References Subjects Anatomic site Presentation Intervention Surgical margins Follow-up Outcomes Posner et al. [55 ] Retrospective review (
) Extra-abdominal Primary (
) Surgery (
) Not reported Median F/U 88 months 36% LR Median time to LR: 15 months Negative microscopic margins predictive of EFS Pritchard et al. [56 ] Retrospective review (
) Extra-abdominal Primary (
) Observation (
) XRT (
) Surgery (
) Surgery + XRT (
) Negative (
) Positive/marginal (
) Minimum F/U 48 months Observation/XRT: 1/6 no progression Negative margins: 2/15 LR; 13/15 no LR Positive/marginal margins: 12/29 LR; 17/29 no LR Merchant et al. [57 ] Prospective cohort (
) Extra-abdominal Primary (
) Surgery (
) Adjuvant XRT (
) Negative (
/105) Positive (
/105) Mean F/U 49 months Negative margin: 14/58 LR (24%) Positive margin: 12/47 LR (26%) No difference in LR with adjuvant XRT Gronchi et al. [58 ] Retrospective review (
) Extra-abdominal Primary (
) Recurrent (
) Surgery (
) Negative margin (
) Positive margin (
) Median F/U 135 months De novo DT: 76% LR Recurrent DT: 59% LR Positive margins not predictive of recurrence for de novo DT Latchford et al. [59 ] Retrospective review (
) FAP associated Extra-abdominal and abdominal Primary (
) (32 tumors) Surgery (
) Medication (
) Not reported Median F/U 60 months 42% LR in macroscopically complete resections No desmoid-related mortalities Bonvalot et al. [61 ] Retrospective review (
) Extra-abdominal Primary (
) Nonoperative (
) Observation (
) Medication (
) Surgery (
) Medication (
) Adjuvant XRT (
) Negative (
/89) Positive (
/89) Median F/U 76 months Nonoperative group: 14/23 stable disease Surgical resection: 57/89 LR (64%) Similar EFS with nonoperative treatment and negative margin surgical resection Tumor location and negative margin predictive of EFS Stoeckle et al. [62 ] Retrospective review (
) Extra-abdominal and abdominal Primary (
) Recurrent (
) Medication (
) XRT (
) Surgery (
) Negative (
) Positive (
) Median F/U 129 months Increased LR with recurrent disease Functional impairment correlates with number of surgeries Time to stable disease increased with number of surgeries Fiore et al. [63 ] Retrospective review (
) Extra-abdominal and intra-abdominal Primary (
) Recurrent (
) Observation (
) Medication (
) N/A Median F/U 33 months Observation: 5 year PFS: 50% Medical therapy: 5 year PFS: 59% Nieuwenhuis Vase [64 ] Retrospective review (
) FAP associated Extra-abdominal and abdominal Primary (
) Surgery (
) Non-operative (
) Not reported Median F/U 96 months Abdominal DT: similar PFS with operative and non-operative therapy Extra-abdominal DT: PFS favors surgical resection Salas et al. [65 ] Retrospective review (
) multicenter database Extra-abdominal and abdominal Primary (
) Observation (
) Medication (
) XRT (
) Surgery (
) Surgery + XRT (
) Negative margin (
) Positive margin (
) Unknown (
) Median F/U 54 months Observation only: 78% stable/remission Negative margins: 44% LR; 64% no LR Positive margins: 67% progression; 33% no progression