High-Dose Chemotherapy with Stem Cell Rescue in Desmoplastic Small Round Cell Tumor: A Single-Institution Experience and Review of the Literature

Table 2

Demographics of patients with desmoplastic small round cell tumor treated at Children’s Hospital at Montefiore and in literature review.

All patients
Median age at diagnosis	18.3 years
Overall survival	3 years, 36%	5 years, 13%
Category		Percentage
Female	34	13
Male	220	86
Unknown	2	1
Primary abdominal/pelvic disease	198	77
Primary nonabdominal disease	24	9
Unknown	34	13
Liver metastasis	126	49
No liver metastasis	54	21
Unknown	76	30
Extra-abdominal metastasis	62	24
No extra-abdominal metastasis	142	55
Unknown	52	20
SCT	71	28
SCT not in remission	23
SCT in remission	13
SCT unknown remission	35
No SCT	185	72
Radiation	86	34
No radiation	167	65
Unknown	3	1
HiPEC	16	6
No HiPEC	202	79
Unknown	38	15
Doxorubicin	130	51
No doxorubicin	15	6
Unknown	111	43
Alkylating agent	134	52
Alkylating-like agent	8	3
No alkyating agent	3	1
Unknown	111	43
0 treatment	9	4
1 treatment	52	20
2 treatments	87	34
3 treatments	65	25
4–5 treatments	38	15
Unknown	4	2
Complete surgical excision (2,3,4)	56	22
Incomplete surgical excision (0,1)	39	15
Unknown	161	63

SCT: stem cell transplant; HiPEC: hyperthermic intraperitoneal chemotherapy.