Desmoid tumours: a perfect example for making progress in treatment management through international collaboration

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 32.86ñ12.64 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.