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PATHOLOGY OF COMMON
OCULAR AND ORBITAL
TUMOURS
Epithelial tumours
■ Squamous cell carcinoma NOS
■ Squamous cell carcinoma In situ with questionable stromal
invasion
■ Squamous cell carcinoma , keratinizing , NOS
■ Basaloid squamous cell carcinoma
■ Spindle cell carcinoma
■ Mucoepidermoid carcinoma
■ Adenosquamous carcinoma
■ Keratoacanthoma
■ Hereditary benign intraepithelial dyskeratosis
■ Conjunctival squamous intraepithelial neoplasia
■ Sebaceous carcinoma
■ Oncocytoma
■ Squamous papilloma
■ Tumour like lesions
■ Cysts
■ Reactive epithelial hyperplasia
■ Pseudoglandular and pseudoepitheliomatous hyperplasia
Melanocytic tumours
■ Melanoma NOS
■ Nodular melanoma
■ Melanoma in situ
■ Junctional naevus
■ Compound naevus
■ Subepithelial naevus
■ Inflamed juvenile naevus
■ Blue naevus
■ Spitz naevus
■ Benign epithelial melanoses
Hematolymphoid tumours
■ Lymphoma
■ Papillary / follicular conjunctivitis
■ Reactive lymphoid hyperplasia
■ Soft tissue tumours
■ Leiomyosarcoma
■ Rhabdomyosarcoma NOS
■ Embryonal rhabdomyosarcoma
■ Spindle cell rhabdomyosarcoma
■ Botyroid rhabdomyosarcoma
■ Anaplastic rhabdomyosarcoma
■ Alveolar rhabdomyosarcoma
■ Pleomorphic rhabdomyosarcoma
■ Kaposi sarcoma
■ Angiosarcoma
■ Benign fibrous histiocytoma
■ Conjunctival stromal tumour
■ Myxoma
■ Hemangioma
■ Lymphangioma
■ Neurofibroma
■ Schwannoma
■ Juvenile xanthogranuloma
Hamartomas and choriostomas
■ Epibulbar choristoma
■ Epibulbar osseous choristoma
■ Phakomatous choristoma
■ Secondary tumours
WHO classification of tumours of
the iris , ciliary body , choroid
■ Tumours of the iris
■ Uveal melanoma
■ Iris melanoma
■ Iris naevus
■ Ocular melanocytosis
■ Lisch nodule
■ Pigmented epithelial cyst
■ Implantation cyst
■ Ectopic lacrimal gland
■ Histiocytic tumours
■ Tumours of the ciliary body
■ Melanocytoma
■ Ciliary body adenocarcinoma
■ Ciliary body adenoma
■ Adenomatous hyperplasia
■ Reactive epithelial hyperplasia
■ Medulloepithelioma , benign
■ Medulloepithelioma , malignant
■ Teratoid medulloepithelioma , benign
■ Teratoid medulloepithelioma , malignant
■ Leiomyoma
■ Schwannoma
■ Glioneuroma
WHO classification of the tumours of
the retina and retinal pigment
epithelium
■ Tumours of the neurosensory
retina
■ Retinoblastoma
■ Astrocytoma NOS
■ Nodular and massive retinal gliosis
■ Hemangioblstoma
■ Cavernous hemangioma
■ Vitreoretinal lymphoma
■ Leukemia NOS
■ Secondary tumours
■ Tumours of the retinal
pigment epithelium
■ Retinal pigment epithelium
adenocarcinoma
■ Retinal pigment epithelium
adenoma
■ Reactive hyperplasia
■ Simple and combined hamartomas
■ Congenital hypertrophy
WHO classification of tumours of
the optic disc and optic nerve
■ Primary tumours
■ Astrocytic NOS
■ Pilocytic astrocytoma
■ Glioblastoma
■ Meningioma NOS
■ Medulloepithelioma , benign
■ Medulloepithelioma , malignant
■ Teratoid medulloepithelioma , benign
■ Teratoid medulloepithelioma , malignant
■ Solitary fibrous tumour NOS
■ Melanocytoma
■ Secondary tumours
WHO classification of tumours of
the lacrimal gland
Epithelial tumours
■ Adenoid cystic carcinoma
■ Mucoepidermoid carcinoma
■ Adenosquamous carcinoma
■ Carcinosarcoma
■ Adenocarcinoma of the lacrimal
gland
■ Carcinoma ex pleomorphic
adenoma
■ Pleomorphic adenoma
■ Myoepithelioma
■ Myoepithelial carcinoma
■ Hematolympoid tumours
■ Extranodal marginal zone
lymphoma of mucosa associated
lymphoid tissue
■ Follicular lymphoma
■ Diffuse large B cell lymphoma
■ Reactive lymphoid hyperplasia
■ Secondary tumours
■ Oncocytoma
■ Oncocytic carcinoma
■ Epithelial- myoepithelial carcinoma
■ Low grade intraductal carcinoma
■ Secretory carcinoma
■ Hybrid neoplasms
■ Acinic cell carcinoma
■ Adenolymphoma /warthin tumour
WHO classification of tumours of
the lacrimal drainage system
■ Epithelial tumours
■ Squamous cell carcinoma NOS
■ Non – keratinizing squamous cell
carcinoma
■ Adenocarcinoma NOS
■ Mucoepidermoid carcinoma
■ Adenosquamous carcinoma
■ Adenoid cystic carcinoma
■ Lymphoepithelial carcinoma
■ Sinonasal papilloma , inverted
type
■ Sinonasal papilloma , exophytic
■ Other tumours
■ Melanoma NOS
■ Extranodal marginal zone
lymphoma of mucosa associated
lymphoid tissue
■ Secondary tumours
Steps for processing of specimen
■ Fixation to preserve the tissue
■ Gross dissection to prepare the specimen for histologic sectioning
■ Tissue processing
■ Slide preparation and tissue staining
Hemangioma
■ Capillary hemangioma , the most common periocular vascular
tumour in infancy and childhood
■ Solitary , bright red , smooth
■ A variant of capillary hemangioma , often called
hemangioendothelioma is observed at birth , infancy or early
childhood
■ Histologically , the capillary hemangioma is composed primarily
of capillaries lined by plump endothelial cells
Capillary hemangioma : clinical
appearance of tumor
Tumor composed of blood vessels of
predominantly capillary size
High magnification shows capillaries
and endothelial cells
■ Cavernous hemangioma is the most common primary orbital
tumour producing exophthalmos
■ It is a well- encapsulated tumour , usually within the muscle
cone
Gross appearance of surgically
removed hemangioma
Histologic section shows large, blood filled spaces
of tumor lined by endothelium and separated by
fibrous septa of different thicknesses
Diagnostic criteria for extranodal
lymphomas
■ Histologic criteria
■ Features strongly associated with lymphoma
■ Relatively monomorphic lymphoid infiltrate
■ Cytologic atypia
■ Infiltrative growth with effacement of architecture
■ Plasma cells
■ Dutcher bodies
■ Features suggestive of lymphoma
■ Lymphoepithelial lesions
■ Reactive features often associated with low-grade extranodal
lymphomas
■ Reactive follicular centers , mantle zones often irregular or
absent
Non-Hodgkin’s lymphoma – B cell clinical
appearance of left exophthalmos
Histologic section shows extensive
nodular lymphoid infiltrate of orbital
tissue
Increased magnification shows monotonous
uniform lymphocytic infiltrate and many
mitotic figures
Burkitt’s lymphoma : African boy has
massive left orbital infiltrate
Histocytes , which often display phagocytosis
, scattered among lymphoid tumor cells ,
giving characteristic starry-sky appearance
Undifferentiated, anaplastic , large lymphoid cells
contain ovoid vesicular basophilic nuclei and
prominent nucleoli . Histiocytes contain abundant ,
almost clear cytoplasm and phagocytosed cellular
debris
Malignant melanoma : a pigmented
conjunctival lesion near the limbus , present
since childhood , had undergone recent rapid
growth
Increased magnification shows
spindle cell nature of tumor
Epitheliod cell tumour
Immunohistochemistry -IHC
■ Positive stains :
■ S100(nuclear and cytoplasmic )
■ SOX10(nuclear)
■ MelanA /MART1( Cytoplasmic)
■ HMB45(Cytoplasmic)
■ Tyrosinase (cytoplasmic )
■ MiTF(nuclear)
Retinoblastoma
■ One of the most common childhood malignancies
■ The most common childhood intraocular neoplasm
■ 20% of the world’s retinoblastoma patients live in india , which
has 1500 new cases annually , more cases than any other country
Characteristically, sections of retinoblastoma stained with
hematoxylin and eosin and viewed under low
magnification show dark blue areas surrounded by light
pink areas . The dark areas represent viable cells and
calcium deposition , whereas the light areas represent
tumour necrosis
Increased magnification shows viable (dark blue) tumor cells
clustered around central blood vessels and surrounded in turn by
a mantle of necrotic cells (pink).Numerous Flexner-wintersteiner
rosettes are present
Types of rosettes : A Flexner- wintersteiner rosette
consists of a central lumen lined by cuboidal tumour cells
that contain nuclei positioned basally (away from the
lumen )
Homer Wright rosettes are found more frequently in
medulloblastomas and neuroblastomas than in retinoblastomas.
In these rosettes , the cells line up around an acellular area that
contains cobweb- like material
Fleurettes are flower like groupings of tumor cells in the
retinoblastoma . The cells of fleurettes show clear
evidence of differentiation into photoreceptor elements
Immunohistochemistry - IHC
■ POSITIVE STAINS
■ SOX2
■ MAP2
■ Neuron specific enolase
■ Synatophysin
■ Retinoblastoma associated protein
Papilloma with dysplasia : clinical appearance of typical
limbal sessile conjunctival papilloma
Histologic section shows a sudden and abrupt transition from the
normal conjunctival to a markedly thickened epithelium. The
lesion is broad based and shows numerous blood vessels
penetrating into the thickened epithelium
Squamous cell carcinoma : the patient had a vascularised
elevated pearly lesion at the temporal limbus in the right
eye
Histologic section shows full thickness atypia and loss of
polarity . A diagnosis of carcinoma in situ would be made
here
Malignant epithelial cells in the substantia propria of the
conjunctiva ,forming keratin pearls in some areas
representing invasive squamous cell carcinoma
Pigmented squamous cell carcinoma : clinical photograph
of elevated, pigmented temporal conjunctival lesion
Pigmented dendritic melanocyte among atypical squamous
cells
Immunohistochemistry -IHC
■ Positive stains :
■ P53: increased immunoreactivity in dyplasia
■ Ki67: increased proliferation rates in suprabasal epithelium in
dysplasia
■ P16: greater intensity and percentage staining in high grade
dysplasia
Sebaceous gland carcinoma: upper lid lesion resembles a
chalazion . Note loss of cilia in area of lesion
Excisional biopsy shows large tumour nodules in the
dermis, most of which exhibit central necrosis
Increased magnification shows numerous cells resembling
sebaceous cells. A number of mitotic figures are present
Oil red-O fat stain shows marked positivity in the
cytoplasm of abnormal cells
Large tumour cells are scattered throughout the surface
epidermis , simulating paget’s disease(i.e.pagetoid change). The
cancerous invasion of the epithelium can cause a chronic
blepharoconjunctivitis (masquerade syndrome)
Immunohistochemistry –IHC
■ Positive stains
■ Androgen receptor
■ Keratin
■ EMA
■ LeUM1
■ KI67-increased relative to sebaceous adenoma and sebaceoma
■ P53-increased relative to sebaceous adenoma and sebaceoma
■ BerEP4
Thank you

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Pathology of common ocular and orbital tumours.pptx

  • 1. PATHOLOGY OF COMMON OCULAR AND ORBITAL TUMOURS
  • 2. Epithelial tumours ■ Squamous cell carcinoma NOS ■ Squamous cell carcinoma In situ with questionable stromal invasion ■ Squamous cell carcinoma , keratinizing , NOS ■ Basaloid squamous cell carcinoma ■ Spindle cell carcinoma ■ Mucoepidermoid carcinoma ■ Adenosquamous carcinoma
  • 3. ■ Keratoacanthoma ■ Hereditary benign intraepithelial dyskeratosis ■ Conjunctival squamous intraepithelial neoplasia ■ Sebaceous carcinoma ■ Oncocytoma ■ Squamous papilloma ■ Tumour like lesions ■ Cysts ■ Reactive epithelial hyperplasia ■ Pseudoglandular and pseudoepitheliomatous hyperplasia
  • 4. Melanocytic tumours ■ Melanoma NOS ■ Nodular melanoma ■ Melanoma in situ ■ Junctional naevus ■ Compound naevus ■ Subepithelial naevus ■ Inflamed juvenile naevus ■ Blue naevus ■ Spitz naevus ■ Benign epithelial melanoses
  • 5. Hematolymphoid tumours ■ Lymphoma ■ Papillary / follicular conjunctivitis ■ Reactive lymphoid hyperplasia ■ Soft tissue tumours ■ Leiomyosarcoma ■ Rhabdomyosarcoma NOS ■ Embryonal rhabdomyosarcoma ■ Spindle cell rhabdomyosarcoma ■ Botyroid rhabdomyosarcoma ■ Anaplastic rhabdomyosarcoma
  • 6. ■ Alveolar rhabdomyosarcoma ■ Pleomorphic rhabdomyosarcoma ■ Kaposi sarcoma ■ Angiosarcoma ■ Benign fibrous histiocytoma ■ Conjunctival stromal tumour ■ Myxoma ■ Hemangioma ■ Lymphangioma ■ Neurofibroma ■ Schwannoma ■ Juvenile xanthogranuloma
  • 7. Hamartomas and choriostomas ■ Epibulbar choristoma ■ Epibulbar osseous choristoma ■ Phakomatous choristoma ■ Secondary tumours
  • 8. WHO classification of tumours of the iris , ciliary body , choroid ■ Tumours of the iris ■ Uveal melanoma ■ Iris melanoma ■ Iris naevus ■ Ocular melanocytosis ■ Lisch nodule ■ Pigmented epithelial cyst ■ Implantation cyst ■ Ectopic lacrimal gland ■ Histiocytic tumours
  • 9. ■ Tumours of the ciliary body ■ Melanocytoma ■ Ciliary body adenocarcinoma ■ Ciliary body adenoma ■ Adenomatous hyperplasia ■ Reactive epithelial hyperplasia ■ Medulloepithelioma , benign ■ Medulloepithelioma , malignant ■ Teratoid medulloepithelioma , benign ■ Teratoid medulloepithelioma , malignant ■ Leiomyoma ■ Schwannoma ■ Glioneuroma
  • 10. WHO classification of the tumours of the retina and retinal pigment epithelium ■ Tumours of the neurosensory retina ■ Retinoblastoma ■ Astrocytoma NOS ■ Nodular and massive retinal gliosis ■ Hemangioblstoma ■ Cavernous hemangioma ■ Vitreoretinal lymphoma ■ Leukemia NOS ■ Secondary tumours ■ Tumours of the retinal pigment epithelium ■ Retinal pigment epithelium adenocarcinoma ■ Retinal pigment epithelium adenoma ■ Reactive hyperplasia ■ Simple and combined hamartomas ■ Congenital hypertrophy
  • 11. WHO classification of tumours of the optic disc and optic nerve ■ Primary tumours ■ Astrocytic NOS ■ Pilocytic astrocytoma ■ Glioblastoma ■ Meningioma NOS ■ Medulloepithelioma , benign ■ Medulloepithelioma , malignant ■ Teratoid medulloepithelioma , benign ■ Teratoid medulloepithelioma , malignant ■ Solitary fibrous tumour NOS ■ Melanocytoma ■ Secondary tumours
  • 12. WHO classification of tumours of the lacrimal gland Epithelial tumours ■ Adenoid cystic carcinoma ■ Mucoepidermoid carcinoma ■ Adenosquamous carcinoma ■ Carcinosarcoma ■ Adenocarcinoma of the lacrimal gland ■ Carcinoma ex pleomorphic adenoma ■ Pleomorphic adenoma ■ Myoepithelioma ■ Myoepithelial carcinoma ■ Hematolympoid tumours ■ Extranodal marginal zone lymphoma of mucosa associated lymphoid tissue ■ Follicular lymphoma ■ Diffuse large B cell lymphoma ■ Reactive lymphoid hyperplasia ■ Secondary tumours
  • 13. ■ Oncocytoma ■ Oncocytic carcinoma ■ Epithelial- myoepithelial carcinoma ■ Low grade intraductal carcinoma ■ Secretory carcinoma ■ Hybrid neoplasms ■ Acinic cell carcinoma ■ Adenolymphoma /warthin tumour
  • 14. WHO classification of tumours of the lacrimal drainage system ■ Epithelial tumours ■ Squamous cell carcinoma NOS ■ Non – keratinizing squamous cell carcinoma ■ Adenocarcinoma NOS ■ Mucoepidermoid carcinoma ■ Adenosquamous carcinoma ■ Adenoid cystic carcinoma ■ Lymphoepithelial carcinoma ■ Sinonasal papilloma , inverted type ■ Sinonasal papilloma , exophytic ■ Other tumours ■ Melanoma NOS ■ Extranodal marginal zone lymphoma of mucosa associated lymphoid tissue ■ Secondary tumours
  • 15. Steps for processing of specimen ■ Fixation to preserve the tissue ■ Gross dissection to prepare the specimen for histologic sectioning ■ Tissue processing ■ Slide preparation and tissue staining
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  • 20. Hemangioma ■ Capillary hemangioma , the most common periocular vascular tumour in infancy and childhood ■ Solitary , bright red , smooth ■ A variant of capillary hemangioma , often called hemangioendothelioma is observed at birth , infancy or early childhood ■ Histologically , the capillary hemangioma is composed primarily of capillaries lined by plump endothelial cells
  • 21. Capillary hemangioma : clinical appearance of tumor
  • 22. Tumor composed of blood vessels of predominantly capillary size
  • 23. High magnification shows capillaries and endothelial cells
  • 24. ■ Cavernous hemangioma is the most common primary orbital tumour producing exophthalmos ■ It is a well- encapsulated tumour , usually within the muscle cone
  • 25. Gross appearance of surgically removed hemangioma
  • 26. Histologic section shows large, blood filled spaces of tumor lined by endothelium and separated by fibrous septa of different thicknesses
  • 27. Diagnostic criteria for extranodal lymphomas ■ Histologic criteria ■ Features strongly associated with lymphoma ■ Relatively monomorphic lymphoid infiltrate ■ Cytologic atypia ■ Infiltrative growth with effacement of architecture ■ Plasma cells
  • 28. ■ Dutcher bodies ■ Features suggestive of lymphoma ■ Lymphoepithelial lesions ■ Reactive features often associated with low-grade extranodal lymphomas ■ Reactive follicular centers , mantle zones often irregular or absent
  • 29. Non-Hodgkin’s lymphoma – B cell clinical appearance of left exophthalmos
  • 30. Histologic section shows extensive nodular lymphoid infiltrate of orbital tissue
  • 31. Increased magnification shows monotonous uniform lymphocytic infiltrate and many mitotic figures
  • 32. Burkitt’s lymphoma : African boy has massive left orbital infiltrate
  • 33. Histocytes , which often display phagocytosis , scattered among lymphoid tumor cells , giving characteristic starry-sky appearance
  • 34. Undifferentiated, anaplastic , large lymphoid cells contain ovoid vesicular basophilic nuclei and prominent nucleoli . Histiocytes contain abundant , almost clear cytoplasm and phagocytosed cellular debris
  • 35. Malignant melanoma : a pigmented conjunctival lesion near the limbus , present since childhood , had undergone recent rapid growth
  • 38. Immunohistochemistry -IHC ■ Positive stains : ■ S100(nuclear and cytoplasmic ) ■ SOX10(nuclear) ■ MelanA /MART1( Cytoplasmic) ■ HMB45(Cytoplasmic) ■ Tyrosinase (cytoplasmic ) ■ MiTF(nuclear)
  • 39. Retinoblastoma ■ One of the most common childhood malignancies ■ The most common childhood intraocular neoplasm ■ 20% of the world’s retinoblastoma patients live in india , which has 1500 new cases annually , more cases than any other country
  • 40. Characteristically, sections of retinoblastoma stained with hematoxylin and eosin and viewed under low magnification show dark blue areas surrounded by light pink areas . The dark areas represent viable cells and calcium deposition , whereas the light areas represent tumour necrosis
  • 41. Increased magnification shows viable (dark blue) tumor cells clustered around central blood vessels and surrounded in turn by a mantle of necrotic cells (pink).Numerous Flexner-wintersteiner rosettes are present
  • 42. Types of rosettes : A Flexner- wintersteiner rosette consists of a central lumen lined by cuboidal tumour cells that contain nuclei positioned basally (away from the lumen )
  • 43. Homer Wright rosettes are found more frequently in medulloblastomas and neuroblastomas than in retinoblastomas. In these rosettes , the cells line up around an acellular area that contains cobweb- like material
  • 44. Fleurettes are flower like groupings of tumor cells in the retinoblastoma . The cells of fleurettes show clear evidence of differentiation into photoreceptor elements
  • 45. Immunohistochemistry - IHC ■ POSITIVE STAINS ■ SOX2 ■ MAP2 ■ Neuron specific enolase ■ Synatophysin ■ Retinoblastoma associated protein
  • 46. Papilloma with dysplasia : clinical appearance of typical limbal sessile conjunctival papilloma
  • 47. Histologic section shows a sudden and abrupt transition from the normal conjunctival to a markedly thickened epithelium. The lesion is broad based and shows numerous blood vessels penetrating into the thickened epithelium
  • 48. Squamous cell carcinoma : the patient had a vascularised elevated pearly lesion at the temporal limbus in the right eye
  • 49. Histologic section shows full thickness atypia and loss of polarity . A diagnosis of carcinoma in situ would be made here
  • 50. Malignant epithelial cells in the substantia propria of the conjunctiva ,forming keratin pearls in some areas representing invasive squamous cell carcinoma
  • 51. Pigmented squamous cell carcinoma : clinical photograph of elevated, pigmented temporal conjunctival lesion
  • 52. Pigmented dendritic melanocyte among atypical squamous cells
  • 53. Immunohistochemistry -IHC ■ Positive stains : ■ P53: increased immunoreactivity in dyplasia ■ Ki67: increased proliferation rates in suprabasal epithelium in dysplasia ■ P16: greater intensity and percentage staining in high grade dysplasia
  • 54. Sebaceous gland carcinoma: upper lid lesion resembles a chalazion . Note loss of cilia in area of lesion
  • 55. Excisional biopsy shows large tumour nodules in the dermis, most of which exhibit central necrosis
  • 56. Increased magnification shows numerous cells resembling sebaceous cells. A number of mitotic figures are present
  • 57. Oil red-O fat stain shows marked positivity in the cytoplasm of abnormal cells
  • 58. Large tumour cells are scattered throughout the surface epidermis , simulating paget’s disease(i.e.pagetoid change). The cancerous invasion of the epithelium can cause a chronic blepharoconjunctivitis (masquerade syndrome)
  • 59. Immunohistochemistry –IHC ■ Positive stains ■ Androgen receptor ■ Keratin ■ EMA ■ LeUM1 ■ KI67-increased relative to sebaceous adenoma and sebaceoma ■ P53-increased relative to sebaceous adenoma and sebaceoma ■ BerEP4