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20 Cards in this Set
- Front
- Back
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Treatment goals: primary vs secondary bone tumors
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Primary:
Preserve life Preserve limb Maximize limb function Secondary: Relieve pain Preserve mobility Minimize hospitalization Preserve independence |
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Sarcomas: overview
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Cancer of connective tissue cells derived from the mesenchyma
Primary bone cancers are sarcomas (20% of sarcomas) Connective tissue (soft tissue) cancers are sarcomas (80% of sarcomas) -There are over 50 different types! Soft tissue and bone comprises 75% of average body weight Comprise 1% of all adult cancers, and 12% of all pediatric cancers |
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Sarcomas: risk factors
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Prior radiation therapy
Chemical exposure -Vinyl chloride, arsenic Prior injury (scars, burns, etc) Chronic tissue irradiation -Foreign-body implants, lymphedema Neurofibromatosis Paget’s disease of the bone Bony infarcts Genetic cancer syndromes: -Li-Fraumeni (p53), Gardner’s (APC) |
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Sarcoma: clinical presentation
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These patients do NOT look sick
They do NOT have: -Weight loss -Cough -Hemoptysis -Hematuria -Altered bowel/bladder habits -Decreased energy They DO have: -Mass -Pain -Pathologic fracture -Or nothing (serendipitous finding) |
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Soft tissue sarcoma: epidemiology
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Disease of older patients
- >50% of patients are >60 years old - ~20% of patients are <40 years old Not a disease of childhood |
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Soft tissue sarcoma: clinical presentation
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A new mass is a tumor until proven otherwise
Patients are often asymptomatic when the mass develops Can occur at all anatomic body sites, but primarily affect the extremities (including proximal sites) -Thigh buttock, groin -Upper extremity |
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Soft tissue sarcoma: growth and spread
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Growth
-As they grow, they tend to stay within their fascial plane -Normal tissue gets compressed -Pseudocapsule gets formed -Microscopically, however, small fingers of tumor are invading normal tissue --Important when discussing resection Spread -Hematogenous -Lung is main site of mets (85-90%) --Bone 10% -Lymph node involvement is uncommon --2-5% |
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Soft tissue sarcoma: imaging
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Start with plain films to see if it is coming from bone or not
CT or MRI cannot distinguish between benign and malignant tumors Exceptions: -Lipomas -Hemangiomas -Classic ganglions So what do we need to do? Get tissue |
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Soft tissue sarcoma: diagnosis
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Technique is very important when biopsying a lesion thought to be a soft tissue sarcoma
Violated tissue planes spread tumor, locally and distant -No transverse incisions! Need to make an “excisable incision” -A drain tract that can be excised, as it will contain tumor cells This can make the difference between limb salvage and amputation |
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Soft tissue sarcoma: staging and prognosis
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Need to evaluate the common metastastic site:
-Lung with chest x-ray or chest CT -Other imaging is debatable Determine stage after imaging is obtained The larger the tumor, the higher the stage The more distantly spread the tumor, the higher the stage Tumor size, depth, site, etc can help us predict how a person’s soft tissue sarcoma is going to act |
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Soft tissue sarcoma: most common types
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Pleomorphic malignant fibrous histiocytoma (MFH)
Liposarcomas |
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Bone sarcoma: epidemiology
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90% of cases occur in adolescents
Cases in the elderly are usually due to other causes -Radiation, Paget’s, etc. If you see a bony lesion in a patient over 40 years of age, think metastasis first!!!!!!!!! -BLT and a Kosher Pickle -Breast, lung, thyroid, kidney, prostate |
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Bone sarcoma: clinical presentation, common sites, spread
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Localized pain, over several months, that usually begins after an injury
-Can wax and wane Common sites: -Distal femur -Proximal tibia -Middle/proximal femur -Other bones Spread - Hematogenous - Lung is main site of mets - Lymph node involvement is uncommon |
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Bone sarcoma: imaging
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Plain films
IN GENERAL, benign bone lesions can easily have a line drawn around them Endosteal erosions Cortical or trabecular disruptions Soft tissue extension Periosteal reaction Onionskinning -With rapidly growing lesions, the periosteum cannot produce new bone as fast as the lesion is growing -An interrupted pattern of new bone formation occurs as the lesion grows then slows, etc -Peristeum has time to lay down a thin shell of calcified new bone before the next growth spurt Sunburst formation -Due to rapid and steady growth of a lesion that prevents even a thin shell of bone to be laid down -The fibers that connect the periosteum to the bone (Sharpey’s fibers) become stretched perpendicular to the bone and then ossify Codman triangle -With aggressive lesions, the periosteum does not have time to ossify with shells of new bone (even Sharpey’s fibers don’t ossify) -Only the edge of the raised periosteum will ossify |
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Bone sarcoma: diagnosis
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Respect the planes!
-No transverse biopsies Must be able to excise the biopsy tract easily Otherwise can seed the scar with tumor Many are hypervascular and can bleed profusely (with tumor cells riding along) |
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Bone sarcomas subtypes
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Two most common types:
-Chondrosarcomas arise from cartilage -Osteosarcomas arise from bone Fibrosarcomas of bone arise from fibrogenic tissue Hemangioendothelioma and hemangiopericytoma arise from vascular tissue Chordomas arise from notochordal tissue Some (Ewing’s sarcoma family of tumors) we don’t know where they arise from (Texas?) |
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Bone sarcoma: staging
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Perform imaging to complete the staging
The larger the tumor, the higher the stage The more distantly spread the tumor, the higher the stage |
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Sarcomas: treatment overview
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Complicated, depending on site of tumor, grade of tumor, depth of tumor, etc.
Multimodality treatment: -Surgical resection -Chemotherapy (adjuvant or neoadjuvant) -Radiation therapy Why is surgery not enough? -Can only cut out what you can see |
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Sarcomas: treatment metastasectomy
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In general, metastatic disease is incurable
However, for some patients with isolated pulmonary mets, a “met-ectomy” can result in long-term relapse free survival and potential cure 30% of soft tissue sarcoma patients have resectable disease 22% were successfully resected with negative margins Lots of variability: -Anywhere from one to over 50 mets can be resected -Location of mets is important -Size of mets is important -Baseline pulmonary function is important |
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Sarcomas: treatment local modalities
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Radiofrequency ablation
Cryoablation Image-guided radiation therapy Hepatic arterial embolization Radioembolization |
