CTNNB1 S45F Mutation Predicts Poor Efficacy of Meloxicam Treatment for Desmoid Tumors: A Pilot Study.

This study aimed to determine the significance of CTNNB1 (β-catenin) mutations in predicting the treatment outcome in patients with desmoid tumors treated with meloxicam, a cyclooxygenase-2 (COX-2) selective inhibitor. Between 2003 and 2012, consecutive thirty-three patients with extra-peritoneal sporadic desmoid tumors were prospectively treated with meloxicam as the initial systemic medical therapy. The correlation between the efficacy of meloxicam treatment and status of CTNNB1 mutations was analyzed. The CTNNB1 mutation status was of significant prognostic value for meloxicam treatment in patients with sporadic desmoid tumors.

We sought to determine the rates of recurrence after surgery for desmoid tumors and analyze factors predictive of recurrence-free survival (RFS). From February 1976 to October 2011, 233 consecutive patients with desmoid tumors who underwent macroscopically complete resection were included in this study. Clinicopathologic and treatment characteristics were evaluated to determine predictors of recurrence. Regardless of primary or recurrent disease, surgical resection remains central to the management of patients with desmoid tumors. However, there are clearly different prognostic subgroups that could benefit from different therapeutic strategies, and a wait-and-see policy is a possible option for a subset of patients.

Sunitinib is an oral multitargeted tyrosine-kinase inhibitor that blocks activation through the stem cell-factor receptor (Kit) and platelet-derived growth-factor receptor. Sunitinib has shown potent antitumor activity against several solid tumors, including renal cell carcinoma, gastrointestinal stromal tumors, and neuroendocrine tumors in several Phase II/III trials. Recently, sunitinib has been used to treat other solid cancers, such as lung cancer, pancreatic cancer, chondrosarcoma, esophageal cancer, bladder cancer, glioma, and aggressive fibromatosis, and also showed potential efficacy in progression-free survival and overall survival. In this review, we examine the efficacy of sunitinib as a molecular-targeted therapy in patients with different types of solid cancers.

Turcot syndrome is a rare inherited condition of colonic polyposis associated with central nervous system tumors. Multiple tumors may be seen in patients with Turcot syndrome but the occurrence of sarcomas is rare. This case highlights the importance of close follow-up for patients with Turcot syndrome and the importance of a broad differential diagnosis in evaluating a condition in which multiple tumors are frequently seen.

The disappointing results of current treatments and the ability of extra-abdominal desmoid tumors to spontaneously stabilize have increasingly drawn interest toward conservative management. The objective of this study was to evaluate a wait-and-see policy as a first-line management for extra-abdominal desmoid tumors. This two-center retrospective study involved fifty-five patients with a histologically proven extra-abdominal desmoid tumor. The primary outcome was the cumulative probability of dropping out from the wait-and-see policy. The wait-and-see policy included aggressive management of symptoms. We conducted a review of the relevant published series in which a watchful-waiting strategy was used. The cumulative probability of dropping out from the wait-and-see policy was 9.6% at the time of the last follow-up. A wait-and-see policy is an effective front-line management for patients with primary or recurrent extra-abdominal desmoid tumor.

Desmoid-type fibromatosis (DTF) is an uncommon nonmetastatic fibrous neoplasm. We herein report a case of DTF of unknown cause involving the pancreatic head. Pylorus-preserving pancreaticoduodenectomy was performed due to the patient’s debilitating symptoms and suspected malignancy. The pathological examination revealed massive fibroblastic proliferation arising from the musculoaponeurotic tissues, consistent with a diagnosis of DTF. Radical resection is recommended as first-line treatment for pancreatic DTF. Long-term follow-up studies are required to establish the prognosis of pancreatic DTF.