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Neuroendocrine Tumor of the Eyelid A Clinicopathological Case Report

Clemens Lange, MD, PhD1; Annette Schmitt-Graeff, MD2; Hans Mittelviefhaus, MD1; Katharina Beinhofer, MD1; Claudia Auw-Haedrich, MD1
[+] Author Affiliations
1University Eye Hospital Freiburg, Freiburg, Germany
2Institute for Pathology, Freiburg, Germany
JAMA Ophthalmol. 2014;132(5):652-654. doi:10.1001/jamaophthalmol.2014.132.
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Neuroendocrine neoplasms are a rare but challenging oncological entity. They originate from neuroendocrine cells, which are located in different organs such as the lungs, the gastrointestinal system, and the skin and serve as modulators of appetite, airway chemoreceptors, and receptors for tactile stimuli. According to the European Neuroendocrine Tumor Society, well-differentiated low-mitosis tumors (grades 1 and 2) are referred to as neuroendocrine tumors (NETs), whereas the poorly differentiated, high-mitosis neoplasms such as Merkel cell carcinoma (MCC) of the eyelid are classified as high-grade (grade 3) neuroendocrine carcinomas (NECs) (Table).1 We present for the first time, to our knowledge, a clinical-histopathological correlation of a patient with a primary, well-differentiated NET of the eyelid.

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Clinical Photograph and Staining

Macroscopic appearance of the eyelid tumor (A), histological analysis (hematoxylin-eosin) (B), and immunohistochemical staining for synaptophysin (C), cytokeratin 19 (D), MIB-1 (E), and cytokeratin 20 (F) (original magnification ×100 [B-E] and ×200 [F]).

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