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)
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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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