A Prognostic Nomogram for Prediction of Recurrence in Desmoid Fibromatosis

Abstract

Objective To construct a postoperative nomogram to estimate the risk of local recurrence for patients with desmoid tumors. Background The standard management of desmoid tumors is resection, but many recur locally. Other options include observation or novel chemotherapeutics, but little guidance exists on selecting treatment. Methods Patients undergoing resection during 1982-2011 for primary or locally recurrent desmoids were identified from a single-institution prospective database. Cox regression analysis was used to assess risk factors and to create a recurrence nomogram, which was validated using an international, multi-institutional dataset. Results Desmoids were treated surgically in 495 patients (median follow-up 60 months). Of 439 patients undergoing complete gross resection, 100 (23%) had recurrence. Five-year local recurrence–free survival (LRFS) was 69%. Eight patients died of disease, all after R2 resection. Adjuvant radiation was not associated with improved LRFS. In multivariate analysis, factors associated with recurrence were extremity location, young age, and large tumor size, but not margin. Abdominal wall tumors had the best outcome (5-year LRFS 91%). Age, site, and size were used to construct a nomogram with concordance index 0.703 in internal validation and 0.659 in external validation. Integration of additional variables (R1 margin, gender, depth, and primary vs. recurrent presentation) did not importantly improve concordance (internal concordance index 0.707). Conclusions A postoperative nomogram including only size, site, and age predicts local recurrence and can aid in counseling patients. Systemic therapies may be appropriate for young patients with large, extremity desmoids, but surgery alone is curative for most abdominal wall lesions.

Prognostic factors for the recurrence of sporadic desmoid-type fibromatosis after macroscopically complete resection: Analysis of 114 patients at a single institution

“…On the one hand, younger patients presented with a higher risk of local recurrence both in our series and in other previous reports. 8,9,13 On the other hand, we observed that some patients' lesions ceased to grow and even shrank spontaneously after reaching 50 or 60 years of age, although these lesions relapsed frequently after resection in the same individuals when they were in their 20s and 30s. These phenomena remain confusing, but speculation reveals some possible reasons.…”

“…7,8,17,18 Two recent studies with large patient samples obtained different results. In the first study, Zeng et al 7 recruited 233 consecutive DF patients who underwent macroscopically complete resection, and the authors concluded that R1 resection status was a factor associated with worse 16 believed that some of the "close but negative" margins were actually "positive" and that an inadequate sampling of surgical resection margins for the pathologic assessment of margin status may explain why resection margins fail to predict the risk of local recurrence in many previous studies.…”

“…For operable lesions, wide surgical resection is recommended, but the local recurrence rate remains high. 7,8 Few studies have rigorously examined the factors associated with DF recurrence. However, the indicated risk factors, including surgical margin, radiation, age and tumor size, are conflicting between different studies.…”

Robotic-Assisted Distal Pancreatectomy for a Pancreatic Desmoid Tumor: A Case Report and Review of the Literature

“…The National Comprehensive Cancer Network (NCCN) guidelines suggest observation of these tumors unless symptomatic or causing impairment of organ function, therapy then is chosen based on the location and potential resulting morbidity of the procedure [22,23]. There has been no consensus on the surgical margin requirements when performing desmoid resections, some studies have clearly shown that an R1 margin is an independent risk factor for local recurrence while other studies showed no difference between R0 and R1 resections if patients are closely followed [24][25][26].…”

Abdominal Desmoid Tumors: Hands Off?

“…The reported results about the importance of age, location, resection margins, and adjuvant radiotherapy are conflicting. 4,7,[10][11][12] Also, radiotherapy has been widely applied for DT, both in primary and adjuvant settings. Results have been ambiguous, especially for adjuvant radiotherapy.…”