Becky
Primary bone neoplasms are rare.1
Most common tumors are: * Osteosarcoma * Ewing’s sarcoma * Chondrosarcoma * Fibrosarcoma * Malignant fibrous histiocytoma of bone * Giant cell tumor * Aneurismal bone cyst * Chordoma
Etiology:
Adam Cancer of the bone is rare, but research has revealed a couple factors that may cause it:2
Patients having previously received external beam therapy or anticancer drugs are more susceptible to osteosarcomas, especially children
Heredity
Patients with metal bone implants
Signs & Symptoms:
Megan Signs and symptoms associated with bone cancer include local swelling and pain in the involved area. It is uncommon to find joint effusion and pathologic fracture at the time of presentation. The signs and symptoms for Chordoma are dependent on the location of the tumor. If a cord compression or nerve root compression exist, pain and an abnormal gait may be present.3
Diagnostic Procedures:
Kevin Tsai
General 3
History
Physical Examination
Special Studies
Open biopsy, avoiding incision over area not to be irradiated
Bone marrow aspiration and biopsy (for Ewing’s)
Radiologic Studies
Standard
Plain radiography of bone and chest
CT of affected bone, surrounding soft tissue, and lungs
Radionuclide bone scan
MR imaging of affected bone and surrounding soft tissue
Optional
Angiography
Laboratory Studies
Complete blood cell count on admission
Blood chemistry profile
Urinalysis
Erythrocyte sedimentation rate
Histology:
Erin There are many different pathologic types of bone tumors, the most common being the following:4
Osteosarcoma (excluding myeloma)
Usually is poorly differentiated (85% of lesions are grade 3 or 4, with varying amounts of cyst formation, hemorrhage, and necrosis - these are important when radiosensitivity is being considered)
"Skip" bone mets may be seen
Ewing's sarcoma
Chondrosarcoma - grade is an extremely important prognostic factor
Firosarcoma
Malignant fibrous histiocytoma of bone
Giant cell tumor - grade is NOT a reliable prognostic factor
Aneurysman bone cyst
Chordoma
Lymph node drainage:
Spencer In comparison to metastatic bone malignancies, primary bone tumors are rare and do not spread into the lymphatic’s system very often.1 These primary bone malignancies are usually treated without concern for lymphatic spread unless the tumor arises within the trunk of the body.5
Metastatic spread:
Pablo Bone malignancies especially high-grade tumors tend to metastasize hematologically to sites like the lungs, liver, brain and sometimes even other bones. It is estimated that patients with osteosarcoma have an 80% chance of lung metastases 2 years after being diagnosed. Another way that primary bone tumors can affect individuals is by what is called skip metastases. In this case a secondary lesion may appear in the same bone or on the opposing side of a joint space. Spread by way of the lymph system is usually the case of tumors found in the trunk of the body where there lymph nodes have a greater presence. This is not usually of great concern for physicians because hematological spread is much more common.6
Megan The side effects associated with bone cancer radiation are many. Interestingly, the exposure to radiation dosages above 60 Gray, like that used during the treatment of retinoblastoma, can lead to the development of bone cancer. The effects of radiation are directly related to the dose and the treatment volume. And they are inversely related to the age of the patient at the time of therapy. Although limited, scoliosis can be a side effect of vertebral irradiation. It can be compensated for by pelvic tilt. Irradiated bone is more apt for infection, fracture, and necrosis because of radiation induced small vessel changes. Side effects of the radiation administered for Ewing's Sarcoma are if the femur is irradiated, 2/3 of the patients will develop shortening of 2 cm or more ad 1/3 develop pathologic fractures. One in 4 patients with a primary tumor in their tibia, require amputation after completing radiation.8
Prognosis:
Kevin Tsai
The most important factor in osteosarcoma is metastasis at presentation. Radiation-induced osteosarcoma has a worse prognosis1
Prognostic factors in chondrosarcoma include histologic grade, size, cell type, location, stage at presentation, patient age, degree of local aggressiveness, and presence or absence of pain at presentation. Metastases developed in 0% of patients with grade 1 chondrosarcoma, 10% with grade 2, 73% with grade 3.
The most important prognostic indicators for chordoma are site of origin and local extension of tumor.9
Treatments:
Erin Radiation therapy has been a controversial topic in the treatment of bone lesions. Many trials and approaches have been experimented with using radiation therapy.
Osteosarcoma:10
Treatment choice: systemic chemotherapy and surgical resection
A Multi-Institutional Osteosarcoma Study has shown significant benefit to postoperative adjuvant chemotherapy
Memorial Sloan-Kettering has recommended combined radiation therapy and chemotherapy for extrapulmonary mets and small primary lesions in patients with metastatic disease
Resection of pulmonary mets may improve survival
Numerous radiation therapy trials/approaches have been used
Combined chemo and RT (up to 46Gy in 2-3 fractions)
50-60Gy intraoperative RT with or without preoperative chemotherapy
Pulmonary irradiation, with or without chemotherapy
Chondrosarcoma:
Treatment of choice: surgery
Standard surgical procedure is wide total excision with possible amputation
Radiation therapy has been used for inoperable lesions and for palliation
40-60Gy at 2Gy/fraction using multiple fields (MD Anderson reported local control rates of 45-50%)
Entire bone can be treated if medullary involvement is present
Comined photon-neutron therapy (has been done at MD Anderson)
Malignant Fibrous Histiocytoma of Bone:
Treatment of choice: aggresive surgery (involving radical resection, amputation or disarticulation)
Radiation therapy responses have occurred predominately wit hhistiocytic rather than fibrocytic histologies
Combined photons and electrons (median dose of 60Gy in 43 days) - has reported 75% postoperative local control rate
Intraoperative radiation therapy of 15-30Gy
Giant Cell Tumor:
Treatment of choice: surgery
Radiation therapy is used for inoperable lesions, incomplete resections, and local recurrences after surgery
45-55Gy (shows an 80% local tumor control rate)
Aneurysmal Bone Cyst:
Treatment of choice: surgery with curettage and bone grafting or cryosurgery
20-30Gy postoperative radiation therapy (shows a decrease in local recurrence from 32% to 8%)
Chordoma:
Treatment of choice: combined surgery and postoperative irradiation
Radical surgery and radiation therapy are often limited becaues of the proximity of neural structures (this often results in local recurrence)
Ewing's Sarcoma:
Treatment of choice: surgery (for lower-extremity lesions in children)
Amputation - for local failures after radiation therapy
Best results have been achieved with multimodality regimens involving adjuvant irradiation and chemotherapy
VAC-ADR regiment (vincristine sulfate, cyclophosphamide, dactinomycin, and doxorubicin) + 45Gy RT to the primary tumor and smaller doses of radiation to mets (has resulted in 5 yr survival rates of ~30%)
45Gy to the whole bone with two boost of 5Gy each (including the soft tissue mass) to tumor margins of 5cm and 1cm are given
The Pediatric Oncology Group study 8346 showed no advantage of whole-bone irradiation versus more conformal fields with 5cm margin.
TD 5/5:
Spencer Depending on where the primary bone lesion is located, there are a variety of different organs that could be at risk.11
Articular Cartilage: >500 Gy – No Injury
Bladder: 60 Gy – Contracture
Esophagus: 60 Gy – Ulceration and Stricture
Heart: 45 Gy – Pericarditis
Bone Marrow: 2.5 Gy – Aplasia
Intestine: 45 Gy – Ulcer, Perforation, and Hemorrhage
3. Chao C, Perez C, Brady L. Radiation Oncology Management Decisions. 3rd ed. PA: Lippincott Williams and Wilkins; 2011:691-702.
4. Chao KS, Perez CA, Brady LW. Radiation Oncology Management Decisions. 2nd ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2002:605-612.
5. Chao KS, Perez CA, Brady LW. Radiation Oncology Management Decisions. 3rd edition. Philadelphia, PA: Lippincott Williams & Wilkins. 691-702.
6. Washington, CM & Leaver, DT. Principles and Practice of Radiation Therapy. 3rd ed. St. Louis, MO; 2010. P585-589 .
7. Chao KSC, Perez CA, Brady LW. Bone and Ewing’s sarcoma. In: Chao KSC, Perez CA, Brady LW, eds. Radiation Oncology Management Decisions. Philadelphia, PA: Lippincott, Williams and Wilkins; 2011: 691-702.
8. Chao KS, Perez CA, Brady LW. Radiation Oncology Management Decisions. 2nd ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2002:610.
9. Chao C, Perez C, Brady L. Radiation Oncology Management Decisions. 3rd ed. PA: Lippincott Williams and Wilkins; 2011:691-702.
10. Chao KS, Perez CA, Brady LW. Radiation Oncology Management Decisions. 2nd ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2002:605-612 . 11. Washington CM, Leaver D. Principles and Practice of Radiation Therapy. 3rd ed. St. Louis, MO: Mosby Inc; 2010:82.
Primary bone neoplasms are rare.1
Most common tumors are:
* Osteosarcoma
* Ewing’s sarcoma
* Chondrosarcoma
* Fibrosarcoma
* Malignant fibrous histiocytoma of bone
* Giant cell tumor
* Aneurismal bone cyst
* Chordoma
Cancer of the bone is rare, but research has revealed a couple factors that may cause it:2
Signs and symptoms associated with bone cancer include local swelling and pain in the involved area. It is uncommon to find joint effusion and pathologic fracture at the time of presentation. The signs and symptoms for Chordoma are dependent on the location of the tumor. If a cord compression or nerve root compression exist, pain and an abnormal gait may be present.3
There are many different pathologic types of bone tumors, the most common being the following:4
In comparison to metastatic bone malignancies, primary bone tumors are rare and do not spread into the lymphatic’s system very often.1 These primary bone malignancies are usually treated without concern for lymphatic spread unless the tumor arises within the trunk of the body.5
Bone malignancies especially high-grade tumors tend to metastasize hematologically to sites like the lungs, liver, brain and sometimes even other bones. It is estimated that patients with osteosarcoma have an 80% chance of lung metastases 2 years after being diagnosed. Another way that primary bone tumors can affect individuals is by what is called skip metastases. In this case a secondary lesion may appear in the same bone or on the opposing side of a joint space. Spread by way of the lymph system is usually the case of tumors found in the trunk of the body where there lymph nodes have a greater presence. This is not usually of great concern for physicians because hematological spread is much more common.6
G1, low grade:
¨ Parosteal osteosarcoma
¨ Endosteal osteosarcoma
¨ Secondary chondrosarcoma
¨ Fibrosarcoma, low grade
¨ Atypical malignant fibrous histiocytoma
¨ Giant cell tumor
¨ Adamantinoma
G2, high grade:
While there is no universally accepted staging system for bone cancers, the Enneking system can be used and is as follows:3
Grade
G1, low grade
- Parosteal osteosarcoma
- Endosteal osteosarcoma
- Secondary chondrosarcoma
- Fibrosarcoma, low grade
- Atypical malignant fibrous histiocytoma
- Giant cell tumor
- Adamantinoma
G2, high gradeLocal Extent
T1, intracompartmental
- Intraosseous
- Paraosseous
T2, extracompartmentalMetastasis
M0 - No distant metastasis
M1 - Distant metastasis exists
The side effects associated with bone cancer radiation are many. Interestingly, the exposure to radiation dosages above 60 Gray, like that used during the treatment of retinoblastoma, can lead to the development of bone cancer. The effects of radiation are directly related to the dose and the treatment volume. And they are inversely related to the age of the patient at the time of therapy. Although limited, scoliosis can be a side effect of vertebral irradiation. It can be compensated for by pelvic tilt. Irradiated bone is more apt for infection, fracture, and necrosis because of radiation induced small vessel changes.
Side effects of the radiation administered for Ewing's Sarcoma are if the femur is irradiated, 2/3 of the patients will develop shortening of 2 cm or more ad 1/3 develop pathologic fractures. One in 4 patients with a primary tumor in their tibia, require amputation after completing radiation.8
Radiation therapy has been a controversial topic in the treatment of bone lesions. Many trials and approaches have been experimented with using radiation therapy.
Osteosarcoma:10
Chondrosarcoma:
Malignant Fibrous Histiocytoma of Bone:
Giant Cell Tumor:
Aneurysmal Bone Cyst:
Chordoma:
Ewing's Sarcoma:
Depending on where the primary bone lesion is located, there are a variety of different organs that could be at risk.11
2. Bone Cancer. National Cancer Institute Web Site. http://www.cancer.gov/cancertopics/factsheet/Sites-Types/bone. Accessed July 12, 2013.
3. Chao C, Perez C, Brady L. Radiation Oncology Management Decisions. 3rd ed. PA: Lippincott Williams and Wilkins; 2011:691-702.
4. Chao KS, Perez CA, Brady LW. Radiation Oncology Management Decisions. 2nd ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2002:605-612.
5. Chao KS, Perez CA, Brady LW. Radiation Oncology Management Decisions. 3rd edition. Philadelphia, PA: Lippincott Williams & Wilkins. 691-702.
6. Washington, CM & Leaver, DT. Principles and Practice of Radiation Therapy. 3rd ed. St. Louis, MO; 2010. P585-589
.
7. Chao KSC, Perez CA, Brady LW. Bone and Ewing’s sarcoma. In: Chao KSC, Perez CA, Brady LW, eds. Radiation Oncology Management Decisions. Philadelphia, PA: Lippincott, Williams and Wilkins; 2011: 691-702.
8. Chao KS, Perez CA, Brady LW. Radiation Oncology Management Decisions. 2nd ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2002:610.
9. Chao C, Perez C, Brady L. Radiation Oncology Management Decisions. 3rd ed. PA: Lippincott Williams and Wilkins; 2011:691-702.
10. Chao KS, Perez CA, Brady LW. Radiation Oncology Management Decisions. 2nd ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2002:605-612
.
11. Washington CM, Leaver D. Principles and Practice of Radiation Therapy. 3rd ed. St. Louis, MO: Mosby Inc; 2010:82.