Demographics
23 y.o. female
23 y.o. female
Caption
Erosive extra-axial mass centered on the right petrous ridge. Lesion enhances markedly and appears to infiltrate the right cerebellar hemisphere. Lesion is quite vascular with large flow voids
There is obstructive hydrocephalus due to compression of the IVth Ventricle
Plane
Axial
Modality
MR - T1W w/Gadolinium
ACR Codes
1.3
(
This image was
added on 2007-09-17
)
Quiz
case
Ewing Sarcoma of Petrous Bone
History
23 year old woman presents with right ear deafness, ataxia and headache
Exam
Non-contributory
Findings
• Erosive extra-axial mass centered on the right petrous ridge.
• Lesion enhances markedly and appears to infiltrate the right cerebellar hemisphere.
• Obstructive hydrocephalus
• Fluid obstruction in mastoid cells
• Lesion enhances markedly and appears to infiltrate the right cerebellar hemisphere.
• Obstructive hydrocephalus
• Fluid obstruction in mastoid cells
Differential Diagnosis
• Meningioma
• Metastatic disease
• Chondrosarcoma
• Glomus tumor
• Metastatic disease
• Chondrosarcoma
• Glomus tumor
Case Diagnosis
Ewing Sarcoma of Petrous Bone
Diagnosis By
Surgery
Treatment & Follow Up
Surgery followed by radiation therapy
View Topic Images
topic
Ewing Sarcoma
Disease Discussion
• Etiology: unclear but thought to be derived from either reticuloendothelial cells or undifferentiated mesenchymal cells of the bone marrow.
• Age of onset: 75% are under the age of 20 years; 90% are under the age of 30 years.
• Epidemiology: male > female (3:2); 95% are Caucasian
• Location: Younger patients tend to have the tumor arise in tubular bones whereas older patients tend to have tumor arise in axial skeleton, pelvis and shoulder girdle.
• Signs/Symptoms: localized pain and swelling, pyrexia and elevated ESR, weight loss, anemia; usually involves a single bone with extensive involvement of the diaphysis, as exemplified in this case. However, 25% arise primarily in the metaphysis. Bones commonly involved are the femur (most common), tibia and humerus, as well as marrow-rich sites such as the pelvis and ribs
• Radiologic features: Destructive bone lesion with extensive periosteal reaction. Tumor rapidly traverses the cortex and expands in the subperiosteal region. Erosion of the outer cortex occurs, producing so called ‘saucerization’ as mentioned in the findings in this case. Also classic is the ‘onion-peel’ appearance that is formed from episodic growth and repair by the tumor. This feature was not readily apparent in these films. The permeative pattern is produced by infiltration of cortical bone as the tumor spreads through the haversian canals.
• Differential diagnosis: in the early stages includes osteomyelitis or trauma. Both of which produce a periosteal reaction with little bone destruction. Other tumors in the differential include lytic osteosarcoma, primary lymphoma, secondary neuroblastoma, and eosinophilic granuloma.
• Prognosis: 15-30% have metastasis at time of presentation (most commonly to lung). Current treatment protocols report up to a 79% disease-free survival. For patients with recurrence, survival is half of that number. Nonresectable central lesions, particularly those in the pelvis have a worse prognosis, large lesion exceeding 8 cm are also more likely to present with metastasis and/or recur. Other adverse prognostic indicators include older age, elevated ESR and elevated leukocyte count at the time of presentation.
• Treatment: Multiagent chemotherapy (vincristine, dactinomycin, and cyclophosphamide) is used prior to radiation or surgery therapy. In this case, the patient will receive all three treatments (chemo, radiation and surgery)
• Age of onset: 75% are under the age of 20 years; 90% are under the age of 30 years.
• Epidemiology: male > female (3:2); 95% are Caucasian
• Location: Younger patients tend to have the tumor arise in tubular bones whereas older patients tend to have tumor arise in axial skeleton, pelvis and shoulder girdle.
• Signs/Symptoms: localized pain and swelling, pyrexia and elevated ESR, weight loss, anemia; usually involves a single bone with extensive involvement of the diaphysis, as exemplified in this case. However, 25% arise primarily in the metaphysis. Bones commonly involved are the femur (most common), tibia and humerus, as well as marrow-rich sites such as the pelvis and ribs
• Radiologic features: Destructive bone lesion with extensive periosteal reaction. Tumor rapidly traverses the cortex and expands in the subperiosteal region. Erosion of the outer cortex occurs, producing so called ‘saucerization’ as mentioned in the findings in this case. Also classic is the ‘onion-peel’ appearance that is formed from episodic growth and repair by the tumor. This feature was not readily apparent in these films. The permeative pattern is produced by infiltration of cortical bone as the tumor spreads through the haversian canals.
• Differential diagnosis: in the early stages includes osteomyelitis or trauma. Both of which produce a periosteal reaction with little bone destruction. Other tumors in the differential include lytic osteosarcoma, primary lymphoma, secondary neuroblastoma, and eosinophilic granuloma.
• Prognosis: 15-30% have metastasis at time of presentation (most commonly to lung). Current treatment protocols report up to a 79% disease-free survival. For patients with recurrence, survival is half of that number. Nonresectable central lesions, particularly those in the pelvis have a worse prognosis, large lesion exceeding 8 cm are also more likely to present with metastasis and/or recur. Other adverse prognostic indicators include older age, elevated ESR and elevated leukocyte count at the time of presentation.
• Treatment: Multiagent chemotherapy (vincristine, dactinomycin, and cyclophosphamide) is used prior to radiation or surgery therapy. In this case, the patient will receive all three treatments (chemo, radiation and surgery)
ACR Code
444.3281
Location
MSK - Musculoskeletal
Category
Neoplasm, sarcoma
Keywords
ewing sarcoma
PNET
PNET
Reference
Eggli, K.D., et. al. Ewing’s Sarcoma. Radiologic Clinics of North America. 31(2): 325-337; 1993.
Grainger, R.G., Allison, D. A Textbook of Medical Imaging 3rd edition. Volume II. Churchill Livingstone, New York. 1997: 1679-1682.
Resnick, D., Niwayama, G. Diagnosis of Bone and Joint Disorders 2nd edition. Volume 6. W.B. Saunders Co., Philadelphia, 1988: 3845-3855.
External Links
cancernet.nci.nih.gov/cancer_Types/Ewing's_Family_of_Tumors.shtml
(
This topic was
added on 2000-11-07
and
last edited on 2007-09-18
)
Original Source
Steven J Goldstein
Steven J Goldstein
Author
Steven J Goldstein
Steven J Goldstein
Author Affiliation
University of Kentucky
University of Kentucky
Approved By
James G. Smirniotopoulos, M.D.
James G. Smirniotopoulos, M.D.
Approver Affiliation
Uniformed Services University
Uniformed Services University
Case Affiliation
University of Kentucky
University of Kentucky
Author
Steven J Goldstein
Steven J Goldstein
Author Affiliation
University of Kentucky
University of Kentucky
Approved By
James G. Smirniotopoulos, M.D.
James G. Smirniotopoulos, M.D.
Approver Affiliation
Uniformed Services University
Uniformed Services University
Topic Affiliation
Penn State University
Penn State University
Case Source
Patricia McKay, USU
Patricia McKay, USU
Author
Donald J Flemming
Donald J Flemming
Affiliation
Penn State University
Penn State University
Approved By
James G. Smirniotopoulos, M.D.
James G. Smirniotopoulos, M.D.
Affiliation
Uniformed Services University
Uniformed Services University