WHO classification of eye tumors

The World Health Organizatiοn (WHO) classification of eye tumors is a widely used pathologic classification system of neoplasms of the orbit. The revision, part of the 4^th 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

  • uveal melanocytoma

  • adenocarcinoma

  • ciliary body adenoma

  • adenomatous hyperplasia

  • reactive epithelial hyperplasia

  • medulloepithelioma of the ciliary body

  • leiomyoma

  • schwannoma

  • glioneuroma

• 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

• primary tumors

  • astrocytoma and glioblastoma

  • meningioma of the optic nerve

  • medulloepithelioma

  • solitary fibrous tumor/hemangiopericytoma

  • melanocytoma

• secondary tumors

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