Radiology - Ewing Sarcoma
- “onion skin”
- Permeative, destructive, cortical erosion
- Soft tissue mass may be difficult to see without MRI
Treatment - Osteosarcoma
- Local control
- Systemic control
- Ewing Sarcoma
- Local control
- Systemic control
Outcomes - Osteosarcoma
- Local disease only
- 55-65% overall
- 80-85% if > 90% necrosis
- Metastatic disease
- Ewing Sarcoma
- Local disease only
- Metastatic disease
- 40% if only to lungs
- <20% if elsewhere
Late effects - Osteosarcoma
- Chemotherapy related
- Surgery related
- Ewing Sarcoma
- Chemotherapy related
- Surgery related
- Radiation related
Soft Tissue Sarcomas - Rhabdomyosarcoma MOST COMMON
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
- Bone Marrow Evaluation
- Look for metastatic disease
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
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
Clinical Presentation - Detected by mass appearance or functional disturbance
- ‘systemic’ symptoms are Rare
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
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
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
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
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
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
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;
From ABP Certifying Exam Content Outline - Know that the presenting symptom of osteosarcoma is usually bone pain or swelling
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