Review Article

Extra-Abdominal Desmoid Tumors Associated with Familial Adenomatous Polyposis

Table 3

Surgical and nonoperative outcomes from selected studies.

ReferencesSubjectsAnatomic sitePresentationInterventionSurgical marginsFollow-upOutcomes

Posner et al. [55]Retrospective review
( )
Extra-abdominalPrimary ( )Surgery ( )Not reportedMedian 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-abdominalPrimary ( )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-abdominalPrimary ( )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-abdominalPrimary ( )
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 abdominalPrimary ( ) (32 tumors)Surgery ( )
Medication ( )
Not reportedMedian F/U
60 months
42% LR in macroscopically complete resections
No desmoid-related mortalities
Bonvalot et al. [61]Retrospective review
( )
Extra-abdominalPrimary ( )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 abdominalPrimary ( ) 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-abdominalPrimary ( ) Recurrent ( )Observation ( )
Medication ( )
N/AMedian 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 reportedMedian 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 databaseExtra-abdominal and abdominalPrimary ( )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

EFS: event free survival; PFS: progression free survival; LR: local recurrence; F/U: follow-up; DT: Desmoid tumor; XRT: radiation therapy.