Open Access Research

Recurrence and prognostic factors in patients with aggressive fibromatosis. The role of radical surgery and its limitations

Emilio Bertani1*, Alessandro Testori2, Antonio Chiappa1,3, Pasquale Misitano1, Roberto Biffi4, Giuseppe Viale3,5, Giovanni Mazzarol5, Tommaso De Pas6, Edoardo Botteri7, Gianmarco Contino1, Francesco Verrecchia2, Barbara Bazolli7 and Bruno Andreoni1,3

Author Affiliations

1 Division of General and Laparoscopic Surgery, European Institute of Oncology, Via G. Ripamonti, 435, 20141, Milan, Italy

2 Division of Melanoma and Muscle-Cutaneous Sarcomas, European Institute of Oncology, Via G. Ripamonti, 435, 20141, Milan, Italy

3 University of Milan, Milan, Italy

4 Division of Abdomino-Pelvic Surgery, European Institute of Oncology, Via G. Ripamonti, 435, 20141, Milan, Italy

5 Division of Pathology, European Institute of Oncology, Via G. Ripamonti, 435, 20141, Milan, Italy

6 Division of Medical Oncology, European Institute of Oncology, Via G. Ripamonti, 435, 20141, Milan, Italy

7 Division of Epidemiology and Biostatistics, European Institute of Oncology, Via G. Ripamonti, 435, 20141, Milan, Italy

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World Journal of Surgical Oncology 2012, 10:184 doi:10.1186/1477-7819-10-184

Published: 10 September 2012

Abstract

Background

Surgery is still the standard treatment for aggressive fibromatosis (AF); however, local control remains a significant problem and the impact of R0 surgery on cumulative recurrence (CR) is objective of contradictory reports.

Methods

This is a single-institution study of 62 consecutive patients affected by extra-abdominal and intra-abdominal AF who received macroscopically radical surgery within a time period of 15 years.

Results

Definitive pathology examination confirmed an R0 situation in 49 patients and an R1 in 13 patients. Five-year CR for patients who underwent R0 vs R1 surgery was 7.1% vs 46.4% (P = 0.04) and for limbs vs other localizations 33.3% vs 9.9% (P = 0.02) respectively. In 17 patients who had intraoperative frozen section (IFS) margin evaluation R0 surgery was more common (17 of 17 vs 32 of 45, P = 0.01) and CR lower (five-year CR 0% vs 19.1%, respectively, P = 0.04). However, in multivariate analysis only limb localization showed a negative impact on CR (HR: 1.708, 95% CI 1.03 to 2.84, P = 0.04).

Conclusions

IFS evaluation could help the surgeon to achieve R0 surgery in AF. Non-surgical treatment, including watchful follow-up, could be indicated for patients with limb AF localization, because of their high risk of recurrence even after R0 surgery.

Keywords:
Aggressive fibromatosis; Desmoid tumors; Surgery; Frozen sections; Local recurrence; Risk factors