Rationale and Objectives
Abdominopelvic computed tomography (APCT) is often performed in patients with skeletal Ewing sarcoma family of tumors during initial staging and for subsequent clinical indications, such as metastasis surveillance; however, its clinical impact is unknown. The purpose of this study was to evaluate whether these computed tomographic examinations alter oncologic management and therefore patient outcomes.
Materials and Methods
One hundred eight consecutive patients with skeletal Ewing sarcoma family of tumors seen from 1985 to 2008 were retrospectively reviewed to identify imaging workup, pathology, primary site, evidence of metastatic disease, and patient outcomes. Data were analyzed using Wilcoxon’s rank sum tests.
Results
Sixty-five of the 108 patients (60%) underwent 342 abdominopelvic computed tomographic examinations during a mean follow-up period of 8.9 years. During this time period, only one of the 65 patients (1.5%) who underwent APCT was discovered to have abdominal metastatic disease. There was no significant difference in the incidence of metastatic disease to the skeleton or chest between the groups without and with APCT ( P = .10). There were 26 pelvic and lumbosacral primaries (24%) and 82 limb primaries (76%). Subgroup analysis performed on the 82 patients with limb primaries without ( n = 36) and with ( n = 46) APCT showed no significant differences in metastatic incidence to the skeleton or chest ( P = .14).
Conclusions
This study indicates that APCT, associated with increased radiation exposure and health expenditure, has a limited role in initial staging and follow-up in patients with skeletal Ewing sarcoma, particularly in patients with limb primaries.
Ewing sarcoma family of tumors (ESFT) consists of small round blue cell tumors that include Ewing sarcoma of bone, primitive neuroectodermal tumor, extraosseous Ewing sarcoma, and small-cell tumor of the thoracopulmonary region (Askin tumor) . These tumors share a common translocation of the EWS gene located on chromosome 22 . Ewing sarcoma is the second most common primary bone tumor of childhood after osteosarcoma , predominantly affecting older children and adolescents, with a mean age of 15 years at presentation . The annual incidence of ESFT in the United States is approximately 200 cases, with a rate of approximately 2.8 cases per million children .
During the initial staging and subsequent follow-up of these patients, the Children’s Oncology Group (COG) recommends plain radiography and/or magnetic resonance imaging (MRI) of the primary tumor . Evaluation for bone and chest metastases includes bone scintigraphy, 2- [ 18 F]fluoro-2-deoxyglucose positron emission tomography (PET), and chest computed tomography (CT), with no recommendations for routine abdominopelvic CT (APCT) . In patients with primaries in the lumbar spine or pelvis, preoperative imaging for baseline involvement and for surgical planning with CT or MRI of the abdomen and/or pelvis is obviously indicated, but currently, the literature does not support follow-up imaging of the abdomen in patients with pelvic primaries or similar imaging of the pelvis in patients with lumbar primaries .
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Materials and methods
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Table 1
Demographics and Outcomes for Patients Followed with and without APCT
Patients without APCT Patients with APCT Variable ( n = 43) ( n = 65)P Total number 43 (40%) 65 (60%) Age (y) 17.8 (6.6, 49.2) 19.5 (0.4, 50.7) .38 Male 29 (67%) 36 (55%) .21 Female 14 (33%) 29 (45%) .19 Number of abdominopelvic computed tomographic exams 0 5.3 (1, 31) Patients who underwent PET 0 (0%) 20 (31%) <.0001 Metastatic disease (bones/lungs) 13 (30%) 30 (46%) .10 Metastatic disease at presentation (bones/lungs) 2 (5%) 3 (5%) .63 Metastatic disease (bones/lungs) subsequently found after initial presentation 11 (26%) 27 (42%) .63 Local recurrence 1 (2%) 5 (8%) .40 No metastasis at presentation or follow-up 27 (63%) 29 (45%) .064 Lost to follow-up (if seen only once) 2 (5%) 0 (0%) .16 Abdominal metastasis NA 1 (2%)
APCT, abdominopelvic computed tomography, NA, not applicable (without abdominal imaging, it was not possible to truly quantitate the incidence of abdominal metastases); PET, positron emission tomography.
Data are expressed as number (percentage) or as mean (minimum, maximum).
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Data Review
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Statistical Analysis
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Results
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Subset Analysis Between Patients with Primary Pelvic and Lumbosacral Versus Limb Ewing Sarcoma
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Subset Analysis Between Patients with Limb Ewing Sarcoma Who Underwent APCT Versus Clinical Follow-up Only
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PET Subset Analysis
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Discussion
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Conclusions
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