Value of imaging findings in predicting post-operative recurrence of desmoid-type fibromatosis

The present study aimed to predict the recurrence rate and time by retrospectively analyzing the clinical data (sex, age and recurrence time), imaging findings [tumor location, maximum diameter, border, computed tomography (CT) enhancement ratio, magnetic resonance enhancement ratio and T2 signal ratio] and pathological features (Ki-67 and microscopic margin) in a total of 102 cases of pathologically confirmed desmoid-type fibromatosis. The cohort comprised of 73 females and 29 males, with mean age of 32.86ñ12.64 years (range, 6-78 years). The 1-year and 2-year recurrence rate was 31 and 54%, respectively. Univariate analysis indicated that sex, maximum tumor diameter, CT enhancement ratio and Ki-67 had a significant effect on the recurrence time. Furthermore, multivariate analysis revealed that sex, maximum tumor diameter, Ki-67 and T2 signal ratio were independently associated with the time of recurrence, and the risk ratios were 0.424, 1.100, 1.084 and 1.268, respectively. Therefore, in male patients with a larger maximum tumor diameter, positivity for Ki-67 and a higher T2 signal ratio, desmoid-type fibromatosis was more likely to recur after surgery.

Multifocal fibromatosis is a rare entity. We report on two cases where multifocal disease developed after surgical resection. Chronic inflammation and repetitive trauma may be considered a risk factor for developing multifocal disease.

Desmoid-type fibromatosis is an uncommon disease which mimics sarcoma but is more locally aggressive but with less metastatic potential than sarcoma. Diagnosis is difficult and treatment protocols have changed to include more non-surgical options as compared with sarcoma. This is a retrospective study of the clinical presentation, imaging findings, treatment given, and outcomes of pathologically proven desmoid-type fibromatosis patients who presented to government medical college Kozhikode.

The present study aimed to predict the recurrence rate and time by retrospectively analyzing the clinical data (sex, age and recurrence time), imaging findings [tumor location, maximum diameter, border, computed tomography (CT) enhancement ratio, magnetic resonance enhancement ratio and T2 signal ratio] and pathological features (Ki‑67 and microscopic margin) in a total of 102 cases of pathologically confirmed desmoid‑type fibromatosis. The cohort comprised of 73 females and 29 males, with mean age of 32.86±12.64 years (range, 6‑78 years). The 1‑year and 2‑year recurrence rate was 31 and 54%, respectively. The median age at recurrence was 29 years. Univariate analysis indicated that sex, maximum tumor diameter, CT enhancement ratio and Ki‑67 had a significant effect on the recurrence time. Furthermore, multivariate analysis revealed that sex, maximum tumor diameter, Ki‑67 and T2 signal ratio were independently associated with the time of recurrence, and the risk ratios were 0.424, 1.100, 1.084 and 1.268, respectively. Therefore, in male patients with a larger maximum tumor diameter, positivity for Ki‑67 and a higher T2 signal ratio, desmoid‑type fibromatosis was more likely to recur after surgery.

Clinical experience suggests a high prevalence of emotional distress in patients with desmoid tumor (DT). We examine longitudinal Distress Assessment and Response Tool (DART) scores to estimate prevalence and persistence of distress, and compare cross sectional data between DT and malignant sarcoma cohorts, to identify predictors of distress. Patients with DT completed DART at: T1- diagnosis, T2- during, T3- <6 months and T4- ≥6 months, post-treatment. DART includes patient-reported outcome measures of physical symptoms (ESAS-r), depression (PHQ-9), anxiety (GAD-7) and social difficulties (SDI-21). Descriptive prevalence and persistence of anxiety, depression and wellbeing are reported, and mixed model regression analyses determine predictors of distress. Between 2012 and 2018, a total of 152 DART screens from 94 patients with DT were completed- (T1: n=44, T2: n=31, T3: n=22, T4: n=55). Patients had a mean age 40 years, 78% were female and DT locations were abdominal wall (48%), extremity (30%) and mesentery (22%). Moderate to severe ESAS-r scores (≥4) persisted at T4 for anxiety (20%), depression (13%) and poor wellbeing (31%). Compared to 402 patients with malignant sarcoma, patients with abdominal wall sited DT reported severe PHQ-9 and GAD-7 scores twice as frequently. Abdominal wall location, female sex, history of mood problems and psychosocial concerns were significant predictors of anxiety, depression and poor wellbeing in DT. Adults with DT experience persistently high emotional distress compared to patients with malignant sarcoma. Women with abdominal wall DT, prior mood and current psychosocial concerns need early attention within multidisciplinary treatment settings to reduce persistent distress.

Retroperitoneal desmoid-type fibromatosis (RPDF) is a rare mesenchymal neoplasm, and it covers a broad spectrum of aggressive monoclonal, fibroblastic proliferation. There is no evidence-based or established optimal treatment available for this intriguing disease yet. Therefore, we here investigated the clinicopathological characteristics, surgical, and survival outcomes in RPDF among Chinese patients. Patients with histologically confirmed RPDF were retrospectively studied from 2010 to 2018 within the West China Hospital of Sichuan University. Demographics, clinicopathological characteristics, treatment, and survival outcome data were collected. Of the 29 cases of RPDF, 19 were females. Tumor diameter ranged from 4 to 40 cm, with a median of 10 cm. Of these patients, surgical resection was the primary treatment adopted for RPDF in 26 cases; while 3 patients underwent watchful waiting. In surgical group, complete and incomplete macroscopic resection was achieved in 21 (80.77%) and 6 (19.23%) cases, respectively. Totally, 21 (80.77%) cases underwent multi-visceral resection. With a median follow-up duration of 48 months, 11 patients experienced tumor progression for the entire cohort. Tumor progression was observed for those patients with incomplete and complete macroscopic resection in 2/5 (40.0%) and 6/21 (28.6%) cases, respectively. In the watchful waiting group, there were no documented cases of RPDF regression. The progression-free survival rate was 86.1%, 71.5%, and 62.3% at 1-, 2-, and 3-years, respectively.RPDFs are rare types of tumor, which have characteristically varied natural histories. Surgical resection had a relative favorable outcome, but some patients were associated with burden of significant surgical complications.