Intraocular Melanoma as an Initial Presentation of Birt–Hogg–Dubé Syndrome

Birt–Hogg–Dubé syndrome is a rare autosomal-dominant genodermatosis that affects approximately 2 in every 1 million people. A 68-year-old man presented to his yearly wellness visit with concerns of intermittent sensations and blurriness in his left eye, prompting referral to the ophthalmology depart...

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Main Authors: Emily Saurborn, William Snider, Charles Meadows
Format: Article
Language:English
Published: American College of Physicians 2025-06-01
Series:Annals of Internal Medicine: Clinical Cases
Online Access:https://www.acpjournals.org/doi/10.7326/aimcc.2024.1087
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author Emily Saurborn
William Snider
Charles Meadows
author_facet Emily Saurborn
William Snider
Charles Meadows
author_sort Emily Saurborn
collection DOAJ
description Birt–Hogg–Dubé syndrome is a rare autosomal-dominant genodermatosis that affects approximately 2 in every 1 million people. A 68-year-old man presented to his yearly wellness visit with concerns of intermittent sensations and blurriness in his left eye, prompting referral to the ophthalmology department. Fundoscopic examination revealed an intraocular mass and a diagnosis of choroidal melanoma in the left eye was made. The patient received radiation therapy. The patient was referred to a geneticist, who detected a heterozygous germline pathogenic variant in FLCN: c.1389C>G (p.Tyr463*). It is important to incorporate a multidisciplinary approach, including a geneticist, to appropriately manage further work-up.
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series Annals of Internal Medicine: Clinical Cases
spelling doaj-art-8eed9087630d43d1913033da18beef7a2025-08-20T03:21:34ZengAmerican College of PhysiciansAnnals of Internal Medicine: Clinical Cases2767-76642025-06-014610.7326/aimcc.2024.1087Intraocular Melanoma as an Initial Presentation of Birt–Hogg–Dubé SyndromeEmily Saurborn0William Snider1Charles Meadows21Department of Internal Medicine, Joan C. Edwards School of Medicine, Marshall University, Huntington, West Virginia1Department of Internal Medicine, Joan C. Edwards School of Medicine, Marshall University, Huntington, West Virginia1Department of Internal Medicine, Joan C. Edwards School of Medicine, Marshall University, Huntington, West VirginiaBirt–Hogg–Dubé syndrome is a rare autosomal-dominant genodermatosis that affects approximately 2 in every 1 million people. A 68-year-old man presented to his yearly wellness visit with concerns of intermittent sensations and blurriness in his left eye, prompting referral to the ophthalmology department. Fundoscopic examination revealed an intraocular mass and a diagnosis of choroidal melanoma in the left eye was made. The patient received radiation therapy. The patient was referred to a geneticist, who detected a heterozygous germline pathogenic variant in FLCN: c.1389C>G (p.Tyr463*). It is important to incorporate a multidisciplinary approach, including a geneticist, to appropriately manage further work-up.https://www.acpjournals.org/doi/10.7326/aimcc.2024.1087
spellingShingle Emily Saurborn
William Snider
Charles Meadows
Intraocular Melanoma as an Initial Presentation of Birt–Hogg–Dubé Syndrome
Annals of Internal Medicine: Clinical Cases
title Intraocular Melanoma as an Initial Presentation of Birt–Hogg–Dubé Syndrome
title_full Intraocular Melanoma as an Initial Presentation of Birt–Hogg–Dubé Syndrome
title_fullStr Intraocular Melanoma as an Initial Presentation of Birt–Hogg–Dubé Syndrome
title_full_unstemmed Intraocular Melanoma as an Initial Presentation of Birt–Hogg–Dubé Syndrome
title_short Intraocular Melanoma as an Initial Presentation of Birt–Hogg–Dubé Syndrome
title_sort intraocular melanoma as an initial presentation of birt hogg dube syndrome
url https://www.acpjournals.org/doi/10.7326/aimcc.2024.1087
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AT williamsnider intraocularmelanomaasaninitialpresentationofbirthoggdubesyndrome
AT charlesmeadows intraocularmelanomaasaninitialpresentationofbirthoggdubesyndrome