[Giant cell fibroblastoma. A rare soft tissue tumor in childhood].
We report on a 9-year-old girl with a giant cell fibroblastoma of the right anterior chest wall. This rare soft tissue tumour occurs predominantly in the first two decades of life. The typical clinical presentation is a solitary, usually 2-6 cm large, non-tender mass of blue-greyish colour which is mostly located on the back, anterior chest wall, thigh or groin. The histology shows a loose infiltrate of predominantly bland spindle cells in dermis and subcutis. Characteristic elements of the tumour are large angiectoid branching spaces lacking any endo- or epithelium, and relatively small multinucleated cells (floret cells). The recurrence rate is high if the tumour is not excised with adequate safety margins. Metastases are not reported. It is of pre-eminent importance to differentiate this rare benign tumor from sarcomas, in order to avoid an inappropriately aggressive therapy.
Bastian
B C
BC
Hautklinik, Universität, Wurzburg.
Harms
D
D
Kreipe
H H
HH
Hamm
H
H
Bröcker
E B
EB
D014408:Biomarkers, Tumor / Q000032:analysis; D001706:Biopsy; D002648:Child; D003238:Connective Tissue / Q000473:pathology; D018223:Dermatofibrosarcoma / Q000473:pathology*; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D008854:Microscopy, Electron; D012867:Skin / Q000473:pathology; D012983:Soft Tissue Neoplasms / Q000473:pathology*
D014408:Biomarkers, Tumor
Das Riesenzellfibroblastom. Ein seltener Weichteiltumor des Kindesalters.