尤文氏肉瘤的影像学表现

Jennifer Son, Year III Gillian Lieberman, MD Imaging of Ewing’s Sarcoma Jen Son Harvard 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 Patient PA Chest film Jennifer Son, Year III Gillian Lieberman, MD MRI Suspicion 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 Biopsy Biopsy (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 Findings Example 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 #4 Decreased 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’d Bone 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 References Handley 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 Oncologist May 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 America Jan 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! Imaging of Ewing’s Sarcoma Slide Number 2 MRI Slide Number 4 Differential Diagnosis Differential Diagnosis cont’d Biopsy Ewing’s Sarcoma Clinical Presentation Plain Film: Typical Findings Slide Number 11 Slide Number 12 Atypical Presentation: Pt #2 Slide Number 14 Pt #2: MRI #4 Our Patient: Metastatic Workup Metastatic Workup cont’d Our Patient: Bone Scan Treatment Prognosis Treatment Evaluation PET Our Patient: Bone Scan, post-treatment Imaging Algorithm References Acknowledgements