Recurrent orbital schwannomas: clinical course and histopathologic correlation

BMC Ophthalmology

Case Report

Steven M Archer¹  Brenda L Bohnsack¹  Alon Kahana²  Jonathan B McHugh³  Michelle Kron⁴ 

[1] Department of Ophthalmology and Visual Sciences, University of Michigan, Ann Arbor, MI, USA;Department of Ophthalmology and Visual Sciences, University of Michigan, Ann Arbor, MI, USA;Helmut F. Stern Career Development Professor, Ophthalmology and Visual Sciences, University of Michigan, Ann Arbor, MI, USA;Department of Pathology, University of Michigan, Ann Arbor, MI, USA;University of Michigan Medical School, Ann Arbor, MI, USA;

关键词: Acute Lymphocytic Leukemia;    Malignant Peripheral Nerve Sheath Tumor;    Complete Excision;    Vestibular Schwannomas;    Juvenile Myelomonocytic Leukemia;   

DOI  :  10.1186/1471-2415-12-44

 received in 2011-12-31, accepted in 2012-08-27,  发布年份 2012

来源: Springer

【 摘 要 】

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

【 授权许可】

Unknown   
© Kron et al.; licensee BioMed Central Ltd. 2012. This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.