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 cancera
Yes
6 (32)
No
10 (53)
Not informed
3 (15)
Symptoms at diagnosisb
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 stagingc
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].