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A 10-year-old girl with multiple eyelid neuroproliferative tumors
Digital Journal of Ophthalmology 2021
Volume 27, Number 3
July 12, 2021
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Kristin Torroella | George Washington School of Medicine and Health Sciences, Washington, DCGeorge Washington School of Medicine and Health Sciences, Washington, DC Jana Bregman, MD | Department of Ophthalmology, Childrens National Hospital, Washington, DC Maria Isabel Almira-Suarez, MD, FASCP | Neuropathology Service, Division of Anatomic Pathology, Childrens National Hospital, Washington, DC Marijean Miller, MD | Department of Ophthalmology, Childrens National Hospital, Washington, DC
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| Differential Diagnosis | This patient’s past medical history of MEN2B, thickened eyelids, prominent corneal nerves and reoccurring eyelid lesions prompted a most likely diagnosis of multiple eyelid schwannomas. However, it was important to consider other possible eyelid lesions, including both benign and malignant tumors. Chalazia are the most common benign eyelid tumors resulting from blockage of a meibomian gland and present as dome-shaped, elevated, smooth lesions, much like our patient’s.(1,2) However, histopathology of a chalazion would show signs of chronic inflammation, not present in our patient.(3) Neurofibroma was also considered, given the known association with MEN2B, although it is more commonly found in neurofibromatosis type 1.(4) Plexiform neurofibromas are benign nerve sheath tumors that may cause proptosis, ptosis, and cosmetic disfigurement.(4) On histopathology, they present as a combination of Schwann cells, axons, perineural cells, and fibroblasts, whereas schwannomas are composed predominantly of Schwann cells.(5) Plexiform neurofibromas have malignant potential, unlike our patient’s plexiform schwannomas, which have no malignant potential.(4) Additional benign lesions, such as epidermal inclusion cyst, sebaceous cyst, and papilloma were less likely, based on appearance.
Malignant eyelid lesions are generally rare in the pediatric population. Examples include basal cell carcinoma, squamous cell or sebaceous cell carcinoma, and keratoacanthoma, which is categorized as a premalignant lesion. Malignant features such as sentinel vessels, bleeding or ulceration, distortion of the lid margin, or madarosis were all absent in this case.
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