    • Difficult to distinguish between benign enchondroma and low grade chondrosarcoma.
  • 46. Chondrosarcoma
    • Osteolytic lesion of the skull
  • 47. Chondrosarcoma
    • Lesion affecting the femur before and after surgical repair
  • 48. Metastatic Disease
    • Most common malignancy in bone.
    • Must be considered in any differential diagnosis of a bone lesion in a patient Over 40 years old.
    • May have virtually any appearance.
    • May be lytic or blastic.
    • Majority of metastases to bone originate in Breast, Prostate, Lung, Kidney and Thyroid.
  • 49. Metastatic Disease
    • Most common sites for bony metastases include thoracic and lumbar spine, pelvis, femur, rib, proximal humerus and skull
  • 50. Metastatic Disease
    • Bone mets from Lung CA
  • 51. Multiple Myeloma
    • Tumor made up of malignant monoclonal plasma cells.
    • Usually affects patients Over 40 years of age.
    • Patients often present with malaise, bone pain, or a pathologic fracture.
    • Classic radiographic appearance is multiple lytic “punched out” areas in bone.
    • Frequently involves the calvarium.
  • 52. Multiple Myeloma
    • Lesions often do not show uptake of isotope on bone scan, making a skeletal survey the most important radiographic test.
    • Treatment consists of palliative chemotherapy or bone marrow transplant.
  • 53. Multiple Myeloma
    • Characteristic “punched-out” lesions
  • 54. Multiple Myeloma
    • “ Punched-out” lesion
  • 55. Conclusion
    • Plain film findings of Cortical Destruction, Periostitis and Zone of Transition are helpful in assessing benign versus aggressive lesions.
    • When combined with the age of the patient, and the location of the lesion, a reasonable differential diagnosis can be formulated.
  • 56. Resources
    • Brant, William E., Helms, Clyde A. Fundamentals of Diagnostic Radiology. 2 nd ed. 1999 Pgs 981-997.
    • Daffner, Richard H. Clinical Radiology The Essentials. 1993 Pgs 271-321.
    • Brown, David E., Neuman, Randall D. Orthopedic Secrets 3 rd ed. 2004 Pgs 76-85.
    • Images from Bonetumors.org, Radiologyeducation.com