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

Table 2

Details on radiation therapy in 21 patients with desmoid tumor.

Disease status at time of RT	Timing of RT	RT dose (Gy)	Long-term status	Site of relapse	Time to relapse or progression after RT (months)	Delayed second RT
Microscopic disease (𝑛=10)	First line	54.4	Relapse	Out	22	—
	First line	50	Relapse	In	12	—
	First line	45	Relapse	In	10	—
	After relapse	45	CRem	—	No
	After relapse	55	CRem	—	No
	After relapse	50	CRem	—	No
	After relapse	50	CRem	—	No
	After relapse	45	CRem	—	No
	Progressive disease	50	CRem	—	No
	Progressive disease	52	Progressive disease	In	20	No
Macroscopic disease (𝑛=12)	First line	50	CRem	—	—
	First line	45	CRem	—	—
	First line	45	CRem	—	—
	First line	50	Relapse	Out	45	—
	After relapse	51	CRem	—	No
	After relapse	50	CRem	—	No
	After relapse	50	CRem	—	Yes
	After relapse	50	Relapse	Out	4	No
	After relapse	45^*	Relapse	In	22	Yes
	After relapse	50	Relapse	Out	8	Yes
	After relapse	35^*	Progressive disease	In	0	Yes
	Progressive disease	40^*	Progressive disease	In	3	No
Negative (𝑛=1)	After relapse	44	Relapse	In	17	No
Unknown (𝑛=2)	First line	45	CRem	—		—
	First line	46	Relapse	Out	0	—

Abbreviations: RT dose: radiation dosage. Microscopic disease: R1 margins after surgery. Macroscopic disease: R2 margins after surgery or no prior surgery (^*). Negative disease: surgery with negative margins R0. Unknown disease: status of margins unknown after surgery. Relapse In: relapse in radiation fields. Relapse Out: relapse outside of radiation fields. CRem: complete remission.