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Bone and Soft Tissue Sarcomas PowerPoint Presentation

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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 : 3136 | Downloads : 10

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Slide 1-

Bone and Soft Tissue Sarcomas Resident Education Lecture Series
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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
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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%
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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?
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Cell of origin may be mesenchymal stem cell Osteoblastic Fibroblastic Chondroblastic Telangiectatic Small Cell
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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
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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
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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
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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
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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
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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
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Radiographs OS Usually blastic May be lytic or mixed bone destruction and production Poorly marginated Cortical destruction Soft tissue ossification
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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
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Treatment: Multimodal OS Surgery control of bulk disease Chemotherapy control of micrometastases Radiation Tumors not very radiosensitive, so this usually reserved for palliation
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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
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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%
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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
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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
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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
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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
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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
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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
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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
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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)
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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%)
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Location EWS central axis (47%): pelvis, chest wall, spine, head & neck extremities (53%) #1 Femur #2 Ilium #3 Tibia/Fibula
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Location EWS Classical presentation is diaphyseal Actually more common in metadiaphysis or metaphysis
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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
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Radiographs EWS Destructive Poorly Marginated Permeative Endosteal Cortical Erosion Layered periosteal new bone “Onion skinning”
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Radiographs EWS
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Radiology EWS Large soft tissue mass MRI necessary to determine Soft tissue extent Intraosseous extent
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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
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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
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Treatment: Multimodal EWS Surgery local control where possible Radiation local control where surgery not possible or incomplete Chemotherapy control of micrometastases
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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
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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
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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
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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
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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
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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
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Bone tumors: “Compare & Contrast”
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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
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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
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Associations / Risk Factors Osteosarcoma Lots Radiation Hereditary retinoblastoma Li-Fraumeni Rothmund-Thomson Cytogenetics None characteristic Ewing Sarcoma None established Cytogenetics t(11;22)
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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
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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
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Treatment Osteosarcoma Local control Surgery NO radiation Systemic control Chemotherapy Ewing Sarcoma Local control Surgery and/or radiation Systemic control Chemotherapy
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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
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Late effects Osteosarcoma Chemotherapy related Surgery related Ewing Sarcoma Chemotherapy related Surgery related Radiation related
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Soft Tissue Sarcomas Rhabdomyosarcoma MOST COMMON
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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
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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
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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
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Clinical Presentation Detected by mass appearance or functional disturbance ‘systemic’ symptoms are Rare
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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
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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
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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
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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
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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
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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
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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;
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From ABP Certifying Exam Content Outline Know that the presenting symptom of osteosarcoma is usually bone pain or swelling
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Credits Anne Warwick MD MPH