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Neuro-Oncologic Considerations in Pediatric Oculoplastic Surgery

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Pediatric Oculoplastic Surgery

Abstract

Primary orbital and periorbital tumors are rare in children and usually benign (Khan and Sepahdari Saudi J Ophthalmol 26(4):373–383, 2012). While some may threaten life, others may cause vision loss and disfigurement. For tumors that threaten only vision, treatment should balance the potential benefit of vision preservation against the potential late effects of surgery and other therapies. Clinical management of orbital tumors varies depending on tumor type and can present challenges to the oculoplastic surgeon. These tumors are best treated by a team of physicians with expertise in the orbital surgery, medical oncology, and radiation oncology as well as any underlying genetic predisposition.

Epidemiological information about orbital and periorbital tumors is scant and often derives from major referral centers whose data may be skewed by population bias based on the orbital tumors in which they specialize (Shields et al. Ophthalmology 111(5):997–1008, 2004; Kodsi et al. Am J Ophthalmol 117(2):177–182, 1994). A review of these reports demonstrates that the most common masses that arise in or near the orbit include benign cysts, inflammatory masses, and vascular lesions, accounting for 50–75% of patients seen (Browning and Camitta Ophthal Plast Reconstr Surg 19(5):340–344, 2003). Masses that are frequently managed with the input of a pediatric neuro-oncologist include benign or malignant tumors with intracranial extension, those that threaten to invade intracranially or that are commonly seen in genetic tumor predisposition syndromes (such as neurofibromatosis types 1 and 2). This chapter will review the most common primary orbital tumors, including optic pathway or optic nerve gliomas, optic nerve sheath meningiomas, orbital/periorbital plexiform neurofibromas (OPPN), and orbital rhabdomyosarcoma. It will review the epidemiology of these masses, their common clinical and radiographic presentations, their diagnosis and treatment considerations, as well as areas of future research. This chapter is not exhaustive, but is intended to provide an introduction to the concerns and considerations of the pediatric oncologist when considering orbital and periorbital masses.

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de Blank, P., Coffey, M.D., Fisher, M.J. (2018). Neuro-Oncologic Considerations in Pediatric Oculoplastic Surgery. In: Katowitz, J., Katowitz, W. (eds) Pediatric Oculoplastic Surgery. Springer, Cham. https://doi.org/10.1007/978-3-319-60814-3_15

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