Fibrous tumours in children: imaging features of a heterogeneous group of disorders

Abstract

Background. Fibrous tumours are predominantly soft tissue lesions which are relatively frequent in childhood but are little known. Imaging is often used in the evaluation of these tumours but their characteristics, particularly on US or MRI, have not been studied systematically. Objectives. To provide an overview of the clinical and imaging features of the different disorders, and to correlate them with the currently used classification schemes. Material and methods. Twenty-five patients with fibrous tumours were evaluated retrospectively. Clinical histories were studied for the histopathological diagnosis, age, signs and symptoms at presentation, mode of therapy and follow-up where available. Imaging findings were analysed for the following variables: number, location, size, margin and architecture of soft tissue and/or visceral lesions and the presence and pattern of osseous involvement. Comparison with the available literature was performed. Results. The following tumour types were encountered: desmoid fibromatosis (n = 9), myofibromatosis (n = 7), fibromatosis colli (n = 2), congenital-infantile fibrosarcoma (n = 2), adult-type fibrosarcoma (n = 2), fibrous hamartoma of infancy (n = 1), angiofibroma (n = 1) and hyaline fibromatosis (n = 1). Conclusions. While some tumours were non-specific in their clinical and radiological manifestation, others such as myofibromatosis, fibromatosis colli, fibrous hamartoma of infancy and angiofibroma exhibited a characteristic pattern which allowed a diagnosis to be made even without histology.