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Conjunctival and Corneal Tumors: Systemic Associations

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Clinical Ophthalmic Oncology

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Abstract

Conjunctival tumors may be some of the prominent manifestations of systemic inherited diseases. In such instances, ophthalmologists should recognize the systemic association and initiate appropriate systemic and genetic evaluation. Conjunctival tumors and tumor-like conditions associated with systemic disorders can be categorized as pigmented lesions (Peutz–Jeghers syndrome), benign tumors (Goldenhar syndrome), malignant tumors (xeroderma pigmentosum), and amyloidosis. We present a review of this group of conjunctival lesions and their associated systemic disorders. Other entities may also have associated eyelid tumors and are covered in detail elsewhere. Where applicable, the genetic underpinnings of the syndromic association are discussed.

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Abbreviations

AL:

Light chain

CHRPE:

Congenital hypertrophy of the retinal pigment epithelium

GS:

Goldenhar syndrome

MEN:

Multiple endocrine neoplasia

OAV:

Oculoauriculovertebral dysplasia

OAVS:

Oculoauriculovertebral spectrum

PJS:

Peutz–Jeghers syndrome

PS:

Proteus syndrome

TTR:

Transthyretin

XP:

Xeroderma pigmentosum

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Author information

Authors and Affiliations

  1. Department of Pediatric Ophthalmology and Strabismus, Center for Genetic Eye Diseases, Cole Eye Institute (i-32), Cleveland Clinic, Cleveland, OH, USA

    Matteo Scaramuzzi & Elias I. Traboulsi

  2. Department of Ophthalmic Oncology, Cole Eye Institute, Cleveland Clinic, Cleveland, OH, USA

    Lucy T. Xu & Arun D. Singh

Authors
  1. Matteo Scaramuzzi
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  2. Lucy T. Xu
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  3. Arun D. Singh
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  4. Elias I. Traboulsi
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Corresponding author

Correspondence to Elias I. Traboulsi .

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Editors and Affiliations

  1. Ocular Onocology Service and Ophthalmic Pathology Laboratory, Department of Ophthalmology, Hadassah - Hebrew University Medical Center, Jerusalem, Israel

    Jacob Pe'er

  2. Department of Ophthalmic Oncology, Cole Eye Institute, Cleveland Clinic, Cleveland, OH, USA

    Arun D. Singh

  3. Nuffield Department of Clinical Neurosciences, University of Oxford, Oxford, UK

    Bertil E. Damato

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Scaramuzzi, M., Xu, L.T., Singh, A.D., Traboulsi, E.I. (2019). Conjunctival and Corneal Tumors: Systemic Associations. In: Pe'er, J., Singh, A., Damato, B. (eds) Clinical Ophthalmic Oncology. Springer, Cham. https://doi.org/10.1007/978-3-030-06046-6_24

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  • DOI: https://doi.org/10.1007/978-3-030-06046-6_24

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