Primary adrenal Ewing sarcoma: A systematic review of the literature

Table 1 Demographics and clinical characteristics

	Number of patients	Percentage (%)
Gender
Male	25	37.9
Female	38	57.6
Not reported	3	4.5
Age (yr)	26.4 ± 15.4 (range 2-74)
Presentation	Reported in 56 patients
Abdominal/flank pain	26	46.4
Palpable mass	14	25.0
Incidentaloma	10	17.9
Mean duration of symptoms	2.6 ± 3.1 mo (range 2-365 d)
Hormone hypersecretion	5/42	11.9
Imaging modality	Reported in 54 patients
CT scan	44	81.5
MRI	11	20.4
PET-CT	6	11.1
Not reported	12
Laterality	Reported in 56 patients
Right	27	48.2
Left	28	50.0
Bilateral	1	1.8
Not reported	10
Mean tumor size (cm)	11.4 ± 4.8 range 3-24

CT: Computed tomography; MRI: Magnetic resonance imaging; PET-CT: Positron emission tomography-computed tomography.

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.