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Diagnostic and therapeutic considerations in patients with bilateral diffuse uveal melanocytic proliferation

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Abstract

Purpose

This review aims to summarize the current knowledge concerning the clinical features, diagnostic work-up, and therapeutic approach of bilateral diffuse uveal melanocytic proliferation (BDUMP).

Methods

A meticulous literature search was performed in the PubMed database. A supplementary search was made in Google Scholar to complete the collected items. Our search strategy utilized the following keywords: “bilateral diffuse uveal melanocytic proliferation”, “BDUMP”, and “Paraneoplastic Syndrome”. Articles were considered based on their relevance, with the search spanning publications up to 2023. Studies were excluded if they did not contribute pertinent information or lacked methodological rigor. A critical appraisal of included studies was conducted, assessing study design, sample size, methodology, and potential bias, ensuring a thorough and transparent review process.

Results

BDUMP is a rare and potentially sight-threatening condition characterized by the bilateral proliferation of melanocytes within the uvea. BDUMP is typically observed in middle-aged or elderly individuals and is often associated with an underlying malignancy, most commonly of gastrointestinal origin. BDUMP is frequently misdiagnosed as a benign nevus or choroidal metastasis, leading to delayed diagnosis and treatment. The ophthalmic symptoms and signs typically precede the diagnosis of a systemic malignancy, emphasizing the crucial role of ophthalmologists in the recognition of BDUMP. Several diagnostic modalities can aid in the diagnosis of BDUMP, including ophthalmic examination, imaging studies such as optical coherence tomography, fluorescein angiography, and indocyanine green angiography, and biopsy of the uveal tissue. Treatment of BDUMP is directed towards the underlying malignancy and may include chemotherapy, radiotherapy, or surgical resection. Additionally, strict monitoring with regular follow-ups may contribute to the detection of new lesions and the reduction in the size of existing ones.

Conclusions

BDUMP can be considered a potential biomarker in the management of malignancies, especially when the primary underlying tumor has not been detected. Further research is needed to better understand the pathogenesis of BDUMP and its association with malignancy.

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Authors and Affiliations

  1. Southampton Eye Unit, University Hospital Southampton, Tremona Rd, Southampton, SO16 6YD, UK

    Dimitrios Kalogeropoulos, Farid Afshar, Gabriella De Salvo, Christina A. Rennie & Andrew John Lotery

  2. Faculty of Medicine, University of Southampton, Southampton, UK

    Andrew John Lotery

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  1. Dimitrios Kalogeropoulos
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  2. Farid Afshar
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  3. Gabriella De Salvo
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  4. Christina A. Rennie
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All authors contributed to the study’s conception and design. Material preparation, data collection, and analysis were performed by DK and GDS. The first draft of the manuscript was written by DK and all authors commented on previous versions of the manuscript. All authors read and approved the final manuscript.

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Correspondence to Gabriella De Salvo.

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Kalogeropoulos, D., Afshar, F., De Salvo, G. et al. Diagnostic and therapeutic considerations in patients with bilateral diffuse uveal melanocytic proliferation. Int Ophthalmol 44, 149 (2024). https://doi.org/10.1007/s10792-024-03087-9

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