A Desmoid Tumor-Staging System Separates Patients with Intra-Abdominal, Familial Adenomatous Polyposis-Associated Desmoid Disease by Behavior and Prognosis

Abstract

Desmoid staging identifies tumors by prognosis and its use for designing prospective treatment studies is reasonable.

Robotic-Assisted Distal Pancreatectomy for a Pancreatic Desmoid Tumor: A Case Report and Review of the Literature

“…This tumor behavior has been historically labelled as "locally malignant" [4]. Church reported a 10% spontaneous resolution rate of DTs, and a 10% rapid progression rate, while 50% remain stable on follow up and 30% have cycles of progression and resolution [4,19]. Little is known about the natural history of pancreatic DTs; most (give the n) were resected and the two deaths reported in the literature were not treated with surgery.7 Pancreatic DTs are hard to diagnose preoperatively.…”

Intra-abdominal fibromatosis with vesico-colonic fistula: case of a benign disease running a malignant course

“…It has been reported that, in over 100 FAP patients with desmoid disease, 10% experienced complete regression of their tumor and 7% died from aggressive, unresponsive disease; the remaining 83% experienced variable growth but were not severely affected. 14 …”

Diagnosis and multidisciplinary treatment of sporadical desmoid tumors

“…The role of endocrine factors in the pathogenesis are supported by clinical observations such as the female predominance, regular occurrence in the childbearing age, progression via the use of oral contraceptives, and regression in post-menopausal or post-oophorectomy patients or those undergoing antiestrogen therapy (8,11,15,25). An antecedent history of trauma to the site of the tumors, often surgical in nature (prophylactic proctocolectomy, Cesarean section), has been identified in about 25% of cases (5,27,30,33).…”

“…It is characterised not only by tumor growth, proliferation, and disease progression but also by stabilization and spontaneous remission (2,7,8,11,25,28,29). Church et al (2008) found that 10% of agressive fibromatoses resolved spontaneously, 30% were unpredictably interchanging between progression and resolution, 50% remained stable following diagnosis, and 10% were characterised by rapid progression (30). Variant desmoid growth patterns have been identified: some progress rapidly and aggressively whereas others are more indolent and may remain stable (2,8,11,15).…”

“…to 85% in 10 years following primary treatment, and recurrence is more frequent with extraabdominal than intra-abdominal desmoids (2,5,8,11,15,25,(30)(31)(32). About 80% of recurrences are observed within 3 years postoperatively, even after radical excision (32)(33)(34).…”