Clinical Characteristics, Management, and Outcomes of 19 Nonpediatric Patients with Desmoplastic Small Round Cell Tumor: A Cohort of Brazilian Patients

Table 1

Patient and tumor characteristics.

Variable	Number of patients (%)
Gender
Male	13 (68)
Female	6 (32)
Age (years), median (range)	26 (15–41)
Family history of cancer^a
Yes	6 (32)
No	10 (53)
Not informed	3 (15)
Symptoms at diagnosis^b
Pain	10 (53)
Ascites	7 (37)
Constipation	5 (27)
Weight loss	6 (32)
Dyspepsia	5 (26)
Palpable mass	2 (11)
Others (fever, cholestasis, nausea/vomits)	6 (32)
Presenting site
Abdominopelvic cavity	17 (90)
Lung	1 (5)
Mediastinum	1 (5)
Number of tumor deposits
Single	3 (16)
Multiple	16 (84)
Largest tumor size (cm), median (IQR)	13 (16–11)
Visceral metastasis at presentation
Yes	11 (58)
No	8 (42)
Site of metastatic deposits (n = 11)
Liver	8 (73)
Lung	3 (27)
Spleen	2 (18)
Others (bone, pancreas, breast)	4 (36)
Lymphadenopathy
Yes	15 (79)
No	2 (10.5)
Not informed	2 (10.5)
Extra-abdominal metastasis
Yes	6 (32)
No	13 (68)
MD Anderson staging^c
1	4 (21)
2	6 (32)
3	4 (21)
4	5 (26)

^aIncludes first- and second-degree relatives. ^bAll patients had symptoms at diagnosis. ^cBased on Hayes-Jordan et al. [15].