Primary adrenal Ewing sarcoma: A systematic review of the literature

Table 2 Operative and pathological characteristics

	Number of patients	Percentage (%)
Metastatic disease
Metastatic during initial diagnosis	17/61	27.9
Inferior vena cava/renal vein neoplastic thrombus	14/61	23.0
Treatment
Neoadjuvant chemotherapy	10/53	18.9
Open adrenalectomy	48/60	80.0
Extensive resection	17/60	27.9
Laparoscopic resection	4/60	6.6
Robotic resection	1/60	1.6
Biopsy without resection	7/60	11.7
Complete surgical resection	42/47	89.4
Tumor confirmation testing
Positive CD99 immunohistochemistry staining	60/61	98.4
Positive molecular EWSR1-FLI1translocation	27/27 patients	100
Adjuvant therapy	Required in 32 patients
Chemotherapy	20/32	62.5
Radiotherapy	1/32	3.1
Chemoradiotherapy	11/32	34.4
Outcomes
Survival data	42 patients
Median overall survival	15 mo
24-mo overall survival	17/42	40.5
Median disease-free survival	10 mo
24-mo disease-free survival	14/42	33.3

EWSR1-FLI1: EWS RNA binding protein 1-Friend leukemia integration 1.