Desmoid tumor (DT) is a rare and locally invasive proliferative disease. The standard first-line treatment used to be radical surgical resection. However, during recent years it has been debated whether to offer an aggressive and morbidity treatment to a potentially indolent tumor. Desmoid tumor has uncertain behavior, some are aggressive cases, others indolent and there are some reports of spontaneous involution. Nowadays, the guidelines suggest to watch and wait before offering an aggressive treatment, and avoiding the surgical treatment. But in our experience patients had less morbidity and disease progression with a surgical approach.
Patients with histological diagnosis of DT and treated at INCA between January 1998 and December 2015 were identified and their medical records were analyzed.
Of 191 patients evaluated, there were 137 females (71.2%). The median age at diagnosis was 34 years (range:17.79-76.96y). Tumor locations were: thoracoabdominal wall (n = 100, 52.3%), extremities (n = 53,27.7%), abdominal cavity (n = 26; 13.6%), and head and neck (n = 12, 6.2%). Tumor sizes were documented in 152 cases (79.5%) and ranged from 1 cm to 37 cm (median, 10 cm). Twenty-seven pts (14.1%) received systemic therapy (ST) (n = 5 vinblastine and methotrexate; n = 22 tamoxifen) and 164 pts (85.8%) were submitted to surgery (4pts received adjuvant tamoxifen) as first-line treatment. There was no difference between gender (p = 0.86), tumor location (p = 0.30), or tumor size (p = 0.53) when choosing first-line treatment. The odds of severe morbidity were 2.13 higher with ST than with upfront surgery. Four pts were submitted to surgery after systemic first-line treatment. After a median follow-up of 71 months, there was significantly more disease progression in the ST group (17pts – 62.9%) than in the surgery group (55 pts – 33.5%) (p = 0.005), and they received more subsequent treatment (p = 0,01). There were 2 deaths in the ST group and 9 deaths in the surgery group, with 10-year survival of 93.2% and 92%, respectively.
DT is an indolent disease but has the propensity for locally invasive growth and recurrence. Although ST is a less aggressive treatment, it was associated with higher severe morbidity, more disease progression, and more subsequent treatment in this trial.
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All authors have declared no conflicts of interest.