Jennifer Son, Year III

Gillian Lieberman, MD

Imaging of Ewing’s Sarcoma

Jen SonHarvard Medical School

BIDMC, Boston

Gillian Lieberman, MD

Jennifer Son, Year III

Gillian Lieberman, MD

Area of mottled sclerosis and lucency in the distal half of the right clavicle, slightly expansive

Lesion borders are ill-defined with associated periosteal

reaction and cortical thinning

Associated soft tissue mass, normal contour lost

Lesion concerning for malignancy—MRI recommended

6 yo

female presents with right clavicular

mass and pain

Our PatientPA Chest film

Jennifer Son, Year III

Gillian Lieberman, MD

MRISuspicion of malignancy

-provides information on marrow and soft tissue involvement—spread through medullary

better

seen than on plain radiographs; can detect presence of skip lesions in bone

-must be obtained before biopsy because postoperative changes can confuse true extent of disease

Jennifer Son, Year III

Gillian Lieberman, MD

Sagittal

and axial T2 with fat suppression sequences show a soft tissue mass surrounding the right clavicle with associated edema.

There is communication of the soft tissue mass with the medullary

cavity.

The soft tissue mass showed enhancement with gadolinium.

Our Patient: MRI

Jennifer Son, Year III

Gillian Lieberman, MD

Differential Diagnosis

•

Important things to consider when evaluating bony lesions:

-age of patient-location, size-cortical destruction-associated soft tissue mass

Jennifer Son, Year III

Gillian Lieberman, MD

Differential Diagnosis cont’d

•

Osteomyelitis•

Osteosarcoma

•

Ewing’s sarcoma•

Lymphoma

•

Leukemia•

Metastatic neuroblastoma

•

Langerhans

cell histiocytosis

Can all have “moth-

eaten”

pattern and surrounding edema

Can have similar lytic

pattern

May have patterns of sclerosis

PEAK AGES:0-5: LCH, neuroblastoma, leukemia

5-10: Osteosarcoma

10-20: Ewing’s sarcoma

Jennifer Son, Year III

Gillian Lieberman, MD

BiopsyBiopsy (with CT guidance)

Our patient’s results showed

Ewing’s sarcoma

small round blue cells

Jennifer Son, Year III

Gillian Lieberman, MD

Ewing’s Sarcoma•

Described in 1921 by Dr. James Ewing

•

2nd most common bone tumor in children

•

Usually occurs in 2nd

decade of life; rarely

occurs after age 30

•

Whites affected much more than other races

•

Found mostly in flat and long bones (diaphysis)

Jennifer Son, Year III

Gillian Lieberman, MD

Clinical Presentation•

Pain--usually intermittent at first, but can progress to intense pain

•

Can present like osteomyelitis: Fever, anemia, leukocytosis, increased ESR or LDH

•

Eventually a large mass may be palpable

•

Less commonly, can present with pathological fracture

Jennifer Son, Year III

Gillian Lieberman, MD

Plain Film: Typical Findings

●Ill-defined, destructive margins ●“moth-eaten”

appearance (purple arrow)

●overlying soft tissue mass ●expanded cortex with displacement of periosteum

(Codman’s triangle) ●

“onion peel”

appearance due to periosteal

reaction (orange arrows)

http://utdol.com/utd/content/image.do?imageKey=

onco_pix/radiog10.gif

Example patient #1 Example patient #2

Jennifer Son, Year III

Gillian Lieberman, MD

•

80% to 90% have soft tissue mass—best seen on T2-weighted/T1-weighted C+; heterogeneous contrast-enhancement

•

Coronal or sagittal

T1- weighted images can

demonstrate intramedullary extent (arrows)

MRI: Typical FindingsExample patient #3

Jennifer Son, Year III

Gillian Lieberman, MD

Now that we have seen a typical presentation of Ewing’s sarcoma,

let’s review an atypical presentation.

Jennifer Son, Year III

Gillian Lieberman, MD

Atypical Presentation: Pt #2

15 yo

male, initially presented with fevers and hip pain

Plain film of pelvis appears normal overall

Jennifer Son, Year III

Gillian Lieberman, MD

Initial diagnosis of osteomyelitis

made based on clinical presentation and findings on plain films and MRI

Debridement, antibiotics—pt still had pain

Plain film of right hip s/p debridement shows heterotopic

bone along the right ilium--likely related to the debridement

Another MRI done—findings consistent with osteomyelitis; a biopsy was non-specific

Pt #2: Plain film

Jennifer Son, Year III

Gillian Lieberman, MD

Pt #2: MRI #4Decreased signal in rt

ilium

Another biopsy done after

repeated failure to respond to

antibiotics— Ewing’s sarcoma

diagnosed

Jennifer Son, Year III

Gillian Lieberman, MD

Our Patient: Metastatic Workup•

Need to assess lung (most common site of metastases) with chest CT

•

Our patient’s chest CT showed no evidence of metastatic disease; however, can visualize cortical destruction of clavicle (yellow arrow)

Jennifer Son, Year III

Gillian Lieberman, MD

Metastatic Workup cont’dBone scintigraphy:

Whole body scan using Tc

99m-MDP-

technetium-99m (radioactive) is linked to methylene-diphosphonate

(MDP) which is taken up by bone -

‘hot spot’

occurs where tracer

accumulates; denotes areas of ↑ physiological function (fracture, tumor)

Jennifer Son, Year III

Gillian Lieberman, MD

Our Patient: Bone Scan

Jennifer Son, Year III

Gillian Lieberman, MD

Treatment•

Chemotherapy

-reduces local tumor volume-believed that majority of cases have subclinical metastatic disease at time of presentation

•

Surgical resection of tumor

•

Adjuvant radiation therapy if needed

•

~80% of limbs can be salvaged

Jennifer Son, Year III

Gillian Lieberman, MD

Prognosis•

Unfavorable:

-presence of metastases (30% survival w/isolated lung mets, <20% w/bone mets)

-large size of primary tumor (>200ml)

-axial location vs. extremity

-male sex, age >12, anemia, ↑

LDH, radiation therapy only for local control, poor chemo course

Jennifer Son, Year III

Gillian Lieberman, MD

Treatment Evaluation•

MRI

-necrotic intraosseous

lesion with increased signal on T2-can have well-defined margin-however, changes in signal can reflect changes in bone marrow structure or nonspecific fibrosis can make detecting residual tumor difficult

•

Bone scan

Jennifer Son, Year III

Gillian Lieberman, MD

PET•

Positron emission tomography (PET) with fluorodeoxyglucose

(FDG)

-most sensitive to detect changes in tumor metabolism following treatment

-glucose analog is taken up and retained by tissues with high metabolic activity (brain, liver, most malignant tumors)

(also a possible role for metastatic workup)http://en.wikipedia.org/wiki/FDG-PET

Jennifer Son, Year III

Gillian Lieberman, MD

Our Patient: Bone Scan, post-treatment

S/P chemo and resection; no evidence of uptake in previous

area of neoplasm or osseus

metastases

Jennifer Son, Year III

Gillian Lieberman, MD

Imaging Algorithm•

Plain films (at least 2 planes)

•

MRI for better characterization of extent and involvement

•

Biopsy (CT guidance or open) for definitive diagnosis

•

Chest CT and bone scan for evaluation of metastases

•

MRI, bone scan, PET-FDG for treatment assessment

Jennifer Son, Year III

Gillian Lieberman, MD

ReferencesHandley ER, Rosebrook

JL, et al. Ewing’s sarcoma. BrighamRAD. brighamrad.harvard.edu/Cases/bwh/hcache/378/full.html.

Children’s Hospital Boston, PACS.

Avril

NE, Weber WA. Monitoring response to treatment in patients utilizing PET. Radiologic Clinics of North America Jan 2005; 43: 189-204.

Bernstein M, Kovar

H, et al. Ewing’s sarcoma family of tumors: Current management. The OncologistMay 2006;11:503-519.

Franzius

C, Sciuk

J, et al. FDG-PET for detection of osseous metastases from malignant primary bone tumours: Comparison with bone scintigraphy. Eur J Nucl Med. Sep 2000;27:1305-11.

Jadvar

H, Alavi

A, Mavi

A, Shulkin

BL. PET in pediatric disease. Radiologic Clinics of North AmericaJan 2005; 43:135-152.

Luedtke

LM, Flynn JM, Ganley

TJ, et al. The orthopedists’

perspective: Bone tumors, scoliosis, and Trauma. Radiologic Clinics of North America July 2001; 39: 803-821.

Miller SL, Hoffer

FA. Malignant and benign bone tumors. Radiologic Clinics of North America July 2001; 39: 673-699.

Strauss LG. Ewing Sarcoma. E-Medicine 2002. www.emedicine.com/radio/topic275.htm.

Jennifer Son, Year III

Gillian Lieberman, MD

Acknowledgements

•

Dr. Jimmy Kang•

Dr. Jeanette Perez

•

Ms. Pamela Lepkowski•

Ms. Jane Choura

•

Dr. Gillian Lieberman•

Larry Barbaras

THANK YOU!