Bone Sarcoma Flashcards
Most common subtypes
VIDEO**
Osteosarcoma and Ewings sarcoma
Prevention and screening
No known or accepted techniques
Pathophysiology
Capacity for both local recurrence and distant menostasis.
Low-grade lesion (Well or moderately differentiated) vs High-grade (poorly differentiated or undifferentiated) lesions.
Disseminates hematogenously and via lymph nodes. C
an have second tumor site in same bone as primary tumor.
Natural history of disease
Risk of distant mets directly correlated to size and histologic grade of primary tumor.
80% develop mets if no therapy after surgery
Osteo mets -90% lung, 10% bone
Ewing -50% lung, 25% bone, 25% bone marrow
Signs and symptoms
Most common pain or swelling in a joint or bone. Fever and weight-loss (Often misleading)
Osteo - metaphyses of tubular bone
Ewings -flat bone, diaphyses of tubular bone
Treatment -surgery only
15% cure.
Limb sparing procedure if feasible
Staging
Tnm.
T = Tumor size +/- 8 cm
N = Note lymph node involvement rare
1a and 1b = low grade
All others = high grade
Treatment – standard for osteo
Neoadjuvant chemo 2-6 cycles, surgery, adjuvant chemo 2-12 cycles.
5yr efs 78% (cure)
(MAP) +/-high-dose methotrexate alternating with doxorubicin, cisplatin.
If good pathological response use same chemo for neoadjuvant and Adjuvant. Otherwise switch chemo.
Treatment – radiation osteo
Relatively resistant to radiation.
Use for palliation. And limited in combination with chemotherapy.
Treatment –ewings
69% 5yr EFS.
Neoadjuvant chemo 12-24wks,
Surgery +/- xrt
adjuvant chemo. (VAC/IE) vincristine, doxorubicin, cyclophosphamide alternating with ifosfamide/ etoposide q21days.
If incomplete surgery resection or not a surgical candidate use radiation prior to adjuvant chemo.
FYI - intergroup Ewing’s sarcoma study
Diagnosis – work up
Plain radiograph and MRI of entire affected bone. To evaluate metastatic disease CT scan chest and bone scan (consider bmbx in ewings). Open biopsy or core biopsy (histology drives dx and thus tx)
Prognosis – osteosarcoma -6
Worse =
Larger tumor,
axial site of disease,
tumor necrosis following neoadjuvant chemo,
primary mets at diagnosis,
incomplete surgical resection,
age > 40
sarcoma’s of the bone -4
Osteosarcoma and Ewing’s sarcoma are most common types
Mostly seen in childhood and adolescence, rarely in elderly
Other histologic subtypes are fibrosarcoma, chondrosarcoma, malignant fibrous histiocytoma
Locally invasive and can have metastatic spread
Prognosis – Ewing sarcoma -7
Worse =
mets,
larger tumor,
axial site of tumor,
poor response to initial chemo,
incomplete surgical resection,
pnet or extraosseous histology,
older age
osteosarcoma - tx -4
Mets to lung (90%) and bone (10%)
Surgery: either limb-sparing or amputation. Only 15% are cured with surgery alone
Radiation: relatively resistant to XRT
Chemo: improves the cure rate in combo with surgery to around 75%
