Hybridtumor eines Riesenzellfibroblastoms und eines Dermatofibrosarcoma protuberans

Zusammenfassung

Wir berichten über ein zum Zeitpunkt der Erstmanifestation 10 Monate altes Kind mit einem Hybridtumor aus Riesenzellfibroblastom (RZFB) und Dermatofibrosarkoma protuberans (DFSP). Das RZFB ist ein seltener Tumor, der mit dem DFSP die Translokation (t17;22) (q22;13) und immunphänotypische Eigenschaften teilt und daher von seinen Erstbeschreibern als kindliche Form des DFSP eingeordnet wurde. Das RZFB hat mit einer Kombination aus Spindelzellproliferaten, häufig hypozellulären Arealen mit kollagenfaserreichem oder myxoidem Stroma, sinusoid-artigen pseudovaskulären Aufweitungen zwischen den Kollagenfasern, die von multinukleären Riesenzellen umsäumt werden, jedoch ein ihm eigenes histologisches Bild. Das RZFB, das sich klinisch als langsam wachsende, i.d.R. schmerzlose Schwellung manifestiert, tritt präferentiell im Kindesalter auf. Das mittlere Erkrankungsalter liegt bei 6 Jahren. Die Therapie der Wahl ist die Exzision mit einem ausreichenden Sicherheitsabstand. Unsere junge Patientin zeigt ein Jahr nach Exzision mit 2 cm Sicherheitsabstand einen rezidivfreien Verlauf.

Abstract

We report on a 10 months old infant with a hybrid lesion of giant cell fibroblastoma (GCF) and dermatofibrosarcoma protuberans (DFSP). GCF is a rare tumor, which shares with DFSP the (t17;22) (q22;13) translocation and immunophenotypic characteristics; therefore GCF is considered as juvenile form of DFSP. GCF is characterized by fascicles of slender spindle cells, often hypocellular areas with collagenous or myxoid stroma and pseudovascular spaces lined by multinuclear giant cells. Clinically, GCF usually presents as a slow growing painless tumor, predominantly evolving in childhood with a mean age of 6 years. Excision with adequate safety margins is the treatment of choice. Our patient was one year after tumor resection with 2 cm margin in complete remission.

Schlüsselwörter

Riesenzellfibroblastom - Dermatofibrosarkoma protuberans - Hybridtumor - pädiatrische Tumore

Keywords

Giant cell fibroblastoma - dermatofibrosarcoma protuberans - hybrid tumor - childhood tumor

Publication History

Article published online:
24 October 2022

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