Programm                 "Degeneration und Regeneration– Grundlagen, Diagnostik und Therapie"

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Sclerosing Angiofibroma of the Orbit

Guthoff R.
Universitäts-Augenklinik Würzburg

Purpose: We present an orbital process difficult to classify, which was diagnosed histopathologically as sclerosing angiofibroma.
Method: A 58-year old male patient presented with painful proptosis of the left eye. Clinical examination revealed a proptosis of 4mm and slight conjunctival injection. Posterior segment and the right eye were uneventful. Ultrasound examination revealed a 10x15x9 mm low reflective homogenous lesion of the upper temporal orbit. CT-scan and MRI showed a well circumscribed tumor contrast enhancing at the outer margin. A lateral orbitotomy (Krönlein) with complete tumor resection was performed. 16 months later there were no signs of recurrence nor complaints.
Results: On macroscopic examination a light-coloured, solid, well circumscribed tumor was seen. Micropscopically the lesion was composed of connective tissue with few fibroblasts. In the central parts of the tumor numerous small obliterated vessels with broad hyalinized walls were present. At the tumor margin normal vessels with inflammatory reaction and eosinophils were found. Immunohistochemically reaction of CD34 and actin was positive and proliferation fraction of MIB-1 positive cells was less than 5%.
Conclusions: Clinically and histopathologically this case is compatible with an idiopathic sclerosing inflammation of the orbit while the well circumscribed character is not typical for an orbital pseudotumor. Multifocal fibrosclerosis which is associated with sclerosing orbital pesudotumor o

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