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Fibroblastic and Myofibroblastic Pediatric Soft Tissue Tumors

  • Matthew P. Walters
  • Eduardo V. ZambranoEmail author
Chapter
  • 837 Downloads
Part of the Molecular and Translational Medicine book series (MOLEMED)

Abstract

Infantile/congenital fibrosarcoma is a rare tumor which peaks in incidence within the first 3 months of life, may be present at birth in up to 40% of cases, and predominates within the first 4 years (Pediatr Pathol 14(1):133–150, 1994). Clinically it presents as a rapidly growing, painless mass in the deep soft tissues of the distal limbs (Eur J Cancer 32A(12):2094–2100, 1996). Less commonly it involves the trunk or head and neck (Enzinger and Weiss’s soft tissue tumors, 5th edn, 2008). This tumor represents a distinct clinical entity from the adult form of fibrosarcoma based on its behavior as well as its signature chromosomal translocation t(12;15). In general, patients do quite well with surgical excision showing >90% 5 year survival, only approximately one-third of tumors recurring, and a very small minority metastasizing (J Surg Oncol 78(4):225–231, 2001; Cancer 38(2):729–739, 1976; J Pediatr Hematol Oncol 24(9):722–726, 2002; Cancer 40(4):1711–1721, 1977). The tumor histologically resembles the adult-type fibrosarcoma with intersecting highly cellular fascicles of monotonous spindled cells commonly with interspersed lymphocytes (Fig. 9.1). Mitoses and necrosis are variably present. Alternate histology is sometime seen to include hemangiopericytic vasculature, calcification, central necrosis, infantile myofibromatosis-like areas, and variable amounts of pleomorphism after treatment (Cancer Genet Cytogenet 132(1):1–13, 2002). The differential diagnoses to be considered encompass adult-type fibrosarcoma, monophasic synovial sarcoma, malignant peripheral nerve sheath tumor, infantile hemangiopericytoma, infantile myofibromatosis, spindled rhabdomyosarcoma, and the cellular variant of infantile fibromatosis. Close examination of H&E histology of these tumors, with most only staining positive for Vimentin, can help pare down this differential. However, molecular assessment of the tumor either by FISH or RT-PCR usually on paraffin-embedded tissue is the key diagnostic component.

Keywords

Fibrosarcoma Fibroma Fibromatosis Spindle cell sarcomas Congenital neoplasias Chromosomal translocations 

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Copyright information

© Springer Science+Business Media New York 2012

Authors and Affiliations

  1. 1.Department of PathologyAurora Baycare Medical CenterGreen BayUSA
  2. 2.Department of PathologyFroedtert Hospital/Medical College of WisconsinMilwaukeeUSA

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