Splenic Artery Aneurysm Invaded by Desmoid-Type Fibromatosis.

Despite the benign histologic appearance and negligible metastatic potential, desmoid tumors can be locally aggressive, invading into adjacent structures and organs. We report an unusual case of desmoid-typefibromatosis causing the death of an otherwise healthy individual by rupturing the splenic artery.

In a review of 320 surgical specimens diagnosed as desmoid tumor, 94 (29%) were discovered to be misclassified as such. The most common lesions in this series were Gardner fibroma, scar tissue, superficial fibromatosis, nodular fasciitis, myofibroma, and collagenous fibroma. Four sarcomas were also misinterpreted as desmoid-typefibromatosis (3 low-grade fibromyxoid sarcomas and 1 unclassified spindle-cell sarcoma). We take this opportunity to compare and contrast desmoid tumor and several of the soft tissue tumors that should be considered in the differential diagnosis thereof.

The consensus for treatment has changed over the past decade, with most centres moving away from primary radical surgery towards a front-line ‘watch-and-wait’ policy. Therefore, imaging has an increasingly important role to play in both the diagnosis and follow-up of these patients. This review will discuss the typical imaging characteristics of these lesions and suggest diagnostic and follow-up magnetic resonance imaging protocols, with details of suitable sequences and scanning intervals.

In the present report, we describe a case of breast fibromatosis that developed adjacent to a breast implant and demonstrated a relatively well-defined border even though it invaded the surrounding structures. We also explore the specific imaging features for diagnosing breast fibromatosis in association with implants by reviewing previous literature

We present a case of a 63-year-old woman who complained of abdominal pain, and a contrast-enhanced computed tomography scan revealed a well-circumscribed tumor in the pancreatic tail, measuring 5.1 cm. A left-sided, spleen-preserving pancreatic resection was performed, and pathological analysis showed a mesenchymal tumor. The diagnosis of a pancreatic desmoid tumor was made based on the characteristic morphology and the immune phenotype of the tumor. The English-language literature on pancreatic desmoid tumors was reviewed. In total, 16 previous cases were identified.

CTNNB1 mutations or APC abnormalities have been observed in ∼85% of desmoids examined by Sanger sequencing and are associated with Wnt/β-catenin activation. We sought to identify molecular aberrations in “wild-type” tumors (those without CTNNB1 or APC alteration) and to determine their prognostic relevance.