Liposclerosing Myxofibrous Tumor: A Separated Clinical Entity?

<b>Introduction</b>: Liposclerosing myxofibrous tumors (LSMFTs) have been described as an infrequent and peculiar fibrous dysplasia variant with a predilection for the intertrochanteric femoral region and are not globally considered a distinct tumor. Given their features, they may be confused with a variety of entities. Our aim is to analyze the clinical, radiological, histopathological and molecular features of LSMFTs. <b>Material and Methods</b>: We report 15 new LSMFT cases managed in our tertiary referral hospital and compare our findings with those of the 241 previous LSMFT cases published in the English medical literature. <b>Results</b>: In plain radiography and computerized tomography, LSMFTs are well-defined intraosseous lytic masses with peripheral sclerotic rims and variable amounts of internal calcifications. Histopathologically, LSFMTs consist of variable amounts of spindle cells, bone matrix, adipose tissue, and cystic spaces embedded in a predominantly fibromyxoid stroma. Molecular tests reveal <i>GNAS</i> and <i>TP53</i> mutations. <b>Conclusions</b>: Knowledge of LSMFT and its typical radiological appearance with heterogeneous histopathological findings-especially in small biopsies-are key to preventing the misdiagnosis and overtreatment of affected patients.