Purpose: In a population-based cohort of 960 uveal melanoma (UM) patients, we describe patients with three additional malignancies, including one unique patient with four synchronous primary malignancies.
Method: A descriptive presentation of the clinical course and outcome for UM patients with three additional primary malignancies.
Results: After more than 20 years of follow-up of the UM cohort, 11 patients (1.1%) were diagnosed with three additional primary malignancies before, simultaneously or after UM. Among these, one patient had four synchronous primary malignancies, detected during workup for a symptomatic UM. All diagnoses were treated during the following 4 months, firstly the breast cancer, thereafter, the lung and pancreatic cancers and finally the UM. The patient died 3 years later of abdominal carcinomatosis due to the pancreatic cancer. The family history and gene testing did not disclose any genetic predisposition for cancer. A comparison of the four synchronous tumours, morphologically and immunohistochemically, showed no similarities and the expression of antibodies was different.
Conclusion: Patients with UM may be diagnosed with non-ocular additional primary cancers. Thus, a comprehensive workup is obligatory and a further follow-up of the UM patients seems necessary. The UM is not always the main problem.
Keywords: BAP1; additional primary malignancy; immunohistochemistry; synchronous cancers; uveal melanoma.
© 2024 The Authors. Acta Ophthalmologica published by John Wiley & Sons Ltd on behalf of Acta Ophthalmologica Scandinavica Foundation.