Analysis of Imaging and Pathological Features in 12 Cases of Schwannomatosis and Literature Review

Abstract Objective We analyzed US, CT, and MR images plus the pathological characteristics of 12 patients with pathologically confirmed schwannomatosis to explore the relationship between imaging and pathology.Method A retrospective analysis was undertaken over a 10-year period (01/01/2000-31/12/2019) of 12 patients undergoing Imaging and pathological examination for schwannomatosis, and the relevant literature was reviewed.Results The median age at diagnosis was 39.4 years (range, 22–56 years). We included the mean diameter of 36 lesions (5.10 ± 0.84 cm; range, 0.5–9.1 cm) in the focal observation. US showed that the mass boundary was clear, and most of the masses were low echo. CT showed that tumors were low density; the plain-scan CT value was 22–35 HU and enhancement amplitude 10–30 HU. MRI showed that most of the tumors were low or medium signal on T1-weighted image (T1WI) and high signal on T2WI; enhancement could be homogeneous or inhomogeneous. We could see the cellular Antoni A and myxoid Antoni B areas of the microscopic morphology. According to the proportions of the two areas, masses could be divided into four types: I, II, III and IV. Immunohistochemical (IHC) staining showed that the expression of S-100 protein was diffusely positive (36/36).Conclusions Imaging examination in schwannomatosis is helpful for localization and characterization of focus, as well as observation of the relationship between the tumor and the surrounding tissue structure. However, the specific diagnosis should also be based on pathological manifestations, IHC results and genetic analysis. Characteristics of mass imaging correlate with pathological changes in the mass.