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2014 ; 87
(1038
): 20140123
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Multimodality imaging features, metastatic pattern and clinical outcome in adult
extraskeletal Ewing sarcoma: experience in 26 patients
#MMPMID24734938
Somarouthu BS
; Shinagare AB
; Rosenthal MH
; Tirumani H
; Hornick JL
; Ramaiya NH
; Tirumani SH
Br J Radiol
2014[Jun]; 87
(1038
): 20140123
PMID24734938
show ga
OBJECTIVE: To describe the multimodality imaging features, metastatic pattern and
clinical outcome in adult extraskeletal Ewing sarcoma (EES). METHODS: In this
institutional review board-approved, health insurance portability and
accountability act-compliant retrospective study, we included 26 patients (17
females and 9 males; mean age, 36 years; range, 18-85 years) with pathologically
confirmed EES seen at our institute between 1999 and 2011, who had imaging of
primary tumour. Imaging of primary tumour in all 26 patients and follow-up
imaging in 23 was reviewed by two radiologists in consensus. Clinical data were
extracted from electronic medical records. RESULTS: The most common primary sites
were the torso (n = 13), extremities (n = 10) and head and neck (HN) region (n =
3). The mean tumour size was 9 cm (range, 3-22 cm); tumours of the torso were
larger than those of other areas (p > 0.05). Compared with the skeletal muscle,
tumours were isodense on CT (21/21), hypointense (n = 5) to isointense (n = 14)
on T1 weighted image, hyperintense on T2 weighted image (19/19) and were
fluorine-18 fludeoxyglucose ((18)F-FDG)-avid [10/10; mean maximum standardized
uptake value of 7 (range, 3-11)]. Necrosis (15/26), haemorrhage (5/26) and
adjacent organ invasion (14/26) were present without calcification. Median
follow-up was 16 months. 5 patients had local recurrence (torso, 3; extremity, 1;
and HN, 1). Metastases developed in 11 patients (torso, 7; extremities, 3; and
HN, 1; p > 0.05); 8 at presentation, most commonly to lung (9/11), peritoneum
(4/11), muscles (4/11) and lymph nodes (4/11). Nine patients (torso, 7;
extremity, 1; and HN, 1) died (10 months median survival) (p > 0.05). CONCLUSION:
Adult EESs are large tumours, which frequently invade adjacent organs and
metastasize to the lung. EESs of the torso are larger, have more frequent
metastases and poorer outcomes. ADVANCES IN KNOWLEDGE: Adult EESs of the torso
have poor outcomes compared with other EESs.