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Open Access Case report

Recurrent orbital schwannomas: clinical course and histopathologic correlation

Michelle Kron1, Brenda L Bohnsack2, Steven M Archer2, Jonathan B McHugh3 and Alon Kahana24*

Author Affiliations

1 University of Michigan Medical School, Ann Arbor, MI, USA

2 Department of Ophthalmology and Visual Sciences, University of Michigan, Ann Arbor, MI, USA

3 Department of Pathology, University of Michigan, Ann Arbor, MI, USA

4 Helmut F. Stern Career Development Professor, Ophthalmology and Visual Sciences, University of Michigan, Ann Arbor, MI, USA

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BMC Ophthalmology 2012, 12:44  doi:10.1186/1471-2415-12-44

Published: 31 August 2012

Abstract

Background

Schwannomas are slow-growing typically encapsulated tumors composed of differentiated Schwann cells, the primary class of peripheral glial cells.‚ÄČComplete excision is the treatment of choice for orbital schwannomas that cause pain, disfigurement, diplopia, or optic neuropathy.‚ÄČThe presence of multiple schwannomas in a single patient suggests possible association with neurofibromatosis type 2 (NF2) or schwannomatosis.

Case presentation

We present 2 patients who experienced recurrent orbital schwannoma without evidence for neurofibromatosis. The recurrence in one patient, a 59-year old man, occurred 6 years after complete excision of the initial tumor. This recurrence consisted of 2 independent tumors in the same orbit. The recurrence in the second patient, a 5 year-old girl, occurred multiple times within days to weeks of partial excisions until eventually a complete excision was performed.

Conclusion

The clinical history, histopathologic features and particularly the intraoperative findings suggest that the 59 year old man suffers from orbital schwannomatosis, while the rapid recurrence in the second patient correlated with the cellular features of her plexiform schwannoma. Hence, the recurrence in each patient is linked to a different etiology, with implications for treatment and patient counseling given the difficulty in treating orbital schwannomatosis. To our knowledge, this is the first description of isolated orbital schwannomatosis.