Laura Ruspi1, Ferdinando Carlo Maria Cananzi2, Federico Sicoli3, Laura Samà1, Salvatore Lorenzo Renne4, Andrea Marrari5, Nicolò Gennaro6, Piergiuseppe Colombo7, Luca Cozzaglio3, Letterio Salvatore Politi6, Alexia Bertuzzi5, Vittorio Quagliuolo3. 1. Sarcoma, Melanoma and Rare Tumors Surgery Unit, Humanitas Clinical and Research Center - IRCCS, Via Manzoni 56, 20089, Rozzano, Milan, Italy; Department of Biomedical Sciences, Humanitas University, 20090, Pieve Emanuele, Milan, Italy. 2. Sarcoma, Melanoma and Rare Tumors Surgery Unit, Humanitas Clinical and Research Center - IRCCS, Via Manzoni 56, 20089, Rozzano, Milan, Italy; Department of Biomedical Sciences, Humanitas University, 20090, Pieve Emanuele, Milan, Italy. Electronic address: ferdinando.cananzi@hunimed.eu. 3. Sarcoma, Melanoma and Rare Tumors Surgery Unit, Humanitas Clinical and Research Center - IRCCS, Via Manzoni 56, 20089, Rozzano, Milan, Italy. 4. Department of Biomedical Sciences, Humanitas University, 20090, Pieve Emanuele, Milan, Italy; Pathology Unit, Humanitas Clinical and Research Center - IRCCS, Via Manzoni 56, 20089, Rozzano, Milan, Italy. 5. Oncology Unit, Humanitas Clinical and Research Center - IRCCS, Via Manzoni 56, 20089, Rozzano, Milan, Italy. 6. Department of Biomedical Sciences, Humanitas University, 20090, Pieve Emanuele, Milan, Italy; Neuroradiology Unit, Humanitas Clinical and Research Center - IRCCS, Via Manzoni 56, 20089, Rozzano, Milan, Italy. 7. Pathology Unit, Humanitas Clinical and Research Center - IRCCS, Via Manzoni 56, 20089, Rozzano, Milan, Italy.
Abstract
BACKGROUND: Desmoid-Type Fibromatosis (DTF) is a rare mesenchymal neoplasm with a locally invasive pattern and high risk of local recurrence after surgery. Historically, the standard treatment for DTF was surgical resection. However, considering the difficulty of achieving surgical eradication, the possible unnecessary morbidity and the unpredictability of the natural history, a wait-and-see approach has been proposed for asymptomatic DTF. METHODS: We analyzed 87 consecutive patients with histologically-proven sporadic primary DTF, first recurrence or residual disease managed at our institution between 2000 and 2018. Patients and tumor-related variables were reviewed and analyzed. Two different treatment strategies were adopted according to different time periods: in the "early period" (2000-2010) patients underwent surgical treatment irrespective of the clinical presentation, whereas in the "late period" (2012-2018) asymptomatic patients used to undergo a wait-and-see strategy. The event-free survival (EFS) was compared trough a pre-post comparison. RESULTS: In the early period, surgery was performed in 51 (94.4%) patients and watchful waiting in 3 (5.6%). In the late period, the watchful waiting group accounted for 24 (72.7%) patients and the surgical group for 9 (27.3%). No statistically independent prognostic factors were found. EFS did not show statistically significant differences between early and late period groups. CONCLUSION: Wait-and-see policy has shown to be equivalent to upfront surgery in terms of EFS; therefore, a conservative approach is recommended in asymptomatic patients diagnosed with DTF that can be followed through watchful waiting.
BACKGROUND:Desmoid-Type Fibromatosis (DTF) is a rare mesenchymal neoplasm with a locally invasive pattern and high risk of local recurrence after surgery. Historically, the standard treatment for DTF was surgical resection. However, considering the difficulty of achieving surgical eradication, the possible unnecessary morbidity and the unpredictability of the natural history, a wait-and-see approach has been proposed for asymptomatic DTF. METHODS: We analyzed 87 consecutive patients with histologically-proven sporadic primary DTF, first recurrence or residual disease managed at our institution between 2000 and 2018. Patients and tumor-related variables were reviewed and analyzed. Two different treatment strategies were adopted according to different time periods: in the "early period" (2000-2010) patients underwent surgical treatment irrespective of the clinical presentation, whereas in the "late period" (2012-2018) asymptomatic patients used to undergo a wait-and-see strategy. The event-free survival (EFS) was compared trough a pre-post comparison. RESULTS: In the early period, surgery was performed in 51 (94.4%) patients and watchful waiting in 3 (5.6%). In the late period, the watchful waiting group accounted for 24 (72.7%) patients and the surgical group for 9 (27.3%). No statistically independent prognostic factors were found. EFS did not show statistically significant differences between early and late period groups. CONCLUSION: Wait-and-see policy has shown to be equivalent to upfront surgery in terms of EFS; therefore, a conservative approach is recommended in asymptomatic patients diagnosed with DTF that can be followed through watchful waiting.
Authors: Bimal Mayur Kumar Vora; Peter L Munk; Nagavalli Somasundaram; Hugue A Ouellette; Paul I Mallinson; Adnan Sheikh; Hanis Abdul Kadir; Tien Jin Tan; Yet Yen Yan Journal: PLoS One Date: 2021-12-23 Impact factor: 3.240