Intestinal obstruction caused by desmoid tumours: a review of the literature

Intraabdominal desmoid tumours are rare and can cause intestinal obstruction. Based on the review of the literature, surgical resection with negative margins and adjuvant chemotherapy is the optimal strategy for treatment of this pathology.

This portion of the NCCN Guidelines discusses general principles for the diagnosis, staging, and treatment of soft tissue sarcomas of the extremities, superficial trunk, or head and neck; outlines treatment recommendations by disease stage; and reviews the evidence to support the guidelines recommendations.

We describe the case of a 15-year-old boy, a Chinese national, who was diagnosed as having a pancreatic desmoid-type fibromatosis. He underwent a radical resection of the pancreatic mass, partial pancreatectomy, splenectomy, segmental resection of transverse colon and distal jejunum, and subsequently a one-stage jejunojejunostomy and colonic anastomosis. Postoperatively, he was diagnosed as having a desmoid-type fibromatosis of the pancreas by pathological and immunohistochemical studies. Pathological and immunohistochemical testing are essential for confirming diagnosis where demonstration of beta-catenin nuclear staining is probably the single most important characteristic. Other markers, such as vimentin, are usually positive, while S100 protein is usually negative. Our case however, confirms that there are rare cases that can be S100 positive. This is the first diagnosed case of pancreatic desmoid-type fibromatosis with S100 protein positivity.

Desmoid tumor involving the small bowel. Desmoid tumors often occur sporadically, but they occur in 10% to 25% of individuals with familial adenomatous polyposis. Sporadic desmoid tumors can occur in intraabdominal or extraabdominal locations, whereas those associated with familial adenomatous polyposis are most often intraabdominal. The behavior of desmoid tumors can vary from indolent to aggressive.

We performed a retrospective analysis of 22 patients who underwent percutaneous cryoablation of extra-abdominal desmoid tumors between December 2008 and March 2017. Patients were treated with first-line cryoablation (treatment-naïve; n = 11) or salvage cryoablation (disease progression after surgical resection, radiation therapy, and/or systemic therapy; n = 11). Primary endpoints were progression-free survival (PFS) after the first cryoablation and PFS after one or more sessions of cryoablation. PFS was calculated using the Kaplan-Meier method. Predictors of disease progression after the first cryoablation were determined using the log rank test and the Cox proportional hazards model. Tumor sites included abdominal wall (n = 10), back (n = 4), chest wall (n = 6), and upper extremity (n = 2). Disease control was obtained in 15 of 22 patients (68%) after the first cryoablation. Univariate analysis identified age younger than 25 years old (HR: 5.74, P = 0.010), salvage therapy (HR: 8.64, P = 0.017), transaxial diameter >5 cm (HR: 11.37, P = 0.006), and hydrodissection (HR: 0.11, P = 0.041) as predictors of disease progression after the first cryoablation. Univariate analysis found no significant difference in PFS after the first cryoablation based on gender, prior pregnancy, gravidity, or tumor site. Multivariate analysis identified salvage therapy as the only independent predictor of disease progression after the first cryoablation (HR: 13.85, P = 0.05). Five-year PFS rate for all patients after one or more sessions of cryoablation was 88.9% (95% CI 71-100). Disease was ultimately controlled in 21 of 22 patients after one or more sessions of cryoablation (95.5%). A single session of cryoablation appears to provide better control of extra-abdominal desmoid tumors when used as first-line therapy compared to salvage therapy. Given that long-term disease control can be achieved with first-line cryoablation, cryoablation is an appropriate treatment consideration for select treatment-naïve patients with extra-abdominal desmoid tumors.

The present study aimed to assess the clinical utility of three-dimensional arterial spin labeling (3D-ASL) perfusion imaging in discriminating between benign, intermediate and malignant musculoskeletal tumors, as well as to analyze the correlation between tumor blood flow (TBF) and microvessel density (MVD). 3D-ASL was performed on 44 patients with musculoskeletal tumor using a 3.0T magnetic resonance system to obtain TBF values prior to surgery. A receiver operating characteristic (ROC) curve was applied to assess the threshold and diagnostic reliability of TBF. Immunohistochemical staining of tumor specimens was performed using a cluster of differentiation 34 monoclonal antibody to calculate MVD counts. TBF and MVD values in the malignant group were significantly higher compared with that of the benign, and intermediate groups.The results of the present study suggest that 3D-ASL is valuable in discriminating between benign, intermediate and malignant musculoskeletal tumors. 3D-ASL may be utilized to evaluate angiogenesis in musculoskeletal tumors in vivo.