Ewing sarcoma: Difference between revisions
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==Background== | ==Background<ref>Niederhuber, John E., et al. “Sarcomas.” Abeloff's Clinical Oncology E-Book. Elsevier Health Sciences, 2019, pp. 1604 – 1654.e8.</ref>== | ||
[[File:Distribution of Ewing's sarcoma.jpg|thumb|Distribution of Ewing's sarcoma: Most frequent locations are the large long bones and the pelvis.]] | |||
*Highly aggressive malignant bone or soft tissue tumor arising from cells of the neuroectoderm | *Highly aggressive malignant bone or soft tissue tumor arising from cells of the neuroectoderm | ||
*One-fourth of patients will have metastasis at the time of presentation | *One-fourth of patients will have metastasis at the time of presentation | ||
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*Around 70 to 80 percent survival in those with non-metastatic disease | *Around 70 to 80 percent survival in those with non-metastatic disease | ||
*More common in males | *More common in males | ||
==Clinical Features== | ==Clinical Features== | ||
*Constant pain for months over site of malignancy | |||
**Worse with exercise and at night | |||
*Site will be edematous and tender to palpation | |||
**Mass can sometimes be appreciated | |||
*Most often found in pelvis, axial skeleton, or diaphysis of femur with a small minority arising in soft tissues | |||
*Pelvic tumors are more regularly associated with metastatic disease | |||
*Fever, weight loss, fatigue, night sweats occur in less than 20 percent of patients | |||
*Pathological fractures can occur | |||
==Differential Diagnosis== | ==Differential Diagnosis== | ||
{{Bone tumors and their mimics DDX}} | |||
==Evaluation== | ==Evaluation== | ||
[[File:Ewing sarcoma tibia child.jpg|thumb|X-ray of a child with Ewing's sarcoma of the tibia]] | |||
[[File:Ewing's sarcoma MRI nci-vol-1832-300.jpg|thumb|MRI showing Ewing's sarcoma of the left hip (white area)]] | |||
*Appearance on plain radiographs often described as “onion peel” or “moth-eaten” appearance referring to the respective periosteal bone formation and osseous destruction | |||
*LDH is a prognostic factor | |||
*Definitive diagnosis is made with biopsy | |||
*Work-up often includes PET scan for metastatic disease | |||
*t(11;22) translocation is often seen | |||
==Management== | ==Management== | ||
*Pain control | |||
*Neoadjuvant and adjuvant chemotherapy | |||
*Surgery and/or radiation therapy for local control of disease | |||
==Disposition== | ==Disposition== | ||
*Home with outpatient follow up | |||
*Admission if necessary for pain control or significant disease burden | |||
==References== | ==References== | ||
<references/> | <references/> | ||
[[Category:Orthopedics]] | |||
[[Category:Heme/Onc]] | |||
Latest revision as of 21:01, 3 February 2020
Background[1]
- Highly aggressive malignant bone or soft tissue tumor arising from cells of the neuroectoderm
- One-fourth of patients will have metastasis at the time of presentation
- It is assumed that almost all patients have subclinical metastasis
- Presence and location of metastasis is the major prognostic factor for survival
- Metastasis is most frequently seen in bone (spine most common) and lungs
- Around 70 to 80 percent survival in those with non-metastatic disease
- More common in males
Clinical Features
- Constant pain for months over site of malignancy
- Worse with exercise and at night
- Site will be edematous and tender to palpation
- Mass can sometimes be appreciated
- Most often found in pelvis, axial skeleton, or diaphysis of femur with a small minority arising in soft tissues
- Pelvic tumors are more regularly associated with metastatic disease
- Fever, weight loss, fatigue, night sweats occur in less than 20 percent of patients
- Pathological fractures can occur
Differential Diagnosis
Bone tumors and their mimics
Malignant
- Multiple myeloma
- Chondrosarcoma
- Paget disease
- Osteosarcoma
- Adamantinoma
- Chordoma
- Primary bone lymphoma
- Fibrosarcoma
- Myosarcoma
Benign
- Giant cell tumor
- Chrondroblastoma
- Enchondroma
- Langerhans cell histiocytosis of bone
- Osteoblastoma
- Osteochondroma
- Osteoid osteoma
Other
Evaluation
- Appearance on plain radiographs often described as “onion peel” or “moth-eaten” appearance referring to the respective periosteal bone formation and osseous destruction
- LDH is a prognostic factor
- Definitive diagnosis is made with biopsy
- Work-up often includes PET scan for metastatic disease
- t(11;22) translocation is often seen
Management
- Pain control
- Neoadjuvant and adjuvant chemotherapy
- Surgery and/or radiation therapy for local control of disease
Disposition
- Home with outpatient follow up
- Admission if necessary for pain control or significant disease burden
References
- ↑ Niederhuber, John E., et al. “Sarcomas.” Abeloff's Clinical Oncology E-Book. Elsevier Health Sciences, 2019, pp. 1604 – 1654.e8.