Abstract
BackgroundSurgery is an important treatment option for desmoid tumor (DT) patients, but how to decrease and predict the high recurrence rate remains a major challenge.MethodsDesmoid tumor patients diagnosed and treated at Tianjin Cancer Institute & Hospital were included, and a web-based nomogram was constructed by screening the recurrence-related risk factors using Cox regression analysis. External validation was conducted with data from the Fudan University Shanghai Cancer Center.ResultsA total of 385 patients were identified. Finally, after excluding patients without surgery, patients who were lost to follow-up, and patients without complete resection, a total of 267 patients were included in the nomogram construction. Among these patients, 53 experienced recurrence, with a recurrence rate of 19.85%. The 3-year and 5-year recurrence-free survival (RFS) rates were 82.5% and 78%, respectively. Age, tumor diameter, admission status, location, and tumor number were correlated with recurrence in univariate Cox analysis. In multivariate Cox analysis, only age, tumor diameter and tumor number were independent risk factors for recurrence and were then used to construct a web-based nomogram to predict recurrence. The concordance index (C-index) of the nomogram was 0.718, and the areas under the curves (AUCs) of the 3-year and 5-year receiver operating characteristic (ROC) curves were 0.751 and 0.761, respectively. In the external validation set, the C-index was 0.706, and the AUCs of the 3-year and 5-year ROC curves are 0.788 and 0.794, respectively.ConclusionsAge, tumor diameter, and tumor number were independent predictors of recurrence for DTs, and a web-based nomogram containing these three predictors could accurately predict RFS (https://stepforward.shinyapps.io/Desmoidtumor/).
Highlights
Desmoid tumors (DTs), known as aggressive fibromatosis and desmoid-type fibromatosis, have an incidence of approximately 5 per 1 million persons and are characterized by no metastasis, but local progression and recurrence can occur [1, 2]
After excluding patients without surgery, patients who were lost to follow-up, and patients without complete resection, a total of 267 patients were included in the nomogram construction
Tumor diameter, admission status, location, and tumor number were correlated with recurrence in univariate Cox analysis
Summary
Desmoid tumors (DTs), known as aggressive fibromatosis and desmoid-type fibromatosis, have an incidence of approximately 5 per 1 million persons and are characterized by no metastasis, but local progression and recurrence can occur [1, 2]. DTs can be divided into sporadic and familial adenomatous polyposis (FAP)-related DTs. Sporadic DTs are associated with a mutation in the CTNNB1 gene and are usually located in the abdominal wall or outside the abdomen [3]. FAP-related DTs, which are associated with mutations in the APC gene, usually occur in the intra-abdominal region, including the mesentery, retroperitoneal area, or pelvis, and can be very large [2, 4]. The pathogenesis of DTs is related to many factors, including trauma, hormones, and heredity. Surgery is an important treatment option for desmoid tumor (DT) patients, but how to decrease and predict the high recurrence rate remains a major challenge
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