Artikel
Myxoid dermatofibrosarcoma protuberans of the face
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Autoren
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Jochen Krueger
- Department of ORL, Head and Neck Surgery, Borna, Germany
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Christian Wittekind
- Institute of Pathology, University of Leipzig, Leipzig, Germany
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Thomas Mentzel
- Dermatopathology, Friedrichshafen, Germany
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Ajit Joe Pothen
- Department of ORL, Head and Neck Surgery, Borna, Germany
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Thomas Wilhelm
- Department of ORL, Head and Neck Surgery, Borna, Germany
| Veröffentlicht: | 22. Juli 2009 |
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Gliederung
Text
These rare locally aggressive but hardly metastating tumors are usually found at the trunk and rarely in the head and neck region (seven publications of similar cases of head and neck). Due to its slow growth these tumors are generally found late.
In our case a 67 year-old female presented with a skin lesion of the forehead which slowly grew for 20 years and now involved half of the forehead and upper eyelid. Weeks before presentation the tumor changed into a firm swelling. Biopsy revealed a low-grade myxofibrosarcoma.
Before excision involving parts of the M orbicularis occuli and drilling of the ventral wall of the frontal sinus, skin expanders were implanted for 8 weeks, making it possible to cover a defect of 9x8 cm.
The final histology revealed a partially myxoid dermatofibrosarcoma protuberans with CD34 positivity. Tomotherapie radiation was necessary due to R1 resection at the upper lid sparing the orbita unnecessary radiation.
A proposed standard of diagnosis and therapy regarding these rare tumors including Imatinib, a selective PDGF-receptor tyrosinkinase inhibitor is presented with regards to the guidelines of the German Society of Dermatology.
