Choroidal Paraganglioma with Metastases to the Fellow Eye

Autor: Rita Van Ginderdeuren, Guy S. Missotten, Joost van den Oord
Jazyk: angličtina
Rok vydání: 2013
Předmět:
Zdroj: Case Reports in Ophthalmology, Vol 4, Iss 1, Pp 17-22 (2013)
Druh dokumentu: article
ISSN: 1663-2699
DOI: 10.1159/000347169
Popis: Purpose: To report a case of a paraganglioma in the right eye with metastatic disease in the fellow eye 3 years later. Methods: A 70-year-old man presented with a painful amblyopic right eye; rubeosis iridis and a large choroidal tumor were found. The tumor was treated by enucleation. Pathology diagnosed the tumor as a paraganglioma. Screening for other tumors or metastatic disease was negative at that moment. After 3 years, a paraganglioma skin metastasis was detected, and screening revealed metastatic disease in the liver. Another 6 months later he was referred for tumors in the left eye, which were treated by radiotherapy. He succumbed 6 months later. Results: Histopathology of the right eye revealed the typical image of a paraganglioma, with expression of synaptophysin, neuron-specific enolase and chromogranin. S-100 staining was positive in the sustentacular cells; staining for HMB-45, SME, EMA and pan-keratin was negative. Microscopy of the tumors in the skin and liver 3 years later showed a dedifferentiated tumor with the same immunological characteristics, but with higher Ki67 expression and more mitoses. Conclusions: This report documents a very rare choroidal paraganglioma which presented clinically as a melanoma. The patient succumbed 4 years later to generalized metastatic disease. No other primary paraganglioma was found; however, paraganglion cells in the eye have never been described.
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