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Bone Tumors
Osteosarcoma (Conventional)


James C. Wittig, MD
Sarcoma Surgeon
Orthopedic Oncologist    
    


GENERAL INFORMATION

An osteosarcoma is a cancerous spindle cell tumor (sarcoma) that is derived from a mesenchymal stem cell precursor. The malignant spindle cells produce immature woven bone or osteoid which is why the tumor is named osteosarcoma. It is a bone producing sarcoma. A sarcoma that produces bone is by definition an osteosarcoma.

Osteosarcomas most commonly arise from bones although they can also rarely arise from soft tissues of the extremities (muscles and connective tissues of the extremities). 

Spindle Cells are cigar shaped cells in appearance.

An osteosarcoma is a cancerous spindle cell tumor (sarcoma) that is derived from a mesenchymal stem cell precursor. The malignant spindle cells produce immature woven bone or osteoid which is why the tumor is named osteosarcoma. It is a bone producing sarcoma. A sarcoma that produces bone is by definition an osteosarcoma.

Osteosarcomas most commonly arise from bones although they can also rarely arise from soft tissues of the extremities (muscles and connective tissues of the extremities).
Spindle Cells are cigar shaped cells in appearance.

There are many types of osteosarcomas.
The most common is called Conventional Osteosarcoma.
In general osteosarcomas can be classified into three general categories according to where they arise from the bone:
          Intramedullary: Most Common; Arise from middle of bone (medullary canal)
          Surface/Juxtacortical: Arise from the surface or periosteum of the bone
          Intracortical: Arise from within the cortex of the bone
          Intracortical osteosarcomas are extremely rare

Osteosarcoma Classification (Types of Osteosarcoma)

 

Intramedullary (75%)
Conventional
Osteoblastic (82%)
Mixed and Sclerosing
Chondroblastic (5%)
Fibroblastic (3-4%)
MFH-like (3-4%)
Osteoblastoma-like (.5%)
Giant Cell-rich (.5%)
Small-cell (1%)
Epithelioid (.5%)                                                   
Telangiectatic (3%)

Juxtacortical/Surface (7-10%)
Parosteal
Periosteal
High-grade surface
Intracortical (.2%)
Secondary (older population)
Pagets (67-90%); Post RT (6-22%); Bone infarct; Fibrous dysplasia; Metallic implant; Osteomyelitis
OS with specific syndromes
Familial; Retinoblastoma; Rothmund-Thomson
Syndrome; Multifocal; OI

Osteosarcoma is the second most common primary malignant tumor of bone
It is the most common primary cancer of bone in children and adolescents
Osteosarcomas represent 15% of all biopsied primary bone tumors
There are approximately 600 to 700 new cases of osteosarcoma in the United States per year
Osteosarcomas are more common in children than adults
The most common type of osteosarcoma is the Primary, High Grade, Intramedullary (Conventional) Osteosarcoma Represents approximately 75% of all osteosarcomas

Definitions:
Primary Osteosarcoma: arises from the bone in the absence of a benign precursor lesion or treatment
Secondary Osteosarcoma: arises from a precursor lesion or one that is metastatic from a primary osteosarcoma
Synchronous Osteosarcoma: Lesions that affect multiple bones discovered within 6 mos of each other
Metachronous Osteosarcoma: Lesions involving multiple bones discovered more than 6 mos apart


CLINICAL PRESENTATION

Signs/Symptoms associated with an osteosarcoma:

Mild Pain for weeks-months
Pain gradually becomes more severe & accompanied by swelling and limitation of motion
Weight loss is correlated with disseminated disease
Blood tests may demonstrate a high serum alkaline phosphatase

Prevalence:
Slightly more common in males than females (ratio:1.5-2:1) possibly due to longer period of male skeletal growth

Age of developing an osteosarcoma:
Two peak age groups (rare <6y or >60y)

First peak: 15-25 years: Most common age is in childhood and adolescence

Second peak (much smaller) – just over 50 years: Less common in adults

When osteosarcomas occur in adults there is often an underlying predisposing condition such as a history of radiation to the bone, Paget's Disease; Underlying bone infarct
When an osteosarcoma arises from a pre-existing condition of bone it is called a secondary osteosarcoma

Sites of developing an osteosarcoma:
Long Bones:
Most common site (70%-80% of cases);
Most (90%)arise from the metaphysis of the bone and 10 % arise from the diaphysis. Thus in most instances the tumor arises next to a joint. 
Distal Femur: most common site (40%; about twice as common as proximal tibia) 
Proximal Tibia: Second most common site
Proximal Humerus: Third most common site
Axial Skeleton: Pelvis, Spine are much less common sites

Most patients present with grossly detectable (detectable with radiology studies) disease isolated to the extremity. This means that with conventional X-rays, CT scans, MRIs the tumor can only be detected in the extremity (leg, arm, pelvis or spine)

The most common sites for developing metastases are:
Lungs: Most common site
CT of the chest is used for detecting lung metastases

Bones: Second most common site
A whole body bone scan is used for detecting bone metastases

Liver: Rare site
Approximately 15%-20% of patients present with detectable metastases to the lungs. Fewer patients present with bone metastases

Most patients with osteosarcomas do not develop bone metastases without developing lung metastases first. Rarely however bone metastases occur without lung metastases.

Skip metastases are metastases that occur within the same bone as the primary osteosarcoma or across the joint in the adjacent bone. They occur through the intraosseous venous system within the bone or through the transarticular venous system. Intraosseous and transarticular skip metastases occur rarely and may occur without any evidence of pulmonary metastases. They have traditionally been associated with a poor prognosis although recent reports may suggest otherwise. The detection of skip metastases is important to plan surgery. Occasionally skip metastases may dictate that the entire bone be removed.


MRI: Osteosarcoma of Distal Femur with Skip Metastasis to Proximal Femur


HISTORICAL TREATMENT OF OSTEOSARCOMA

Many years ago, before chemotherapy was developed, patients with an osteosarcoma isolated to the extremity (no evidence of pulmonary metastases on chest X-rays) were treated with an amputation. Despite an amputation, 83% of patients developed pulmonary (lung) metastases and died of the disease. Only 17% of patients were cured with an amputation and these were patients with very small tumors that were detected early. This meant that although there was no detectable disease in the lungs at the time of presentation that patients had microscopic disease in their lungs that ultimately grew and resulted in death. Performing an amputation got rid of the disease in the extremity but did not do anything about the microscopic cells in the lungs that ultimately grew to form metastases after the amputation was performed.

Chemotherapy was developed and utilized to eradicate the microscopic disease in the lungs. When patients with isolated high grade osteosarcomas were treated with chemotherapy and surgery, the micrometastatic disease (microscopic cells) in the lungs was eradicated and most patients were capable of being cured.

Over the course of time surgeons found that they were able to also save extremities (arms and legs) of patients with high grade osteosarcomas especially when the chemotherapy was administered before surgery (called preoperative or neoadjuvant chemotherapy). Chemotherapy was given preoperatively which killed the main tumor and made it easier to save extremities instead of performing an amputation. At the same time, the chemotherapy was killing any microscopic disease in the lungs.

When patients were treated with preoperative chemotherapy, their tumors were studied after being removed. It was found that patients who had a good response to preoperative chemotherapy (at that time if approximately 90% of the tumor was dead) had a better prognosis or chance for being cured than those patients who did not have a good response although all patients had a better prognosis than if they did not receive chemotherapy at all. In general, different centers use different percentages of tumor necrosis, induced by preoperative chemotherapy, when attempting to predict prognosis. Some centers believe 90% tumor necrosis from preoperative chemotherapy confers a better prognosis (85%-90% cure rate) while others believe that 99% tumor necrosis confers a better prognosis. This is a controversial subject.



RADIOLOGY

Xray (plain radiographs) of the extremity
MRI with contrast (gadolinium) of the extremity
Best for determining intraosseous extent (size) of the tumor and size of the soft tissue component
Best for determining relationship of tumor to neurovascular structures
Best for detecting skip metastases
Entire bone and adjacent joint should be visualized
             Not good for determining response to preoperative chemotherapy

CT (CAT) Scan of the tumor/extremity
Complimentary to MRI
Useful for detecting subtle tumor calcification and ossification diagnostic of an osteosarcoma
Useful for evaluating cortical bone integrity and penetration beyond the cortex
Useful for evaluating tumor extent when there is extreme edema on the MRI
Useful for evaluating response to preoperative chemotherapy  (pre-chemo CT compared to post chemo CT before surgery)

CT Scan of the Chest: Used to detect pulmonary metastases

Whole Body Bone Scan: Used to detect bone metastases; extent of bony involvement by the tumor and presence of skip metastases
The bone scan should be correlated with the MRI and CT scan

Thallium Scan: special type of bone scan useful for evaluating response to preoperative chemotherapy
Not routinely ordered in all centers

CT PET Scan of Whole Body: Not yet demonstrated to be reliable for detecting metastases nor for evaluating response to preoperative chemotherapy before surgery

Angiography:
Not routinely performed; Indicated in selected circumstances
Angiography involves injection of contrast directly into the main artery of the extremity and then taking X-rays of the blood vessels in the area of the tumor and above and below the tumor. Angiography is the gold standard for evaluating response to preoperative chemotherapy. Osteosarcomas that have had a good response to preoperative chemotherapy do not demonstrate significant uptake of the contrast dye whereas viable tumors (blood supply maintained to tumor) fill with the dye and demonstrate a vascular tumor blush on the arteriogram/angiogram)
Angiography is also useful for determining proximity of the tumor to the blood vessels in the extremity and for showing how the blood vessels are displaced or moved by the tumor.


TREATMENT/SURGERY

Almost all osteosarcomas are high grade tumors (meaning they grow quickly and have a high likelihood of spreading/metastasizing). The exceptions are well differentiated intraosseous osteosarcomas and parosteal osteosarcomas which are both low grade tumors (these grow slowly and rarely spread/metastasize).

Conventional osteosarcomas as well as all the other high grade osteosarcomas are treated with a combination of chemotherapy and surgery. Usually chemotherapy is administered before and after surgery. Chemotherapy administered before surgery is called preoperative/neoadjuvant or induction chemotherapy. Chemotherapy administered after surgery is called adjuvant chemotherapy.

Radiation is rarely recommended for treating osteosarcomas. It is used mostly in pelvic and spine cases when the surgeon can not achieve a wide margin. Some of the decision to give radiation in these cases depends on the size of the tumor and its response to preoperative chemotherapy.

Low grade osteosarcomas are usually treated with surgery alone (parosteal osteosarcoma; well differentiated intramedullary osteosarcoma). Sometimes low grade tumors dedifferentiate or develop high grade areas when they are present for prolonged periods of time. When this occurs, it may not be detected until the entire tumor is removed. In this case chemotherapy may be indicated after surgery.

Periosteal Osteosarcomas are intermediate grade tumors. In most cases, the treatment of these tumors follows that of conventional osteosarcomas
.


SURGERY

Today, most patients with an osteosarcoma of an extremity are capable of being treated with limb sparing surgery and reconstruction instead of an amputation.

Much of the success is do to several factors:

~    Preoperative chemotherapy induces necrosis in the tumor that enables the body to initiate a healing response. Edema decreases and less soft tissue needs to be removed with the tumor. It is easier to determine normal vs. abnormal tissue intraoperatively. The tumor may not shrink because of the presence of osteoid and bone however a significant number of malignant cells may be killed.
~    Advanced radiological imaging modalities (MRI and CT) that help determine the exact extent of the tumor and involvement of neurovascular structures before surgery.
~    Advances in prosthetic design and surgical technique

The type of limb sparing resection for an osteosarcoma of the bone is termed a wide or radical resection. After resection, the bone and or joint is reconstructed along with the soft tissues. There are various methods of reconstruction. One of the more favorable and common methods is a prosthetic reconstruction with a metallic, modular segmental tumor prosthesis. Osteosarcomas most frequently occur next to joints and therefore the neighboring joint often also requires resection and reconstruction. Allografts, free vascularized fibula transfers, bone grafts also have been used for selected cases.

Contraindications for limb sparing surgery for osteosarcomas include:
Inappropriately performed biopsy that contaminates significant surrounding soft tissues (#1 cause for requiring an amputation)
Infected Osteosarcoma
Direct invasion or encasement of important neurovascular structures (relative contraindication)
Occasionally the blood vessels can be removed with the tumor and reconstructed with a graft; there are high complication rates with this procedure and many patients ultimately lose the extremity
Occasionally a nerve may need to be resected with the tumor and the patient can still have a functional extremity
Pathological fracture (Relative contraindication)
If the pathological fracture can be stabilized nonoperatively and heals with preoperative chemotherapy it may be safe (may not compromise survival) to perform limb sparing surgery as long as the contaminated tissue from fracture hematoma can be removed.
An extremely large tumor where resection with a wide margin would result in a useless extremity


RADIOGRAPHIC PRESENTATION CONVENTIONAL OSTEOSARCOMA

There are 3 radiographic presentations for osteosarcomas depending upon the amount of osteoid/ossification and calcium deposition associated with the tumor
Mixed Sclerotic and Lytic Permeative Lesion
Most common radiographic presentation

Purely Osteoblastic Permeative Lesion: Dense sclerosis and osteoid production

Purely Lytic Permeative Lesion: Little osteoid production and/or minimal calcium deposition in osteoid

Conventional Osteosarcomas are permeative lesions on plain radiographs meaning that the borders of the lesion can not be clearly delineated
Wide Zone of Transition from lytic/sclerotic areas of Tumor to normal bone
       Makes borders of lesion hard to define
Most (90%) arise from the metaphysis of the bone
Rarely (10%) arise from the diaphysis
Most conventional osteosarcomas (90-95%) extend through the bone into the soft tissues and form a soft tissue mass outside of the bone
.



X-RAY:  OSTEOSARCOMA OF PROXIMAL HUMERUS
Permative Lesion
Metaphyseal Origin
Mixed Lysis and Sclerosis
Sclerosis represents calcified osteoid
Most common radiographic presentation




X-RAY:  OSTEOSARCOMA OF PROXIMAL HUMERUS
Permeative Lesion
Metaphyseal
Cortical Destruction
Purely Lytic
Malignant Appearance


X-RAY:  BLASTIC OSTEOSARCOMA OF PROXIMAL HUMERUS
Permeative Lesion
Metaphyseal Origin
Purely Blastic
Heavily Calcified Osteoid



X-RAY: CONVENTIONAL OSTEOSARCOMA OF DISTAL FEMUR
Classic radiographic example of a conventional osteosarcoma of the distal femur
Distal Femur is most common site for a conventional osteosarcoma
Permeative lesion with mixed lysis and sclerosis (sclerosis is calcified osteoid)
Metaphyseal Origin
There is a Codman's triangle interrupted type of periosteal reaction
Tumor extends into soft tissue and the soft tissue component is ossified



X-RAY:  OSTEOSARCOMA OF DISTAL FEMUR
Permeative Lesion
Mixed Lysis and Sclerosis
Metadiaphyseal Origin
Ossified Soft Tissue Mass (white arrows)
Codman’s Triangle periosteal Reaction


X-RAY:  Osteosarcoma of Distal Femur
This particular osteosarcoma is barely perceptible on the Xray
Highly permeative with subtle lysis and sclerosis
It protrudes slightly from the bone where it is ossified
There is an adjacent Codman's Triangle
MRI demonstrates this osteosarcoma better than the Xray



X-RAY:  Lateral Xray of same patient the osteosarcoma is barely perceptible.

        
MRI of previous patient with Osteosarcoma of Distal Femur
The Osteosarcoma is much more clearly demonstrated on the MRI than on the previous X-Rays


 




X-RAY:  Osteosarcoma of Proximal Tibia

Proximal tibia is second most common site for conventional osteosarcoma
Permeative lesion with mixed lysis and sclerosis (ossification)
Metaphyseal origin
Soft tissue extension
Hair on End periosteal reaction


X-RAY:  Proximal Tibia Osteoarcoma Subtle Hair on End Periosteal Reaction


X-RAY:  Large Osteosarcoma of Proximal Tibia with Large Ossified Soft Tissue Component



X-RAY:  Large Osteosarcoma of Proximal Femur with Heavily Ossified Soft Tissue Component



X-RAY:  Osteosarcoma of Left Humerus Diaphyseal Osteosarcoma

Permeative Tumor
Diaphyseal Origin
Only 10% of osteosarcomas arise from the diaphysis
Tumor has extended into surrounding soft tissues and formed a mass that is producing osteoid (fluffy cloudlike densities)
Ossification of mass and within bone
Pathological Fracture is present (10% of osteosarcomas present with a pathological fracture)


X-RAY:  Osteosarcoma of Fibula Diaphysis

The plain Xray demonstrates a permeative lesion arising from the fibula
There is a soft tissue component (arrows) with fluffy cloudlike ossification indicative of an osteosarcoma
The fibula is a rare site for developing an osteosarcoma


GROSS PATHOLOGY:  Conventional Osteosarcoma

These are examples of the gross pathology specimens after the conventional osteosarcoma was resected. The gross specimens consist of a combination of bony and soft tissue areas. X-rays of the specimens are included.
Osteosarcomas are composed of ossified or non-ossified tissue
Ossified tissue is yellow-white and hard
Less ossified tissue is soft and less yellow
Non-ossified tissue is tan and fleshy

Most (95%) of conventional osteosarcomas penetrate the cortex and form a large extraosseous soft tissue mass
The lesion is permeative and permeates the marrow spaces
Osteosarcomas usually infiltrate the marrow several centimeters away from the main tumor mass
Skip lesions may be apparent that are separated from the main tumor by normal marrow
Osteosarcomas may also have cartilaginous components that appear as translucent lobules and fibrous components that are tan, soft to firm rubbery areas
Osteoblastic areas are usually white to yellow, firm, hard and gritty
The consistency of the tumor depends on the amount of osteoid deposition, cartilaginous and fibrous areas.
Foci of hemorrhage and necrosis are common
Periosteal reactions such as Codman's Triangle are apparent at periphery of soft tissue mass
Osteosarcomas rarely penetrate the growth plate grossly
Invasion of the joint is uncommon but can occur by cortical penetration, joint capsule extension, or extension along cruciate ligaments


Gross Pathology:  Osteosarcoma of Distal Femur


Gross Pathology:  Conventional Osteosarcoma of Distal Femur

    


Gross Pathology:  Conventional Osteosarcoma of Proximal Tibia


Gross Pathology:  Osteosarcoma of Proximal Tibia

Gross Pathology:  Osteosarcoma of Proximal Humerus
The osteosarcoma originates from the metaphysis of the proximal humerus and extends into the surrounding soft tissues
There is a large soft tissue component that is crossing the glenohumeral joint
This osteosarcoma was removed via an extra-articular resection that included the scapula (Tikhoff-Linberg resection)


MICROSCOPIC PATHOLOGY:  Conventional Osteosarcoma

High Grade Anaplastic Tumor
Hypercellular, spindle cell tumor with extensive pleomorphism (cells are different sizes and shapes)
Large nuclei and little cytoplasm (high nuclear
to cytoplasmic ratio)
Many mitotic figures and abnormal mitoses
Bizarre appearing cells with hyperchromatic nuclei
Osteoid Production
Osteoid is often layed down in lace-like pattern in between the malignant cells
Osteoid stains pink to red with H and E stains
There is no osteoblastic rimming (the cells do not line up on the surface of a trabecula of osteoid as in osteoblastoma or osteoid osteoma)
The osteoid may or may not mineralize. The degree of mineralization determines how well it shows on an Xray
May have other elements such as cartilage, fibrous tissue, small round blue cells, giant cells and telangiectatic changes

Microscopic Pathology:  Conventional Osteosarcoma


Microscopic Pathology:  Conventional Osteosarcoma

 


Microscopic Pathology:  High Power of Conventional Osteosarcoma

Fibrosarcoma subtype of conventional osteosarcoma may show spindle cells that arrange themselves in a "Herringbone" pattern

Malignant Fibrous Histiocytoma (MFH) subtype of conventional osteosarcoma often demonstrates a "Storiform" pattern arrangement of cells. There are pleomorphic spindle cells mixed with large, bizarre cells.

Giant cell rich conventional osteosarcomas may have large sheets of reactive giant cells that may obscure the underlying sarcoma.

Osteoid production may be sparse.
Preoperative chemotherapy changes the microscopic appearance of conventional osteosarcomas. Necrotic osteoblastic foci appear as acellular osteoid matrix. The cells are killed by the chemotherapy but the osteoid remains. Fibrosarcomatous areas are replaced by collagen and scar tissue, granulation tissue and inflammatory cells.

Chondrobalstic foci will have "ghost cells" in lacunae (acellular chondroid tissue)

Differential Diagnosis (Pathologic Differential)
Entities in Differential Diagnosis
Osteoblastoma
Osteoid Osteoma
Fracture Callus
Giant Cell Tumor
"Dedifferentiated" Chondrosarcoma
Malignant Fibrous Histiocytoma (MFH)
Fibrosarcoma of Bone


DIFFERENTIAL DIAGNOSIS (Pathologic Differential)
Entities in Pathological Differential Diagnosis
Osteoblastoma (Benign Bone Forming Tumor)
Osteoblastoma is usually a geographic benign appearing lesion on

Xray
Osteosarcoma is usually permeative on Xray
Osteoblastomas have thick, irregular trabeculae of osteoid and woven bone with osteoblastic rimming
Trabeculae of osteoblastoma are separated by intervening stroma with capillaries and osteoclasts/giant cells
Osteosarcomas infiltrate surrounding lamellar bone whereas osteoblastomas grow with a pushing margin. There is a sharp cut off between the osteoblastoma and normal bone at the periphery of the osteoblastoma. Osteoblastomas do not permeate the surrounding lamellar bone.
There is no cartilage in an osteoblastoma unless it has fractured the bone or the lesion has been biopsied.
Osteoid Osteoma
Fracture Callus
Giant Cell Tumor

"Dedifferentiated" Chondrosarcoma
Dedifferentiated chondrosarcoma consists of a low grade cartilaginous component with a high grade anaplastic component producing osteoid. The high grade anaplastic component is sharply cut off from the low grade cartilaginous component.
Chondroblastic osteosarcoma contains high grade cartilage admixed with osseous component
The Xray of a dedifferentiated chondrosarcoma shows a separate distinct cartilaginous component.
Malignant Fibrous Histiocytoma (MFH)
Fibrosarcoma of Bone

Osteosarcoma vs. Osteoblastoma

     


Osteoblastoma Benign Bone Forming Tumor Well Formed trabeculae lined by Osteoblasts


Osteoblastoma


TREATMENT

Treatment is often dictated by National Children’s Oncology

Group Protocols (COG Protocols)
Preoperative (induction) chemotherapy:
Adriamycin (doxorubicin)
Cisplatinum (cisplatin)
High Dose Methotrexate (HDMTX)
Ifosfamide/Etoposide in some regimens
(Typically: 2 to 3 cycles and then surgery)

Surgery:
Wide surgical resection/limb Salvage (Most extremity lesions can usually be treated with Limb Salvage when detected early)
Amputation (5% of extremity lesions)
Postoperative (adjuvant) chemotherapy:
Same regimen as preop; usually 4 cycles

PREOPERATIVE (NEOADJUVANT) CHEMOTHERAPY
Preoperative chemotherapy essentially kills the main osteosarcoma
and essentially makes it easier to perform a limb sparing surgery instead of an amputation. At the same time the chemotherapy kills cells that may have traveled to the lungs.
Preoperative chemotherapy is one of the main reasons limb sparing surgery can be performed in the majority of patients with an osteosarcoma.
The tumor may not actually shrink much because of the presence of osteoid however when the cells of the tumor are killed the body begins to heal against the tumor. Normal tissue is more clearly delineated from the tumor and abnormal tissue.
Radiological studies may be useful in determining if the osteosarcoma has had a good response to chemotherapy preoperatively
X-rays and CT scans may show that intense ossification of the tumor occurs after a good response to preoperative chemotherapy.
The CT scan will often show a peripheral zone of calcification (egg shell around the tumor) when the tumor has had a good response.
MRI is not useful for evaluating response of osteosarcomas to preoperative chemotherapy.
Bone Scan and/or Thallium scan may be useful. Decreased uptake after preoperative chemotherapy compared to the uptake on initial diagnosis may be indicative of a good response.
Formal angiograms where dye is injected into the blood vessels of the extremity demonstrate the vascularity of the tumor. Viable tumors have significant blood vessels and vascularity and therefore fill with the dye, The dye causes a tumor blush. If an osteosarcoma has had a good response the vascularity will disappear and there will be little to no tumor blush. The angiogram is the gold standard for evaluating response to preoperative chemotherapy before surgery however is not routinely performed because it is an invasive test.

Estimating response of the osteosarcoma to preoperative chemotherapy before the actual surgical procedure may help with surgical planning and may also help in deciding whether a limb sparing surgery or an amputation should be performed.  It is often not necessary to remove as much surrounding normal tissue around an osteosarcoma when the tumor has demonstrated a good response as opposed to those tumors that do not demonstrate a good response.
The final estimate of response to preoperative chemotherapy occurs when the specimen is analyzed by the pathologist. This estimate helps predict prognosis. A "Good Response" (usually greater than 90% of the tumor killed) has been correlated with approximately a 90% cure rate.

X-rays of a Proximal Humerus Osteosarcoma: before and after preoperative chemotherapy demonstrating intense ossification of the tumor indicative of a good response to the preoperative chemotherapy

Examples of Radical Limb Sparing Surgeries for Osteosarcomas in Various Anatomic Locations
Distal Femur Osteosarcoma
Proximal Tibia Osteosarcoma
Proximal Humerus Osteosarcoma
Scapula Osteosarcoma

In each case the osteosarcoma and bone it arose from was resected.

This required meticulous dissection, mobilization and preservation of adjacent pertinent neurovascular structures.  In each case presented here, the defect was reconstructed with a special modular segmental tumor prosthesis. This also replaces the adjacent joint in many instances.


Plain X-Rays:  Osteosarcoma of Distal Femur
    

     
Distal Femur Osteosarcoma Resection and Prosthetic Reconstruction Incision

                   

              

Limb Salvage: Radical Resection of Distal Femur Osteosarcoma and Reconstruction with Distal Femur Tumor Prosthesis

Function After Distal Femur Tumor Prosthesis


Function after Distal Femur Tumor Prosthesis


Function after Distal Femur Tumor Prosthesis


Proximal Tibia Osteosarcoma Resection and Prosthetic Reconstruction
    

   
Proximal Tibia Osteosarcoma Incision|

                  

              

                    

Specimen:  Osteosarcoma of Proximal Tibia

Defect after Resection of Osteosarcoma of Proximal Tibia

Proximal Tibia Tumor Prosthesis

Medial Gastrocnemius Muscle Flap to Cover and Protect Prosthesis with Soft Tissue and Reconstruct Extensor Mechanism of Knee Joint

Final X-Rays:  Proximal Tibia Tumor Prosthesis

 

Conventional Osteosarcoma of Proximal Humerus:  Radical Limb Sparing Extra-Articular Resection and Prosethetic Reconstruction


 

Extended Deltopectroal Approach

Incision Extended Over Top of Shoulder and Along Lateral Margin of Posterior Scapula to Expose Entire Deltoid Muscle

The Scapula and Clavicle are Osteotomized followed by the Proximal Humerus

        

Placement of Cemented Proximal Humerus Tumor Prosthesis

Proximal Humerus Prosthesis Static Stability Achieved with Dacron Tapes
The Prosthesis is Stabilized to the Scapula

Multiple Muscle Rotation Flaps Entire Prosthesis Covered with Soft Tissue Provides Dynamic Stability and Some Function for the Shoulder Girdle

         

Plain Xray: Proximal Humerus Tumor Prosthesis after Extra-articular Resection of Proximal Humerus Osteosarcoma

Function after Proximal Humerus Tumor Prosthesis

          

Function after Proximal Humerus Tumor Prosthesis

            

            

Function after Proximal Humerus Tumor Prosthesis
The Shoulder is usually Stable and Mobile.  The patient can NOT abduct more than 45 degrees actively but can place her hand on top of her head which helps with daily activities

Function after Proximal Humerus Tumor Prosthesis
The shoulder is usually stable which helps with carrying and holding objects there are limitations with positioning the extremity above shoulder level
The shoulder is cosmetically acceptable

Constrained Total Scapula Prosthetic Reconstruction after Resecting an Osteosarcoma of the Scapula Scapula placed on Serratus Anterior along Chest Wall Placed in pocket between serratus anterior and rhomboids

Scapula Placed in pocket between Serratus Anterior and Rhomboid Muscles

SCAPULAR (Non constrained) DESIGN GORTEX 'CAPSULE' Reconstruction

    

SHOULDER GIRDLE MOTION

CONSTRAINED TOTAL SCAPULA
             

SCAPULAR AND PROXIMAL HUMERAL PROSTHESIS

Radical Resection of proximal Humerus Osteosarcoma with Metastasis to Scapula: Reconstruction with Total Scapula Prosthetic Replacement

Outcome of Total Scapula and Proximal Humerus Tumor Prosthesis

Results and Risks Associated with Limb Sparing Surgery and Prosthetic Reconstructions

These types of surgeries are massive, risky, limb sparing surgeries that are performed instead of performing an amputation. The functional results after reconstruction with a tumor prosthesis should always be compared to what the results would be following alternative treatment, namely an amputation. The results never restore the function of the extremity to the same level as the contralateral normal extremity.
The results shown here are particularly good results. While most patients achieve good results, not everybody achieves a good result because of various factors.
Risks include infection; future loosening; prosthetic breakage and failure (prosthesis can wear out in the future); stiff joints; joint weakness; limb length inequality; tumor recurrence; persistent pain, weakness or stiffness in the arm or leg or adjacent joint; nerve or vascular dysfunction/injury; the possibility that complications could require additional surgeries for treatment and even an amputation for treatment.
Patients are advised not to perform activities that require running or jumping as well as activities that place the patient at a significant risk of falling or breaking the prosthesis or bone that the prosthesis is anchored in.

Patients with localized, nonmetastatic osteosarcoma at presentation who are treated with surgery and chemotherapy have cure rates of 70% to 80%.
If there has been a “Good Response” to preoperative chemotherapy as determined by analyzing the specimen once removed then there is approximately a 90% chance of being cured
There is no difference in survival rates whether a limb sparing procedure or an amputation is performed
Patients who present with or develop metastases to the lungs have a worse prognosis although may be capable of being cured if the pulmonary metastases can be resected.
Patients with bone metastases have a dismal prognosis.
Skip metastases confer a poor prognosis
Changing the chemotherapy regimen postoperatively for patients who did not have a “Good Response” has not been shown to change prognosis as of 2008.


 

 


 

 
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