Composite DermatofibrosarcomaProtuberans-Giant Cell FibroblastomaRecurring as Bednář Tumor-Giant CellFibroblastoma with Mucoid Lakes and WithAmputation Neuroma

České info

Kompozitný protuberujúci dermatofibrosarkóm-obrovskobunkovýfibroblastóm rekurujúci ako Bednářov tumor-obrovskobunkový fibroblastóms mukoidnými depozitami a amputačným neurómom

Demonštrujeme prípad kompozitného obrovskobunkového fibroblastómu-protuberujúceho dermatofibrosarkómu,ktorý v druhej recidíve obsahoval štruktúru Bednářovho tumoru. Rekurentnélézie obsahovali extrémne myxoidné časti s tvorbou mukoidných jazierok, ktoré spôsobili podobnosťtumoru s myxoidným liposarkómom. V druhej rekurencii bol vnútri nádoru zreteľný amputačnýneuróm prerastený neoplastickými bunkami a celkový obraz napodobňoval nerve sheathtumor. Popísaný prípad demonštruje spoločnú histogenézu obrovskobunkového fibroblastómu,dermatofibrosarkómu a Bednářovho tumoru. Ukazuje, aké široké môže byť morfologické spektrumkompozitného tumoru, ak tento navyše obsahuje neobvyklé, menej známe štruktúry aleboprerastenú nenádorovú léziu.

Klíčová slova:
dermatofibrosarcoma protuberans - obrovskobunkový fibroblastóm - Bednářovtumor - myxoidný liposarkóm - mäkké tkanivá

Authors: M. Zámečník; M. Michal; A. Chlumská
Authors‘ workplace: Šikl’s Department of Pathology, Faculty Hospital, Charles University, Plzeň, Czech Republic
Published in: Čes.-slov. Patol., , 2002, No. 4, p. 173-177
Category:

Overview

We report an unusual case of composite giant cell fibroblastoma-dermatofibrosarcoma protuberans(DFSP) that, in its second recurrence, contained a pattern of Bednář tumor (BT) and giantcell fibroblastoma (GCF). The recurrent tumor showed extreme myxoid change with creation ofmucoid lakes, which mimicked a pattern of myxoid liposarcoma. One area in the recurrent lesioncontained amputation neuroma overgrown with neoplastic spindle cells, which simulated a nervesheath neoplasm. This case demonstrates common histogenesis of GCF, DFSP and BT, and itshows how broad morphological spectrum can be produced by a composite tumor, especiallywhen the tumor includes unconventional growth pattern or additional non-neoplastic lesion.

Key words:
dermatofibrosarcoma protuberans - giant cell fibroblastoma - Bednar tumor - myxoidliposarcoma - soft tissue

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Article was published in

Czecho-Slovak Pathology

2002 Issue 4