The desmoid tumour: Local control after surgical treatment

Abstract

Desmoid tumours are uncommon non-malignant tumours that show a locally aggressive growth pattern and a high local recurrence rate after surgery. Approximately 10% of the desmoid tumours are associated with familial adenomatous polyposis (FAP). Variable natural history of the disease challenges treatment decision-making in the absence of prospective, randomised data. Association of this rare tumour to GIST is speculated and the tumorigenesis may share common steps. This study reviews given treatment and reports prognostic factors for local control and concurrent neoplasms in patients evaluated by a single soft tissue tumour group. Patients referred to the soft tissue tumour group at Helsinki University Central Hospital (HUCH) for a desmoid tumour (primary or recurred) during 1987-2007 and receiving surgical treatment with or without adjuvant treatment were included in this retrospective review. All locations and also patients with a FAP-associated tumour were included. Extra-abdominal location showed lower local control despite the fact that 27% of patients also received radiation therapy. One amputation was performed. Female sex and location in the rectus abdominis muscle predicted improved local control in multivariate analysis. In this review, the occurrence (14%) of concurrent neoplasms was higher than expected with unusual tumour types noted including two GISTs. In those patients in whom surgical treatment is chosen, adjuvant radiation therapy should also be considered in order to decrease morbidity from aggressive surgery aiming at R0 resection. Further studies are suggested to illuminate the biological association between the desmoid tumour and other neoplasms.

Meta-analysis of the influence of surgical margin and adjuvant radiotherapy on local recurrence after resection of sporadic desmoid-type fibromatosis

“…Sufficient data for analysis were published in two articles; authors from the other 50 articles were contacted and requested to provide the additional data needed. Of these, 14 responded, bringing the total number of included studies to 16 ( Fig. ).…”

“…Baumert et al 36 Bertani et al 37 Bonvalot et al 11 Cates and Stricker 24 Eastley et al 38 Gronchi et al 22 Huang et al 39 Ihalainen et al 40 Kriz et al 41 Pignatti et al 42 Prodinger et al 43 Salas et al 25 Stoeckle et al 44 van Broekhoven et al 23 Wang et al 45 Wilkinson et al 46 Total Heterogeneity: 37 Bonvalot et al 11 Cates and Stricker 24 Eastley et al 38 Gronchi et al 22 Huang et al 39 Ihalainen et al 40 Kriz et al 41 Pignatti et al 42 Prodinger et al 43 Salas et al 25 Stoeckle et al 44 van Broekhoven et al 23 Wang et al 45 Wilkinson et al 46 Total Heterogeneity: χ 2 = 7·97, 3 d.f., P = 0·05; I 2 = 62% Test for overall effect: Z = 2·50, P = 0·01 Bertani et al 37 Bonvalot et al 11 Cates and Stricker 24 Eastley et al 38 Gronchi et al 22 Huang et al 39 Ihalainen et al 40 Kriz et al 41 Pignatti et al 42 Prodinger et al 43 Salas et al 25 Stoeckle et al 44 van Broekhoven et al 23 Wang et al…”

Comparison of sporadic and FAP‐associated desmoid‐type fibromatoses

“…10 Recently, the effects of NSAID Recurrence rates of 23-31% and 5-year recurrence-free survival of 69% have been reported in sporadic desmoid-type fibromatoses. 5,6,[29][30][31] The recurrence rate among FAP-related tumors has also been reported to be at the same level compared to their sporadic counterparts. 28,32 Young age, big tumor size, and tumor location in the extremities have been detected to be independent predictors of recurrence.…”

“…Recurrence rates of 23‐31% and 5‐year recurrence‐free survival of 69% have been reported in sporadic desmoid‐type fibromatoses . The recurrence rate among FAP‐related tumors has also been reported to be at the same level compared to their sporadic counterparts .…”

Risk factors for local recurrence of fibromatosis

“…However, there is no consensus in this regard, since there are desmoid tumours that have been observed to stop growing spontaneously. 3,7,13,14 It does appear clear that as we increase therapeutic measures, in addition to increasing monitoring of the disease, we enhance the functional consequences, those of radical surgery and radiotherapy in particular. 13,14 We must remember, in our series, that treatment by periodic monitoring only took place in patients who had already developed local recurrence, therefore we do not know what would have happened if we had not operated the primary tumours.…”