malignant bone and soft-tissue tumors

Malignant Bone Malignant Bone TumorsTumors

Aaron KabbAaron Kabb

Chicago Medical SchoolChicago Medical School

August 2004August 2004

TopicsTopics

IntroductionIntroduction Imaging ModalitiesImaging Modalities Plain Film Radiographic FindingsPlain Film Radiographic Findings Malignant TumorsMalignant Tumors

IntroductionIntroduction

X-ray examination of the skeletal X-ray examination of the skeletal system is very common.system is very common.

Skeletal radiographs constitute the Skeletal radiographs constitute the second largest group of films seen in second largest group of films seen in a busy radiology practice.a busy radiology practice.

Primary malignant bone tumors are Primary malignant bone tumors are fortunately very rare, however, it is fortunately very rare, however, it is important for the radiologist to important for the radiologist to recognize bone tumors and provide a recognize bone tumors and provide a differential diagnosis.differential diagnosis.

Introduction cont.Introduction cont.

Approximately 2000 cases of Approximately 2000 cases of primary malignant tumors are primary malignant tumors are diagnosed each year in the US diagnosed each year in the US (excludes Multiple Myeloma).(excludes Multiple Myeloma).

(Contrast with the estimated (Contrast with the estimated 165,000 cases of Lung CA, 185,000 165,000 cases of Lung CA, 185,000 cases of Breast CA and 130,000 cases of Breast CA and 130,000 cases of Colon CA).cases of Colon CA).

Imaging ModalitiesImaging Modalities

Plain Radiograph-Plain Radiograph- Best modality for characterizing a bony Best modality for characterizing a bony

lesion as benign or malignant.lesion as benign or malignant. Many lesions have characteristic Many lesions have characteristic

appearances that allow for accurate appearances that allow for accurate diagnosis.diagnosis.

Provides the road map for further Provides the road map for further investigation and diagnosis.investigation and diagnosis.

CT scan-CT scan- Provides diagnostic information of bones Provides diagnostic information of bones

and soft tissue in another plane.and soft tissue in another plane.

Imaging ModalitiesImaging Modalities

CT scan cont.CT scan cont. Mainstay for safe and accurate biopsy Mainstay for safe and accurate biopsy

procedures.procedures. MRIMRI

Imaging procedure of choice for Imaging procedure of choice for determining the extent of a lesion, both determining the extent of a lesion, both in the skeleton and soft tissues.in the skeleton and soft tissues.

If resection of a tumor is contemplated, If resection of a tumor is contemplated, MRI should be performed.MRI should be performed.

Radiographic FindingsRadiographic Findings

Cortical DestructionCortical Destruction PeriostitisPeriostitis Orientation or Axis of the LesionOrientation or Axis of the Lesion Zone of transitionZone of transition

-the above criteria are used to -the above criteria are used to differentiate differentiate an aggressive process i.e., an aggressive process i.e., malignant tumor from malignant tumor from a benign process, but a benign process, but with varying accuracywith varying accuracy

-the above criteria apply to plain films -the above criteria apply to plain films and and

do not apply to CT or MRI in many do not apply to CT or MRI in many instancesinstances

Cortical DestructionCortical Destruction Cortical destruction usually makes one think of a Cortical destruction usually makes one think of a

malignant lesion when using the “gestalt malignant lesion when using the “gestalt approach”.approach”.

However, there are benign processes that can However, there are benign processes that can cause cortical destruction and mimic a malignant cause cortical destruction and mimic a malignant tumor.tumor.

These include:These include:- infection - infection -eosinophilic granuloma -eosinophilic granuloma -benign fibro-osseous lesions (radiolucent -benign fibro-osseous lesions (radiolucent

fibrous fibrous matrix replaces cortical bone)matrix replaces cortical bone)-aneurysmal bone cyst (thinning of the -aneurysmal bone cyst (thinning of the

cortex cortex makes cortex radiographically makes cortex radiographically undetectableundetectable

Cortical DestructionCortical Destruction

Notice in this benignNotice in this benign

Aneurysmal Bone Aneurysmal Bone CystCyst

how the thinned how the thinned cortex cortex

could be mistaken could be mistaken forfor

cortical destruction cortical destruction


Notice again in Notice again in this benign this benign chondroblastoma chondroblastoma the noncalcified the noncalcified chondroid tissue chondroid tissue replacing cortical replacing cortical bone. There is no bone. There is no cortical cortical destruction but destruction but rather replacementrather replacement


This radiograph This radiograph illustrates true illustrates true cortical cortical destruction seen in destruction seen in an osteosarcoma an osteosarcoma affecting the affecting the lateral femur lateral femur


Therefore, the presence of cortical Therefore, the presence of cortical destruction is not a reliable indicator destruction is not a reliable indicator of whether the lesion is a malignant of whether the lesion is a malignant process or a benign process.process or a benign process.

Other radiographic findings must Other radiographic findings must also be examined.also be examined.

PeriostitisPeriostitis

A periosteal reaction will occur A periosteal reaction will occur whenever the periosteum is whenever the periosteum is irritated.irritated.

This may occur due to a malignant This may occur due to a malignant tumor, benign tumor, infection or tumor, benign tumor, infection or trauma.trauma.

There are two types of periosteal There are two types of periosteal reaction, Benign or Aggressive.reaction, Benign or Aggressive.

Periostitis cont.Periostitis cont.

BenignBenign processes such as a slow growing processes such as a slow growing tumor will cause tumor will cause thick, wavy, uniformthick, wavy, uniform or or dense periostitis. Since it is a low grade, dense periostitis. Since it is a low grade, chronic irritation, the periosteum has time to chronic irritation, the periosteum has time to lay down thick new bone and remodel into a lay down thick new bone and remodel into a more normal appearing cortex.more normal appearing cortex.

AggressiveAggressive processes such as a malignant processes such as a malignant tumor cause a periosteal reaction that is more tumor cause a periosteal reaction that is more acute and high-grade. The periosteum does not acute and high-grade. The periosteum does not have time to consolidate. It appears have time to consolidate. It appears lamellated, “onion skinned”, amorphous or lamellated, “onion skinned”, amorphous or “sun-burst”.“sun-burst”.


When periostitis is seen the radiologist When periostitis is seen the radiologist should therefore try to classify it into should therefore try to classify it into benign or aggressive.benign or aggressive.

This classification also has its This classification also has its limitations.limitations.

These limitations are similar to those These limitations are similar to those seen with cortical destruction, such that seen with cortical destruction, such that benign processes such as infection or benign processes such as infection or eosinophilic granuloma can cause an eosinophilic granuloma can cause an aggressive periostitis.aggressive periostitis.

PeriostitisPeriostitis

However, seeing benign periostitis However, seeing benign periostitis can be very helpful, because can be very helpful, because malignant lesions CAN NOT cause malignant lesions CAN NOT cause benign periostitis. benign periostitis.

Periostitis cont.Periostitis cont. Notice the “onion Notice the “onion

skin” appearance skin” appearance of this aggressive of this aggressive periostitis seen in periostitis seen in Ewing’s sarcomaEwing’s sarcoma


Notice the Notice the “sunburst” pattern “sunburst” pattern seen in this seen in this aggressive aggressive Osteosarcoma of Osteosarcoma of the distal femur.the distal femur.


Notice the “thick” Notice the “thick” periostitis seen in periostitis seen in Eosinophilic Eosinophilic Granuloma, a non-Granuloma, a non-neoplastic neoplastic proliferation of proliferation of histiocytes. histiocytes. Remember, EG may Remember, EG may also cause an also cause an aggressive aggressive periostitisperiostitis


Therefore, the characterization of Therefore, the characterization of periostitis as Benign or Aggressive is periostitis as Benign or Aggressive is helpful in determining whether a helpful in determining whether a lesion is benign or malignant, but it lesion is benign or malignant, but it is not absolute. is not absolute.

Orientation or Axis of Orientation or Axis of lesionlesion

Lesions growing in the long axis of long Lesions growing in the long axis of long bones are said to be benign.bones are said to be benign.

Lesions growing in a circular orientation Lesions growing in a circular orientation are said to be malignant.are said to be malignant.

PoorPoor determinant of benign versus determinant of benign versus aggressive lesions.aggressive lesions.

Too many exceptions to each to be useful.Too many exceptions to each to be useful. For example, Ewing’s sarcoma, a For example, Ewing’s sarcoma, a

malignant lesion, usually has its axis malignant lesion, usually has its axis along the shaft of a long bone.along the shaft of a long bone.

Zone of TransitionZone of Transition Describes the border of the lesion with the Describes the border of the lesion with the

normal bone.normal bone. Most reliableMost reliable plain film indicator for benign plain film indicator for benign

versus malignant lesions.versus malignant lesions. ““Narrow”Narrow”, if it is so well defined that it can , if it is so well defined that it can

be drawn with a fine-point pen. A benign be drawn with a fine-point pen. A benign process should be considered as the most process should be considered as the most likely possibility.likely possibility.

““Wide”Wide”, if it is imperceptible and can not be , if it is imperceptible and can not be drawn at all. An aggressive process should drawn at all. An aggressive process should be considered, although not necessarily a be considered, although not necessarily a malignant lesion. malignant lesion.

Zone of TransitionZone of Transition

Zone of transition is always present Zone of transition is always present to evaluate, whereas many lesions, to evaluate, whereas many lesions, whether benign or malignant, will whether benign or malignant, will not necessarily show evidence of not necessarily show evidence of periostitis.periostitis.

Therefore Zone of Transition is the Therefore Zone of Transition is the most useful indicator of benign most useful indicator of benign versus malignant lesion.versus malignant lesion.


““Narrow”Narrow” Zone of Zone of Transition seen in Transition seen in a benign a benign nonossifying nonossifying fibroma. The fibroma. The margins of this margins of this lesion can be lesion can be drawn with a fine-drawn with a fine-point pen.point pen.


Permeative lesions Permeative lesions seen in this primary seen in this primary lymphoma have a lymphoma have a “Wide”“Wide” Zone of Zone of Transition and are Transition and are therefore therefore aggressive. Notice aggressive. Notice also the pathologic also the pathologic fracture seen in the fracture seen in the proximal humeral proximal humeral diaphysisdiaphysis

Summary of Radiographic Summary of Radiographic FindingsFindings

Cortical Destruction is less helpful than Cortical Destruction is less helpful than Periostitis in assessing whether a lesion Periostitis in assessing whether a lesion is benign or aggressive on plain film.is benign or aggressive on plain film.

Axis of a lesion is not helpful.Axis of a lesion is not helpful. Zone of Transition is the most reliable Zone of Transition is the most reliable

plain film indicator of benign versus plain film indicator of benign versus aggressive processes.aggressive processes.

If a lesion is aggressive, it is not If a lesion is aggressive, it is not necessarily malignant!necessarily malignant!

Malignant TumorsMalignant Tumors

Once it is decided that a lesion is Once it is decided that a lesion is malignant the differential diagnosis malignant the differential diagnosis should take into account the age of should take into account the age of the patient.the patient.

Jack Edeiken, a bone radiologist Jack Edeiken, a bone radiologist evaluated 4000 malignant bone evaluated 4000 malignant bone tumors and found that they could be tumors and found that they could be diagnosed correctly 80% of the time diagnosed correctly 80% of the time using the patient’s age.using the patient’s age.


Edeiken classified malignant tumors Edeiken classified malignant tumors into the following age groups:into the following age groups: 1-301-30: Ewing’s sarcoma, osteosarcoma.: Ewing’s sarcoma, osteosarcoma. 30-4030-40: Giant cell tumor, parosteal : Giant cell tumor, parosteal

sarcoma, fibrosarcoma, malignant sarcoma, fibrosarcoma, malignant fibrous histiocytoma, and reticulum cell fibrous histiocytoma, and reticulum cell sarcoma.sarcoma.

Over 40Over 40: Chondrosarcoma, metastatic : Chondrosarcoma, metastatic disease, myeloma.disease, myeloma.


The following malignant tumors will The following malignant tumors will be discussed:be discussed: Ewing’s SarcomaEwing’s Sarcoma OsteosarcomaOsteosarcoma Malignant Fibrous HistiocytomaMalignant Fibrous Histiocytoma ChondrosarcomaChondrosarcoma Metastatic Disease Metastatic Disease Multiple MyelomaMultiple Myeloma

Ewing’s SarcomaEwing’s Sarcoma

Primitive small and round blue cell Primitive small and round blue cell tumor possibly related to primitive tumor possibly related to primitive neuroectodermal cells.neuroectodermal cells.

Tend to occur in children and Tend to occur in children and adolescents ( adolescents ( 1-301-30 age group ). age group ).

Presentation of pain and a mass at Presentation of pain and a mass at the site of tumor with constitutional the site of tumor with constitutional symptoms including fever, anemia, symptoms including fever, anemia, leukocytosis, and an increased leukocytosis, and an increased erythrocyte sedimentation rate.erythrocyte sedimentation rate.

Ewing’s SarcomaEwing’s Sarcoma Most often permeative in appearance Most often permeative in appearance

( multiple small holes ).( multiple small holes ). Often have an “onion skin” type of Often have an “onion skin” type of

periostitis.periostitis. 40% of lesions occur in the diaphysis.40% of lesions occur in the diaphysis. Most commonly affects the femur.Most commonly affects the femur. Differential diagnosis should also include Differential diagnosis should also include

infection and eosinophilic granuloma.infection and eosinophilic granuloma. Treatment is in evolution and includes Treatment is in evolution and includes

neoadjuvant chemotherapy followed by neoadjuvant chemotherapy followed by wide resection and further chemotherapy.wide resection and further chemotherapy.

Ewing’s SarcomaEwing’s Sarcoma

OsteosarcomaOsteosarcoma

Most common primary malignant bone Most common primary malignant bone tumor. tumor.

Typically metaphyseal in location.Typically metaphyseal in location. More than half occur around the knee.More than half occur around the knee. Typically affects patients in their second or Typically affects patients in their second or

third decade, however, there is a second third decade, however, there is a second peak in patients >60 years old ( peak in patients >60 years old ( 1-301-30 age age group ).group ).

Patients present with pain, a mass or Patients present with pain, a mass or occasionally a pathologic fracture.occasionally a pathologic fracture.


Lesions are destructive in nature.Lesions are destructive in nature. Sclerosis is present from either Sclerosis is present from either

tumor new bone formation or tumor new bone formation or reactive sclerosis.reactive sclerosis.

Plain films reveal permeative lesion Plain films reveal permeative lesion with cortical destruction.with cortical destruction.


““Codman’s triangle” of bone appears Codman’s triangle” of bone appears as tumor elevates periosteum from as tumor elevates periosteum from underlying bone.underlying bone.

Cortical soft tissue extension may Cortical soft tissue extension may produce radiating spicules of bone produce radiating spicules of bone called “sunray” appearance.called “sunray” appearance.

Treatment includes chemotherapy Treatment includes chemotherapy and resection.and resection.


Mixed sclerotic Mixed sclerotic and lytic lesion of and lytic lesion of the proximal the proximal humerushumerus


Sclerotic lesion of Sclerotic lesion of the proximal tibiathe proximal tibia


““Sunburst” pattern Sunburst” pattern of distal femurof distal femur


Elevated Elevated periosteum periosteum described as described as “Codman’s “Codman’s triangle”triangle”

Malignant Fibrous Malignant Fibrous HistiocytomaHistiocytoma

Pleomorphic high grade tumor Pleomorphic high grade tumor composed of fibroblast, myofibroblasts composed of fibroblast, myofibroblasts and histiocytes.and histiocytes.

May also be considered a soft tissue May also be considered a soft tissue tumor.tumor.

Found in extremities 70-75% of the time.Found in extremities 70-75% of the time. Common in Common in 30-4030-40 age group. age group. Patients present with a painless mass of Patients present with a painless mass of

several months’ duration.several months’ duration.


Radiologically, they appear as lytic Radiologically, they appear as lytic lesions that may be permeative or lesions that may be permeative or fairly well defined.fairly well defined.

Periosteal reaction is not usually Periosteal reaction is not usually seen. seen.

Treatment is variable and includes Treatment is variable and includes chemotherapy and surgery.chemotherapy and surgery.


Soft tissue Soft tissue sarcoma invading sarcoma invading cortical bonecortical bone

ChondrosarcomaChondrosarcoma

Malignant cartilage forming tumor.Malignant cartilage forming tumor. Occurs in patients in the Occurs in patients in the Over 40Over 40 age group. age group. Affects men twice as common as women.Affects men twice as common as women. Common sites include pelvis (30%), proximal Common sites include pelvis (30%), proximal

and distal femur, ribs, proximal humerus, and distal femur, ribs, proximal humerus, and proximal tibia.and proximal tibia.

Patients present with pain or mass.Patients present with pain or mass. Treatment is excision with a wide margin, as Treatment is excision with a wide margin, as

these lesions are resistant to chemotherapy these lesions are resistant to chemotherapy and radiation.and radiation.


Plain film may show typical Plain film may show typical snowflake, or popcorn-like, snowflake, or popcorn-like, amorphous calcification.amorphous calcification.

Plain films may also show large Plain films may also show large osteolytic lesions.osteolytic lesions.

Difficult to distinguish between Difficult to distinguish between benign enchondroma and low grade benign enchondroma and low grade chondrosarcoma.chondrosarcoma.


Osteolytic lesion of Osteolytic lesion of the skullthe skull


Lesion affecting Lesion affecting the femur before the femur before and after surgical and after surgical repairrepair

Metastatic DiseaseMetastatic Disease

Most common malignancy in bone.Most common malignancy in bone. Must be considered in any differential Must be considered in any differential

diagnosis of a bone lesion in a patient diagnosis of a bone lesion in a patient Over 40Over 40 years old. years old.

May have virtually any appearance.May have virtually any appearance. May be lytic or blastic.May be lytic or blastic. Majority of metastases to bone originate Majority of metastases to bone originate

in Breast, Prostate, Lung, Kidney and in Breast, Prostate, Lung, Kidney and Thyroid.Thyroid.


Most common sites Most common sites for bony for bony metastases include metastases include thoracic and thoracic and lumbar spine, lumbar spine, pelvis, femur, rib, pelvis, femur, rib, proximal humerus proximal humerus and skulland skull


Bone mets from Bone mets from Lung CALung CA

Multiple MyelomaMultiple Myeloma

Tumor made up of malignant monoclonal Tumor made up of malignant monoclonal plasma cells.plasma cells.

Usually affects patients Usually affects patients Over 40Over 40 years of years of age.age.

Patients often present with malaise, bone Patients often present with malaise, bone pain, or a pathologic fracture.pain, or a pathologic fracture.

Classic radiographic appearance is Classic radiographic appearance is multiple lytic “punched out” areas in bone.multiple lytic “punched out” areas in bone.

Frequently involves the calvarium.Frequently involves the calvarium.


Lesions often do not show uptake of Lesions often do not show uptake of isotope on bone scan, making a isotope on bone scan, making a skeletal survey the most important skeletal survey the most important radiographic test.radiographic test.

Treatment consists of palliative Treatment consists of palliative chemotherapy or bone marrow chemotherapy or bone marrow transplant. transplant.


Characteristic Characteristic “punched-out” “punched-out” lesionslesions


““Punched-out” Punched-out” lesionlesion

ConclusionConclusion

Plain film findings of Cortical Plain film findings of Cortical Destruction, Periostitis and Zone of Destruction, Periostitis and Zone of Transition are helpful in assessing Transition are helpful in assessing benign versus aggressive lesions.benign versus aggressive lesions.

When combined with the age of the When combined with the age of the patient, and the location of the patient, and the location of the lesion, a reasonable differential lesion, a reasonable differential diagnosis can be formulated.diagnosis can be formulated.

ResourcesResources Brant, William E., Helms, Clyde A. Brant, William E., Helms, Clyde A.

Fundamentals of Diagnostic Radiology. 2Fundamentals of Diagnostic Radiology. 2ndnd ed. ed. 1999 Pgs 981-997.1999 Pgs 981-997.

Daffner, Richard H. Clinical Radiology The Daffner, Richard H. Clinical Radiology The Essentials. 1993 Pgs 271-321.Essentials. 1993 Pgs 271-321.

Brown, David E., Neuman, Randall D. Brown, David E., Neuman, Randall D. Orthopedic Secrets 3Orthopedic Secrets 3rdrd ed. 2004 Pgs 76-85. ed. 2004 Pgs 76-85.

Images from Bonetumors.org, Images from Bonetumors.org, Radiologyeducation.comRadiologyeducation.com

malignant bone and soft-tissue tumors

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