INTRODUCTION Desmoid tumors are locally destructive but histologically benign. Their management involves close observation and surgical, medical, or hormonal treatment. PRESENTATION OF THE CASE A 36-year-old male was admitted for abdominal pain and fever. A CT scan showed fluid collections and air within a mesenteric mass. Diagnostic laparotomy was performed with drainage of the abscess and b...

BACKGROUND Desmoid tumors (deep fibromatoses) are clonal connective tissue malignancies that do not metastasize, but have a significant risk of local recurrence, and are associated with morbidity and occasionally mortality. Responses of desmoid patients to sorafenib on an expanded access program led us to review our experience. METHODS After Institutional Review Board (IRB) approval, we revie...

objectives: familial adenomatous polyposis (fap) is an autosomal dominant predisposition to colon cancer. this hereditary genetic disease is characterized by more than 100 adenomatous polyps in colon and rectum. additional features may include desmoids tumors, polyps in the upper gastrointestinal tract, osteomas and congenital hypertrophy of the retinal pigment epithelium (chrpe). a mutation in...

CONTEXT Desmoid tumors constitute one of the most important extraintestinal manifestations of familial adenomatous polyposis. The development of desmoids is responsible for increasing morbidity and mortality rates in cases of familial adenomatous polyposis. OBJECTIVES To evaluate the occurrence of desmoid tumors in familial adenomatous polyposis cases following prophylactic colectomy and to p...

OBJECTIVES To improve the prognosis of patients with familial adenomatous polyposis (FAP) by early diagnosis and prophylactic treatment through a coordinated FAP register. DESIGN The establishment and descriptive analysis of the prospective database of the FAP registry. SETTING University surgical unit, Colombo North Teaching Hospital Ragama, Sri Lanka. PATIENTS Probands were identified b...

In 2011, a 37-year old patient with a history of familial adenomatous polyposis underwent a subtotal colectomy and resection of the proximal jejunum and distal duodenum with side-to-side duodenojejunostomy. The procedure was performed for adenocarcinoma of the duodenum (pT3 pN0 L0 V0 R0 G2). In November of 2013, an endoscopically irresectable recurrent adenoma of the anastomosis was seen. Surgi...

Familial adenomatous polyposis (FAP) is characterized by the development of many tens to thousands of adenomas in the rectum and colon during the second decade of life. FAP has an incidence at birth of about 1/8,300, it manifests equally in both sexes, and accounts for less than 1% of colorectal cancer (CRC) cases. In the European Union, prevalence has been estimated at 1/11,300-37,600. Most pa...