WHO classification of eye tumors

Last revised by Craig Hacking on 20 Oct 2022

The World Health Organizatiοn (WHO) classification of eye tumors 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

Tumors of the conjunctiva and caruncle
  • epithelial tumors

    • conjunctival squamous cell carcinoma

    • conjunctival spindle cell carcinoma

    • adenosquamous carcinoma

    • keratoacanthoma

    • hereditary benign intraepithelial dyskeratosis of the conjunctiva

    • sebaceous carcinoma

    • oncocytoma

    • conjunctival squamous papilloma

      • tumor-like lesion

      • cysts

      • reactive epithelial hyperplasia

      • pseudoglandular and pseudoepitheliomatous hyperplasia

  • melanocytic tumors

    • conjunctival melanoma

    • conjunctival melanocytic intraepithelial neoplasia

    • conjunctival junctional, compound, and subepithelial nevi

    • inflamed juvenile conjunctival nevus

    • blue nevus of the conjunctiva

    • Spitz nevus of the conjunctiva

    • benign epithelial melanosis of the conjunctiva

  • hematolymphoid tumors

    • lymphomas

    • papillary/follicular conjunctivitis

    • reactive lymphoid hyperplasia

  • soft tissue tumors

    • leiomyosarcoma

    • rhabdomyosarcoma

    • Kaposi sarcoma

    • angiosarcoma

    • fibrous histiocytoma

    • conjunctival stromal tumor

    • myxoma

    • hemangioma

    • lymphangioma

    • neurofibroma

    • schwannoma

    • juvenile xanthogranuloma

  • hamartomas and choristomas

    • epibulbar choristoma

    • epibulbar osseous choristoma

    • phakomatous choristoma

  • secondary tumors

Tumors of the iris, ciliary body, and choroid
  • tumors of the iris

    • iris melanoma

    • melanocytic nevus

    • ocular melanocytosis

    • Lisch nodule

    • pigmented epithelial cyst

    • implantation cyst

    • ectopic lacrimal gland

    • histiocytic tumors

  • tumors of the ciliary body

  • tumors of the choroid

    • choroidal and ciliary body melanomas

    • choroidal and ciliary body nevi

    • bilateral diffuse uveal melanocytic hyperplasia

    • neurofibroma and ganglioneuroma

    • schwannoma

    • hemangioma

    • osteoma

    • primary choroidal lymphoma

    • secondary lymphomatous infiltration

    • leukemic infiltration

  • secondary tumors

Tumors of the retina and retinal pigment epithelium
  • tumors of the neurosensory retina

    • retinoblastoma

    • retinocytoma

    • astrocytic hamartoma and astrocytoma

    • nodular and massive retinal gliosis

    • hemangioblastoma

    • cavernous hemangioma

    • vitreoretinal lymphoma

    • leukemic infiltration

    • secondary tumors

  • tumors of the retinal pigment epithelium

    • adenocarcinoma

    • adenoma

    • reactive hyperplasia

    • simple and combined hamartomas

    • congenital hypertrophy

Tumors of the optic disc and optic nerve
Tumors of the lacrimal gland
  • epithelial tumors

    • adenoid cystic carcinoma

    • mucoepidermoid carcinoma

    • carcinosarcoma

    • carcinoma ex pleomorphic adenoma

    • pleomorphic adenoma

    • myoepithelial tumors

    • oncocytic tumors

    • rare neoplasms

      • epithelial-myoepithelial carcinoma

      • low-grade intraductal carcinoma

      • secretory carcinoma

      • hybrid neoplasms

      • acinic cell carcinoma

      • Warthin tumor

  • hematolymphoid tumors

    • lymphomas

    • reactive lymphoid hyperplasia

  • secondary tumors

Tumors of the lacrimal drainage system
  • epithelial tumors

    • squamous cell carcinoma

      • non-keratinizing squamous cell carcinoma

    • adenocarcinoma

    • mucoepidermoid carcinoma

    • adenoid cystic carcinoma

    • lymphoepithelial carcinoma

    • exophytic papilloma

    • inverted papilloma

  • other tumors

    • melanoma

    • lymphomas

    • secondary tumors

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