WHO classification of eye tumours

Last revised by Craig Hacking on 20 Oct 2022

The World Health Organizatiοn (WHO) classification of eye tumours is a widely used pathologic classification system of neoplasms of the orbit. The current revision, part of the 4th edition of the WHO series, was published in 2018 and is reflected in the article below 1.

Classification

Tumours of the conjunctiva and caruncle
  • epithelial tumours

    • conjunctival squamous cell carcinoma

    • conjunctival spindle cell carcinoma

    • adenosquamous carcinoma

    • keratoacanthoma

    • hereditary benign intraepithelial dyskeratosis of the conjunctiva

    • sebaceous carcinoma

    • oncocytoma

    • conjunctival squamous papilloma

      • tumour-like lesion

      • cysts

      • reactive epithelial hyperplasia

      • pseudoglandular and pseudoepitheliomatous hyperplasia

  • melanocytic tumours

    • conjunctival melanoma

    • conjunctival melanocytic intraepithelial neoplasia

    • conjunctival junctional, compound, and subepithelial naevi

    • inflamed juvenile conjunctival naevus

    • blue naevus of the conjunctiva

    • Spitz naevus of the conjunctiva

    • benign epithelial melanosis of the conjunctiva

  • hematolymphoid tumours

    • lymphomas

    • papillary/follicular conjunctivitis

    • reactive lymphoid hyperplasia

  • soft tissue tumours

    • leiomyosarcoma

    • rhabdomyosarcoma

    • Kaposi sarcoma

    • angiosarcoma

    • fibrous histiocytoma

    • conjunctival stromal tumour

    • myxoma

    • haemangioma

    • lymphangioma

    • neurofibroma

    • schwannoma

    • juvenile xanthogranuloma

  • hamartomas and choristomas

    • epibulbar choristoma

    • epibulbar osseous choristoma

    • phakomatous choristoma

  • secondary tumours

Tumours of the iris, ciliary body, and choroid
  • tumours of the iris

    • iris melanoma

    • melanocytic naevus

    • ocular melanocytosis

    • Lisch nodule

    • pigmented epithelial cyst

    • implantation cyst

    • ectopic lacrimal gland

    • histiocytic tumours

  • tumours of the ciliary body

  • tumours of the choroid

  • secondary tumours

Tumours of the retina and retinal pigment epithelium
  • tumours of the neurosensory retina

    • retinoblastoma

    • retinocytoma

    • astrocytic hamartoma and astrocytoma

    • nodular and massive retinal gliosis

    • haemangioblastoma

    • cavernous haemangioma

    • vitreoretinal lymphoma

    • leukaemic infiltration

    • secondary tumours

  • tumours of the retinal pigment epithelium

    • adenocarcinoma

    • adenoma

    • reactive hyperplasia

    • simple and combined hamartomas

    • congenital hypertrophy

Tumours of the optic disc and optic nerve
Tumours of the lacrimal gland
  • epithelial tumours

    • adenoid cystic carcinoma

    • mucoepidermoid carcinoma

    • carcinosarcoma

    • carcinoma ex pleomorphic adenoma

    • pleomorphic adenoma

    • myoepithelial tumours

    • oncocytic tumours

    • rare neoplasms

      • epithelial-myoepithelial carcinoma

      • low-grade intraductal carcinoma

      • secretory carcinoma

      • hybrid neoplasms

      • acinic cell carcinoma

      • Warthin tumour

  • hematolymphoid tumours

    • lymphomas

    • reactive lymphoid hyperplasia

  • secondary tumours

Tumours of the lacrimal drainage system
  • epithelial tumours

    • squamous cell carcinoma

      • non-keratinising squamous cell carcinoma

    • adenocarcinoma

    • mucoepidermoid carcinoma

    • adenoid cystic carcinoma

    • lymphoepithelial carcinoma

    • exophytic papilloma

    • inverted papilloma

  • other tumours

    • melanoma

    • lymphomas

    • secondary tumours

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