RESUMO
BACKGROUND: Desmoid type fibromatoses has proven to be a diagnostic and therapeutic challenge, as they often appear primarily as a carcinoma of the breast with a high recurrence risk. PATIENTS: A digital archive search was performed for the period from 2009 to the end of 2018. Inclusion criteria consisted of histological examination of at least the surgical specimen in the reference pathology department and at least a second opinion diagnosis in the reference radiology department. RESULTS: A total of 14 women and 1 man underwent surgery on desmoid type fibromatosis of the breast. The average patient age was 49 years (range: 22-72 years). The mean tumor size was 2.2 cm (range: 0.8-4.2 cm). The tumor was detectable in mammography in 12 out of 13 patients and in all 15 patients in sonography. MRI was performed preoperatively in 6 patients; in all of the patients, the tumor was visualized with inhomogeneous contrast enhancement. In the imaging procedures, all desmoid type fibromatoses were classified as suspicious. Performing the core biopsy, preoperative histology confirmed desmoid fibromatosis in 12 out of 15 patients. Nuclear stain for ß-catenin was positive in 7 out of 10 patients. Negative staining was found for AE1/A3 in 10 out of 10 and CD34 in 12 out of 12 patients. In all of the patients, a single-stage operation without the detection of border-forming tumor margins was performed. The follow-up interval ranged from 16 to 96 months (mean: 44.86 months, median: 43 months). In this follow-up period, no patient was diagnosed with desmoid tumor recurrence. CONCLUSION: In imaging, desmoid type fibromatosis of the breast has typical malignancy-related criteria. Extensive preoperative diagnostics enable the planning of complete primary excision of the lesion and reduce the recurrence risk.
RESUMO
PURPOSE: To retrospectively evaluate known criteria for assessment of dural ectasia by using magnetic resonance (MR) imaging in children, adolescents, and young adults with and those without Marfan syndrome. MATERIALS AND METHODS: Local ethics committee approval and informed consent were obtained. MR images of the lumbar spine in 28 patients with clinically proved Marfan syndrome (group A; 17 male, 11 female; age range, 4-21 years; mean, 12.1 years), seven patients with suspicion of Marfan syndrome (group B; six male, one female; age range, 6-18 years; mean, 10.4 years), and 55 patients without Marfan syndrome (group C; 26 male, 29 female; age range, 4-20 years; mean, 10.7 years) were evaluated retrospectively for dural ectasia criteria (scalloping, dural sac ratio, nerve root sleeve diameter, sagittal dural sac width at S1 greater than that at L4) and according to classifications by Ahn et al and Fattori et al. For statistical comparison of results, one-way analysis of variance with Scheffe post hoc comparisons was used, with an overall two-tailed significance at alpha = .05. RESULTS: No significant differences in scalloping and nerve root sleeve diameter were shown between groups. A significant difference was measured for dural sac ratios at L5 and S1 (F test, P = .003 and P < .001 at L5 and S1, respectively; post hoc t test for groups A vs C, P = .004 and P < .001 at L5 and S1, respectively). Significant differences were also obtained between groups A and C for sagittal dural sac width at S1 greater than that at L4 according to the calculated mean difference (for both F test and post hoc t test, P < .001 and P = .003 at S1 and L4, respectively). The Ahn et al and Fattori et al classifications were of limited value. CONCLUSION: The data suggest that only dural sac ratio at L5 and S1 and a sagittal dural sac width at S1 greater than that at L4 are statistically significant criteria for the assessment of dural ectasia in children, adolescents, and young adults.