Download Free Medical Bone and Soft Tissue Sarcomas PowerPoint Presentation

Bone and Soft Tissue Sarcomas PowerPoint Presentation

DOWNLOAD MEDICAL POWERPOINT PRESENTATION

About This Presentation

Description : Check out this medical presentation on Bone Cancer (Sarcoma of Bone), which is titled "Bone and Soft Tissue Sarcomas", to know about pediatric bone tumors.

Tags : Bone Cancer, Sarcoma of Bone, Bone, Soft Tissue Sarcomas, pediatric bone tumors, Benign, Osteochondroma, Osteoid Osteoma, Enchondroma, Chondroblastoma, Non-ossifying fibroma, benign cortical defect, Hemangioma, Eosinophilic granuloma, Osteomyelitis, Malignant, Osteosarcoma, Ewing sarcoma, Malignant fibrous histiocytoma, Non-Hodgkin Lymphoma, Eosinophilic granuloma

Published on : May 10, 2021 | Views : 3062 | Downloads : 10

Shareable Url :

Embed Code :

Comment

Transcript

Slide 1-

Bone and Soft Tissue Sarcomas Resident Education Lecture Series
Slide 2-

Pediatric Bone “Tumors” Benign Osteochondroma Osteoid Osteoma Enchondroma Chondroblastoma Non-ossifying fibroma aka benign cortical defect Hemangioma Eosinophilic granuloma Osteomyelitis Malignant Osteosarcoma Ewing sarcoma Malignant fibrous histiocytoma Non-Hodgkin Lymphoma Eosinophilic granuloma
Slide 3-

Malignant bone tumors Rare 6% of all childhood malignancies Annual US Incidence in children < 20 yrs 8.7 per million ~ 650 to 700 children/year For perspective, Annual US Incidence Overall 4697 per million Lung 610 per million Breast 633 per million Most often occur in young patients < 25 yrs Most common bone tumors ← will focus on these Osteosarcoma 56% Ewing sarcoma 34%
Slide 4-

Osteosarcoma (OS) Primary malignant tumor of bone Derived from primitive bone forming mesenchyme Malignant spindle cells produce immature neoplastic bone matrix – osteoid Can look heterogeneous under the microscope Cell of origin?
Slide 5-

Cell of origin may be mesenchymal stem cell Osteoblastic Fibroblastic Chondroblastic Telangiectatic Small Cell
Slide 6-

Histologic subtype (WHO) OS Central (medullary) tumors Conventional OS (87%) Osteoblastic – 50% Chondroblastic – 25% Fibroblastic – 25% Telangiectatic (3%) Small cell Intraosseous well-differentiated (1%) Multifocal Surface tumors Parosteal (<5%) Periosteal High-grade surface OS High grade vs. Low grade
Slide 7-

Epidemiology OS Most common during 2nd decade 75% between 10 and 20 yrs Peak during adolescent growth spurt Taller than average Occurs earlier in girls M:F 1.5:1 African-American:Caucasian 1.4:1
Slide 8-

ppt slide no 8 content not found
Slide 9-

Associations or Risk Factors OS Ionizing radiation Hereditary retinoblastoma (Rb mutations) Li-Fraumeni syndrome (p53 mutations) Rothmund-Thomson syndrome No environmental risk factors No consistent cytogenetic abnormality
Slide 10-

Clinical presentation OS Pain: dull, aching, constant, worse at night, often attributed to trauma Average duration of symptoms prior to diagnosis is three months May or may not have a mass Diagnosis of pelvic lesions often delayed 20% have detectable metastases at diagnosis – most often (>90%) pulmonary
Slide 11-

Location OS Most common in long bones May have altered gait or function 90% are metaphyseal May cross growth plate Location: #1 distal femur #2 proximal tibia #3 proximal humerus
Slide 12-

Diagnostic Workup OS History and physical examination Laboratory tests: Blood tests: include LDH, Alkaline phosphatase Also CBC, liver/kidney function tests Pathology Biopsy (open preferred) Radiologic tests Plain films of involved bone MRI of entire involved bone Whole body Bone Scan CXR and CT of Chest PET scan (in future) Pre-therapy evaluation also includes Audiogram, echocardiogram, GFR/creatinine clearance
Slide 13-

Radiographs OS Usually blastic May be lytic or mixed bone destruction and production Poorly marginated Cortical destruction Soft tissue ossification
Slide 14-

ppt slide no 14 content not found
Slide 15-

ppt slide no 15 content not found
Slide 16-

ppt slide no 16 content not found
Slide 17-

Prognostic Factors OS Tumor Grade & Histology Parosteal favorable; telangiectatic unfavorable Disease Extent metastatic disease unfavorable Tumor Size / Site axial skeletal primaries unfavorable Age < 10 yrs unfavorable Response of the primary tumor to pre-operative chemotherapy: very powerful predictor > 80-90% necrosis favorable
Slide 18-

Treatment: Multimodal OS Surgery control of bulk disease Chemotherapy control of micrometastases Radiation Tumors not very radiosensitive, so this usually reserved for palliation
Slide 19-

Treatment: Surgery OS Removal of all gross tumor with wide (>5cm) margins en bloc and biopsy site through normal tissue planes is required Type of surgical procedure depends on tumor location, size, extramedullary extent, presence of distant metastatic disease, age, skeletal development, and life-style preference limb-sparing amputation Metastatic sites must also be resected If/when relapse occurs, retrieval therapy must include resection
Slide 20-

Surgery alone 15-25% 5 year survival Recurrence with local and (50%) metastatic disease within 6 months of resection With multiagent chemotherapy  55-68% No difference between adjuvant or neoadjuvant chemotherapy Those with >90% tumor necrosis and complete resection  80-85%
Slide 21-

Treatment: Chemotherapy OS Bulky disease is considered somewhat chemotherapy resistant Subclinical metastases are sensitive to chemotherapy Most active agents include adriamycin, cisplatinum, high-dose methotrexate, ifosfamide, etoposide Best # and schedule of chemotherapy unclear Role of intensification after local control unclear Immune modulators under study Role of adjuvant chemotherapy after thoracotomy for recurrent disease unclear
Slide 22-

Outcomes OS 60-68% of patients with nonmetastatic osteosarcoma of the extremity will survive without recurrence and be cured 20% of patients with metastatic disease will be cured Therapy with curative intent is possible following relapse: 10-20% of these patients may achieve long term survival
Slide 23-

Complications / Late effects of Therapy OS Surgical Functional outcomes of limb salvage vs. amputation under study Chemotherapy Anthracycline induced cardiomyopathy may arise 15+ years after therapy Cis-platinum induced ototoxicity and renal toxicity Ifosfamide induced renal tubular dysfunction
Slide 24-

Ewing Sarcoma (EWS) Represents a family of tumors including Ewing sarcoma of bone extraosseous Ewing sarcoma and peripheral neuroectodermal tumor (PNET) of bone or soft tissue 2nd most common bone tumor in children
Slide 25-

Pathology EWS One of many ‘small round blue cell’ tumors seen in pediatrics Thought to be of neural origin, derived from post-ganglionic parasympathetic primordial cells tumor cells synthesize acetylcholine transferase
Slide 26-

Small, Round, Blue Cell Tumor Differential Diagnosis Lymphoma/Leukemia Rhabdomyosarcoma Metastatic Carcinoma Neuroblastoma PNET/Ewing Sarcoma Small Cell Osteosarcoma Ewing Tumor without differentiation PNET Tumor with neural differentiation
Slide 27-

Incidence EWS Occurs most commonly in 2nd decade 80% occur between ages 5 and 25 Most common bone tumor in children < 10 yrs ~110 new cases/year < 15 yrs ~200 new cases/year < 20 yrs M:F 1.3:1 < 10 yrs 1.6:1 > 10 yrs Rare in African-Americans and Asians
Slide 28-

Associations or Risk Factors EWS ??? Consistent cytogenetic abnormality, t(11;22)(q24;q12) present in 90-95% resultant fusion gene is EWS/FLI-1 Also seen: t(21;22)(q22;q12)  5-10% EWS/ERG t(7;22) and t(17;22)  the remainder EWS/ETV1 and EWS/E1AF respectively t(1;16)(q21;q13) present along with t(11;22)
Slide 29-

Clinical Presentation EWS Pain & swelling of affected area May also have systemic symptoms: Fever Anemia Weight loss Elevated WBC & ESR Mean duration of symptoms 9 months 20-25% present with metastatic disease Lungs (38%) Bone (31%) Bone Marrow (11%)
Slide 30-

Location EWS central axis (47%): pelvis, chest wall, spine, head & neck extremities (53%) #1 Femur #2 Ilium #3 Tibia/Fibula
Slide 31-

Location EWS Classical presentation is diaphyseal Actually more common in metadiaphysis or metaphysis
Slide 32-

Diagnostic Work-Up EWS History and physical examination Laboratory tests: CBC, liver/kidney function tests, LDH, ESR Urinalysis Pathology Bone marrow aspirate and biopsy Biopsy (open preferred) Radiologic tests Plain films of primary site CT/MRI of primary site CXR/CT of chest Whole body bone scan PET scan (in future) Pre-therapy evaluation also includes echocardiogram/EKG
Slide 33-

Radiographs EWS Destructive Poorly Marginated Permeative Endosteal Cortical Erosion Layered periosteal new bone “Onion skinning”
Slide 34-

Radiographs EWS
Slide 35-

Radiology EWS Large soft tissue mass MRI necessary to determine Soft tissue extent Intraosseous extent
Slide 36-

Prognostic factors EWS Extent of disease Metastatic disease unfavorable Size of disease ??? Primary site Pelvis least favorable Distal bones and ribs most favorable Age Younger (<10) more favorable Histologic ??? Response to chemotherapy Neural differentiation
Slide 37-

Treatment EWS Multidisciplinary approach must provide both local control and systemic therapy Local control measures should not compromise systemic therapy When treatment fails, it is usually due to the development of distant metastatic disease
Slide 38-

Treatment: Multimodal EWS Surgery local control where possible Radiation local control where surgery not possible or incomplete Chemotherapy control of micrometastases
Slide 39-

Treatment: Local Control EWS Surgery and/or Radiation therapy No randomized studies comparing surgery to radiation therapy slightly more local recurrence when radiation used for local control current suggestion for surgery where possible without loss of function and without mutilation Combination therapy if incomplete resection Radiation doses usually 4500 – 5500 cGy
Slide 40-

Surgical Indications EWS Expendable bone (fibula, rib, clavicle) Bone defect able to be reconstructed with modest loss of function May consider amputation if considerable growth remaining Trend toward improved outcomes with chemo + surgery vs. XRT
Slide 41-

Radiation therapy Indications EWS Unresectable without significant morbidity Pelvic lesions Spine lesions Lung metastases May consider chemo + XRT to allow for surgical resection or add XRT if surgical margins positive
Slide 42-

Treatment: Chemotherapy EWS All patients require chemotherapy Active agents include Vincristine, cyclophosphamide, adriamycin, dactinomycin, ifosfamide, etoposide, topotecan, melphalan Effective chemotherapy has improved local control rates achieved with radiation to 85-90% Role of SCT for high risk Ewing sarcoma still under investigation
Slide 43-

Outcomes EWS Local Rx only  >80% distant failure Combination chemotherapy + local control 55-75% EFS – localized tumors 20-30% EFS – metastases present at diagnosis Patients with spine or paravertebral disease have a slightly worse prognosis overall, as well as a higher rate of local failure and tumor recurrence
Slide 44-

Complications / Late effects EWS Radiation Second malignant neoplasms Relatively high rate, especially bone tumors Bone growth arrest Chemotherapy Anthracycline-induced cardiomyopathy Veno-occlusive disease of the liver Infertility Second malignant neoplasms
Slide 45-

Bone tumors: “Compare & Contrast”
Slide 46-

Epidemiology Osteosarcoma #1 bone tumor 2nd decade of life Rare under 10 yr M:F 1.5:1 15% more common in African-Americans than in Caucasians Ewing Sarcoma #2 bone tumor 2nd decade of life Not rare under 10 yr M:F ~1.4:1 Rare in African-Americans, other ethnic groups
Slide 47-

Presentation Osteosarcoma Pain Often mass Duration of symptoms 3 months Ewing Sarcoma Pain Often mass/swelling Duration of symptoms 9 months Systemic symptoms Fever, weight loss
Slide 48-

Associations / Risk Factors Osteosarcoma Lots Radiation Hereditary retinoblastoma Li-Fraumeni Rothmund-Thomson Cytogenetics None characteristic Ewing Sarcoma None established Cytogenetics t(11;22)
Slide 49-

Location of disease Osteosarcoma Majority occur in the distal femur, proximal tibia and proximal humerus Mostly metaphyseal Most mets to lungs Ewing Sarcoma Fairly even split between the extremities and the axial skeleton Axial occur in pelvis, chest wall, spine, head and neck Classically diaphyseal Mets to lungs, bone, bone marrow
Slide 50-

Radiology Osteosarcoma “starburst” Usually blastic, often lytic with cortical destruction Soft tissue calicification Ewing Sarcoma “onion skin” Permeative, destructive, cortical erosion Soft tissue mass may be difficult to see without MRI
Slide 51-

Treatment Osteosarcoma Local control Surgery NO radiation Systemic control Chemotherapy Ewing Sarcoma Local control Surgery and/or radiation Systemic control Chemotherapy
Slide 52-

Outcomes Osteosarcoma Local disease only 55-65% overall 80-85% if > 90% necrosis Metastatic disease 20% if to lungs <10% if to bones Ewing Sarcoma Local disease only 60-75% overall Metastatic disease 40% if only to lungs <20% if elsewhere
Slide 53-

Late effects Osteosarcoma Chemotherapy related Surgery related Ewing Sarcoma Chemotherapy related Surgery related Radiation related
Slide 54-

Soft Tissue Sarcomas Rhabdomyosarcoma MOST COMMON
Slide 55-

Staging Work-Up – What are we looking for? CT/MRI (primary) Helpful to delineate soft tissue planes; pre-surgical evaluation CT (chest) Look for metastatic disease in the lungs (common site of metastases) CT (body) Look for lymph node involvement Bone Scan Look for metastases to bone CT/PET May give helpful information about tumor ‘activity’ and response to therapy Bone Marrow Evaluation Look for metastatic disease
Slide 56-

Rhabdomyosarcoma Malignant tumor of mesenchymal origin, generally in cells of skeletal muscle lineage Small, round, blue cell tumor Two main histological types: embryonal and alveolar About 20% are undifferentiated or have other histological subtypes
Slide 57-

Incidence and Etiology 250 US cases/yr; most <9 M:F ratio of 1.3:1.0 higher in industrialized “West” Histology varies according to age at dx Associated with familial syndromes such as Li-Fraumeni and neurofibromatosis Genetic factors may be involved
Slide 58-

ppt slide no 58 content not found
Slide 59-

Clinical Presentation Detected by mass appearance or functional disturbance ‘systemic’ symptoms are Rare
Slide 60-

Clinical Presentation – Detected by mass appearance or functional disturbance 35% Head/neck (orbit & parameninges) with proptosis, opthalmoplegia (orbital) and nasal/sinus S/S, ICP (parameningeal) 22% GU (bladder or prostate) with S/S of hematuria, obstruction, pelvic or testicular mass, constipation
Slide 61-

Clinical Presentation cont’d 18% Extremities with soft tissue swelling, with or without tenderness and erythema 25% other sites … include trunk, pelvis, retroperitoneum, perineum, biliary tract, liver, brain, trachea, heart, breast, ovary Sometimes primary cannot be found
Slide 62-

Diagnostic Workup/Staging H & P Imaging studies of affected area and to determine mets; used as baseline data Tumor biopsy is necessary for diagnosis Formal ‘staging’ to determine ‘risk group’ a combination of TNM system, classified per tumor histology IRS Clinical Group Stage System
Slide 63-

Prognostic Indicators Histologic subtype Stage & Group Site – often related to size, potential for metastases In general - Better outcome with early response to treatment For Localized tumors: older age, regional lymph node involvement, and bony erosion are associated with worse prognosis
Slide 64-

Treatment and Prognosis Treatment multimodal - per protocol Surgery: resection where feasible; second surgery if residual disease after first surgery Shift from more radical procedures to function-sparing procedures, with support of Chemotherapy and Radiation
Slide 65-

Treatment and Prognosis, cont’d Radiation therapy (RT): rhabdo initially thought to be radio-insensitive, but with increased doses RT shown to be helpful RT to all except completely resected Stage I patients; hyperfractionated vs conventional treatment; dose reduction for selected patients under study Emergency RT for SC compression, IC meningeal extension
Slide 66-

Treatment and Prognosis, cont’d Chemotherapy for all Prognosis: <20% to 95% site, stage & histology dependent --Better: orbital, non-bladder/prostate GU --Worse: pelvic, truncal, retroperitoneal, cranial, parameningeal, paravertebral, extremity; mets at dx; alveolar histology Recurrence rare after 3-4 years;
Slide 67-

ppt slide no 67 content not found
Slide 68-

ppt slide no 68 content not found
Slide 69-

From ABP Certifying Exam Content Outline Know that the presenting symptom of osteosarcoma is usually bone pain or swelling
Slide 70-

Credits Anne Warwick MD MPH