Retroperitoneal fibrohistiocytic tumors in children.Report of five cases

Abstract

Five rare retroperitoneal fibrohistiocytic tumors of children are described mainly in reference to the pathological aspects. A more detailed description is given of a retroperitoneal xanthogranuloma, including an ultrastructural study. This case is particularly interesting for its rapid transformation into a fibroxanthosarcoma. The other cases include two fibroxanthosarcomas, a storiform fibrous xanthoma, and a malignant histiocytoma. The most difficult points in the recognition and in the differential diagnosis of these tumors are discussed, and emphasis is placed on some interesting pathological features. Prognosis of such tumors appears difficult to assess from our series, since two patients died in the immediate postoperative period, one died one and a half years after the discovery of the tumor, and two are alive and well, but with a short follow-up.

Fibrohistiocytic Tumors and Related Neoplasms in Children and Adolescents

“…Fibrohistiocytic tumors in the pediatric age group are found in the dermis, subcutis, and deep soft tissues throughout the body or very occasionally in the cranial cavity and viscera [11]. There is a nearly equal distribution of FHTs in males and females.…”

Synchronous gastrointestinal and musculoskeletal manifestations of different subtypes of inflammatory myofibroblastic tumor: CT, MRI and pathological features

“…Coffin et al showed that IMT and inflammatory fibrosarcoma appear to have many overlapping clinical and pathological features, thus considering them as a part of a continuum of myofibroblastic proliferations with increasing cellular atypia and aggressiveness [5][6][7]. Because of their common origin and morphological overlap, differentiation between IMT and other related pathological entities, such as: malignant fibrous histiocytoma, nodular fasciitis, fibromatosis, or even conventional sarcoma or sclerosing mesenteritis, by means of CT or MR imaging, is difficult [8][9][10].…”