Congenital infantile fibrosarcoma: Report of four cases and review of the literature

BackgroundCongenital infantile fibrosarcoma (CIFS) is a rare but curable soft tissue tumor.Patients and methodsFrom 1997 to 2005 four newborns were diagnosed with CIFS. One patient was depicted on prenatal ultrasound. As imaging modality plain radiographs, US, CT and MRI were performed.ResultsThe CIFS was localized in the elbow, knee, thigh and neck. Plain radiographs showed a soft tissue mass, US and CT a heterogeneous mass with mainly peripheral vascularisation. MRI presented a mixed soft tissue mass predominantly hypointense on T1-weighted, hyperintense on T2-weighted and STIR sequences and a heterogeneous, mainly peripheral, contrast enhancement. The tumor volume varied between 50 and 68 cm3. One patient presented muscular, another one vascular and neural infiltration, whereas no patient had metastases. In all cases the tumor was surgically resected, in one patient neoadjuvant chemotherapy was given. So far, no patient had a tumor relapse.DiscussionImaging findings of CIFS were non-specific and the final diagnosis was obtained after pathologic examination. MRI is the best imaging modality in the assessment of soft tissue, vascular and neural involvement, in planning therapy and monitoring therapeutic response. However, CT should be performed if osseous involvement is suspected.