23 March 2010
Iris melanoma in a patient with neurofibromatosis type 1 and optic nerve glioma in the fellow eye
Krzysztof Mika, Arkadiusz Pogrzebielski, Jolanta Orłowska-Heitzman, Bożena Romanowska-DixonAm J Case Rep 2010; 11:67-69 :: ID: 878477
Abstract
Background: Neurofibromatosis type 1 (NF1) is an autosomal dominant hamartomatous disorder characterized by pigment skin lesions, multiple skin tumors, and spinal and cranial nerve tumors. Uveal melanoma is the most common primary intraocular malignancy in adults. Both diseases are very rarely diagnosed in the same individual.
Case Report: A 32-year-old male with neurofibromatosis type 1 was referred to this clinic with a lesion suspected to be iris melanoma. Taking into consideration optic nerve glioma in the fellow eye, excisional iris biopsy in the left eye was performed. Histopathological examination revealed iris melanoma. Conservative treatment in the form of Ru-106 brachytherapy was chosen. The development of secondary glaucoma and an increase in the iris tumor size led to enucleation.
Conclusions: The common embryological neural crest origin of the tissues involved in the two processes and mutation in the NF1 tumor suppressor gene may explain the higher predominance of uveal melanoma in patients with neurofibromatosis. However, there is still no strong evidence to confirm this thesis. Therefore we should treat the occurrence of both diseases in one patient as a rare coincidence.
Keywords: neurofibromatosis type 1, iris melanoma, Optic Nerve Glioma
In Press
12 Feb 2024 : Case report
Diagnostic Challenges and Imaging Considerations for Intraparotid Facial Nerve Schwannoma: A Case Report an...Am J Case Rep In Press; DOI: 10.12659/AJCR.942870
12 Feb 2024 : Case report
Erdheim-Chester Disease Occult on Radiographs and CT but Visible on MRI and PETAm J Case Rep In Press; DOI: 10.12659/AJCR.941169
12 Feb 2024 : Case report
Surgical Treatment of Spontaneous Superficial Temporal Artery Arteriovenous Malformation: A Case ReportAm J Case Rep In Press; DOI: 10.12659/AJCR.942839
13 Feb 2024 : Case report
Warfarin Woes: A Rare Case of Hemoperitoneum with Intramural Small Bowel HematomaAm J Case Rep In Press; DOI: 10.12659/AJCR.943519
Most Viewed Current Articles
10 Jan 2022 : Case report
A Report on the First 7 Sequential Patients Treated Within the C-Reactive Protein Apheresis in COVID (CACOV...DOI :10.12659/AJCR.935263
Am J Case Rep 2022; 23:e935263
19 Jul 2022 : Case report
Atlantoaxial Subluxation Secondary to SARS-CoV-2 Infection: A Rare Orthopedic Complication from COVID-19DOI :10.12659/AJCR.936128
Am J Case Rep 2022; 23:e936128
05 Mar 2022 : Case report
Acute Limb Ischemia After Self-Injection of Crushed Morphine Tablets into the Radial Artery: Role of Infrar...DOI :10.12659/AJCR.935336
Am J Case Rep 2022; 23:e935336
02 Apr 2022 : Case report
Infective Endocarditis Caused by Pseudomonas luteola in a Pediatric Patient. A Case Report and Review of Li...DOI :10.12659/AJCR.935743
Am J Case Rep 2022; 23:e935743