Desmoid Fibromatosis in Pediatric Patients: Management Based on a Retrospective Analysis of 59 Patients and a Review of the Literature

Table 3

Efficacy of 61 chemotherapy regimens in 32 patients with measurable disease during first or any subsequent line of therapy.

𝑁	CR	PR	SD	PD	Unknown response	Overall response (%)
VA	6	—	1	5	—	—	1 (17%)
IVA	13	3	1	9	—	—	4 (30%)
VAC	3	—	—	1	1	1	0
VAC and tamoxifen	1	—	—	1	—	—	0
Vinblastine-MTX	14	—	7	5	1	1	7 (50%)
Vinblastine-MTX-tamoxifen	7	—	3	3	0	1	3 (43%)
Doxorubicin with other agents^*	5	—	2	0	3	—	2 (40%)
Imatinib mesylate	2	—	—	2	—	—	0
Others^+	10	—	2	1	7	—	2 (20%)
Total	61	3	16	26 (43%)	14 (23%)	2 (3%)	19 (31%)

Abbreviations: VA: vincristine, dactinomycin; IVA: ifosfamide, vincristine, and dactinomycin; VAC: vincristine, dactinomycin, and cyclophosphamide; MTX: methotrexate. 𝑁: number of patients; CR: complete response; PR: partial response; SD: stable disease; PD: progressive disease.
^*Doxorubicin with vincristine, cisplatin, ifosfamide, cyclophosphamide, or tamoxifen.
⁺Others: VINCAEPI (vincristine, carboplatin, and VM 26), MMT95 protocol (IVA, etoposide, epirubicin, and carboplatin), dactinomycin alone, etoposide-ifosfamide, etoposide alone, and carboplatin.