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Ectopic Anaplastic Meningioma of the Orbit

1Helmke B., 2Schmack I., 3Kolling G., 2Dithmar S.,
1Ruprecht-Karls-Universität Heidelberg, Pathologisches Institut (Heidelberg)
2Ruprecht-Karls-Universität Heidelberg, Universitäts-Augenklinik (Heidelberg)
3Ruprecht-Karls-Universität Heidelberg, Universitäts-Augenklinik, Sektion für Schielbehandlung und Neuroophthalmologie (Heidelberg)

Backgroud: Ectopic meningiomas are a rare form of meningioma which does not have a connection to the nerve sheaths nor to the orbital foramina and therefore might have an atypical localization within the orbit. Histologically, meningiomas are classified by the WHO with grade III (anaplastic) representing the least favorable prognosis. To our knowledge this is the first reported case of an ectopic anaplastic meningioma within the orbit.
Patient: A 32-year-old male presented with a painless swelling of the left medial canthus. MRI scans showed a smooth edged oval tumor within the medial orbit. There was no contact to the optic nerve or orbital wall. A transconjunctival biopsy was performed and revealed fibrotic tissue. Because of a progressive enlargement of the tumor, a complete resection was planned. Intraoperatively however, infiltrative tumor growth was found and it was impossible to remove it in total.
Results: Histologic examination releaved an anaplastic meningioma (WHO grade III). The diagnosis was confirmed by the department of neuropathology of the university of Bonn. The patient refused any further treatment. Despite the fact that the tumor was potentially malignant and the resection incomplete there has been no progression of the neoplasm during the follow-up period of two years.
Conclusions: Intraorbital meningiomas usually originate from meningothelial cells of the arachnoid of the optic nerve sheath. The etiology of ectopic meningiomas is unclear. It has been suggested that they are caused by a scattering of meningothelial cells during intrauterine development. Besides surgical treatment, adjuvant radiotherapy is an option. Despite incomplete resection, the ectopic anaplastic meningioma of the orbit, which is described here for the first time, has so far taken a positive course.