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Appendix 1 STAGING OF CONJUNCTIVAL CARCINOMA, 8TH ED. AJCC CANCER STAGING MANUAL, 2017a
| Definition of Primary Tumor (T) | |
| TX | Primary tumor cannot be assessed |
| T0 | No evidence of primary tumor |
| Tis | Carcinoma in situ |
| T1 | Tumor ≤5 mm in greatest dimension, which invades through basement membrane without invasion of adjacent structuresb |
| T2 | Tumor ≥5 mm in greatest dimension, which invades through basement membrane without invasion of adjacent structures |
| T3 | Tumor invades adjacent structures excluding the orbit |
| T4 | Tumor invades the orbit with or without further extension |
| T4a | Tumor invades orbital soft tissues, without bone invasion |
| T4b | Tumor invades bone |
| T4c | Tumor invades adjacent paranasal sinuses |
| T4d | Tumor invades brain |
| Definition of Regional Lymph Nodes (N) | |
| NX | Regional lymph nodes cannot be assessed |
| N0 | No regional lymph node metastasis |
| N1 | Regional lymph node metastasis |
| Definition of Distant Metastasis (M) | |
| M0 | No distant metastasis |
| M1 | Distant metastasis |
| Definition of Histopathologic Grade (G) | |
| GX | Grade cannot be assessed |
| G1 | Well-differentiated |
| G2 | Moderately differentiated |
| G3 | Poorly differentiated |
| G4 | Undifferentiated |
| Definition of Primary Tumor (T) | |
| TX | Primary tumor cannot be assessed |
| T0 | No evidence of primary tumor |
| Tis | Carcinoma in situ |
| T1 | Tumor ≤5 mm in greatest dimension, which invades through basement membrane without invasion of adjacent structuresb |
| T2 | Tumor ≥5 mm in greatest dimension, which invades through basement membrane without invasion of adjacent structures |
| T3 | Tumor invades adjacent structures excluding the orbit |
| T4 | Tumor invades the orbit with or without further extension |
| T4a | Tumor invades orbital soft tissues, without bone invasion |
| T4b | Tumor invades bone |
| T4c | Tumor invades adjacent paranasal sinuses |
| T4d | Tumor invades brain |
| Definition of Regional Lymph Nodes (N) | |
| NX | Regional lymph nodes cannot be assessed |
| N0 | No regional lymph node metastasis |
| N1 | Regional lymph node metastasis |
| Definition of Distant Metastasis (M) | |
| M0 | No distant metastasis |
| M1 | Distant metastasis |
| Definition of Histopathologic Grade (G) | |
| GX | Grade cannot be assessed |
| G1 | Well-differentiated |
| G2 | Moderately differentiated |
| G3 | Poorly differentiated |
| G4 | Undifferentiated |
a
Data from reference 1.
b
Adjacent structures include cornea, forniceal conjunctiva, palpebral conjunctiva, tarsal conjunctiva, lacrimal punctum and canaliculi, plica, caruncle, anterior and posterior eyelid lamellae, eyelid margin, and intraocular compartments.
Appendix 2 STAGING OF CONJUNCTIVAL MALIGNANT MELANOMA, 8TH ED. AJCC CANCER STAGING MANUAL, 2017a
| Definition of Primary Clinical Tumor (cT) | |
| TX | Primary tumor cannot be assessed |
| T0 | No evidence of primary tumor |
| T1 | Tumor of the bulbar conjunctiva |
| T1a | Tumor of bulbar conjunctiva involving <1 quadrant |
| T1b | Tumor of bulbar conjunctiva involving >1 but <2 quadrants |
| T1c | Tumor of bulbar conjunctiva involving >2 but <3 quadrants |
| T1d | Tumor of bulbar conjunctiva involving >3 quadrants |
| T2 | Tumor of nonbulbar (forniceal, palpebral, tarsal) conjunctiva and the caruncle |
| T2a | Noncaruncular tumor involving <1 quadrant of the nonbulbar conjunctiva |
| T2b | Noncaruncular tumor involving <1 quadrant of the nonbulbar conjunctiva |
| T2c | Caruncular tumor involving <1 quadrant of the nonbulbar conjunctiva |
| T2d | Caruncular tumor involving >1 quadrant of the nonbulbar conjunctiva |
| T3 | Tumor of any size with local invasion |
| T3a | Tumor of any size invading the globe |
| T3b | Tumor of any size invading the eyelid |
| T3c | Tumor of any size invading the orbit |
| T3d | Tumor of any size invading the nasolacrimal duct, lacrimal sac, or paranasal sinuses |
| T4 | Tumor of any size with invasion of the central nervous system |
| Definition of Primary Pathological Tumor (pT) | |
| TX | Primary tumor cannot be assessed |
| T0 | No evidence of primary tumor |
| Tis | Tumor confined to the conjunctival epithelium |
| T1 | Tumor of bulbar conjunctiva with the invasion of substantia propria |
| T1a | Tumor of bulbar conjunctiva with the invasion of substantia propria, <2.0 mm in thickness |
| T1b | Tumor of bulbar conjunctiva with the invasion of substantia propria, >2.0 mm in thickness |
| T2 | Tumor of nonbulbar conjunctiva with the invasion of substantia propria |
| T2a | Tumor of nonbulbar conjunctiva with the invasion of substantia propria |
| T2b | Tumor of nonbulbar conjunctiva with the invasion of substantia propria, <2.0 mm in thickness |
| T3 | Tumor of nonbulbar conjunctiva with the invasion of the substantia propria, >2.0 mm in thickness |
| T3a | Tumor of any size with local invasion |
| T3b | Tumor of any size invading the globe |
| T3c | Tumor of any size invading the eyelid |
| T3d | Tumor of any size invading the orbit |
| T4 | Tumor of any size invading the nasolacrimal duct, lacrimal sac, or paranasal sinuses Tumor of any size with invasion of the central nervous system |
| Regional Lymph Nodes (N) | |
| NX | Regional lymph nodes cannot be assessed |
| N0 | No regional lymph node metastasis |
| N1 | Regional lymph node metastasis |
| Distant Metastasis (M) | |
| M0 | No distant metastasis |
| M1 | Distant metastasis |
| Definition of Primary Clinical Tumor (cT) | |
| TX | Primary tumor cannot be assessed |
| T0 | No evidence of primary tumor |
| T1 | Tumor of the bulbar conjunctiva |
| T1a | Tumor of bulbar conjunctiva involving <1 quadrant |
| T1b | Tumor of bulbar conjunctiva involving >1 but <2 quadrants |
| T1c | Tumor of bulbar conjunctiva involving >2 but <3 quadrants |
| T1d | Tumor of bulbar conjunctiva involving >3 quadrants |
| T2 | Tumor of nonbulbar (forniceal, palpebral, tarsal) conjunctiva and the caruncle |
| T2a | Noncaruncular tumor involving <1 quadrant of the nonbulbar conjunctiva |
| T2b | Noncaruncular tumor involving <1 quadrant of the nonbulbar conjunctiva |
| T2c | Caruncular tumor involving <1 quadrant of the nonbulbar conjunctiva |
| T2d | Caruncular tumor involving >1 quadrant of the nonbulbar conjunctiva |
| T3 | Tumor of any size with local invasion |
| T3a | Tumor of any size invading the globe |
| T3b | Tumor of any size invading the eyelid |
| T3c | Tumor of any size invading the orbit |
| T3d | Tumor of any size invading the nasolacrimal duct, lacrimal sac, or paranasal sinuses |
| T4 | Tumor of any size with invasion of the central nervous system |
| Definition of Primary Pathological Tumor (pT) | |
| TX | Primary tumor cannot be assessed |
| T0 | No evidence of primary tumor |
| Tis | Tumor confined to the conjunctival epithelium |
| T1 | Tumor of bulbar conjunctiva with the invasion of substantia propria |
| T1a | Tumor of bulbar conjunctiva with the invasion of substantia propria, <2.0 mm in thickness |
| T1b | Tumor of bulbar conjunctiva with the invasion of substantia propria, >2.0 mm in thickness |
| T2 | Tumor of nonbulbar conjunctiva with the invasion of substantia propria |
| T2a | Tumor of nonbulbar conjunctiva with the invasion of substantia propria |
| T2b | Tumor of nonbulbar conjunctiva with the invasion of substantia propria, <2.0 mm in thickness |
| T3 | Tumor of nonbulbar conjunctiva with the invasion of the substantia propria, >2.0 mm in thickness |
| T3a | Tumor of any size with local invasion |
| T3b | Tumor of any size invading the globe |
| T3c | Tumor of any size invading the eyelid |
| T3d | Tumor of any size invading the orbit |
| T4 | Tumor of any size invading the nasolacrimal duct, lacrimal sac, or paranasal sinuses Tumor of any size with invasion of the central nervous system |
| Regional Lymph Nodes (N) | |
| NX | Regional lymph nodes cannot be assessed |
| N0 | No regional lymph node metastasis |
| N1 | Regional lymph node metastasis |
| Distant Metastasis (M) | |
| M0 | No distant metastasis |
| M1 | Distant metastasis |
a
Data from reference 1.
Appendix 3 STAGING OF EYELID CARCINOMA, 8TH ED. AJCC CANCER STAGING MANUAL, 2017a
| Definition of Primary Tumor (T) | |
| TX | Primary tumor cannot be assessed |
| T0 | No evidence of primary tumor |
| Tis | Carcinoma in situ |
| T1 | Tumor ≤10 mm in greatest dimension |
| T1a | Tumor does not invade the tarsal plate or the eyelid margin |
| T1b | Tumor invades the tarsal plate or the eyelid margin |
| T1c | Tumor involves full-thickness of the eyelid |
| T2 | Tumor >10 mm but ≤20 mm in greatest dimension |
| T2a | Tumor does not invade the tarsal plate or the eyelid margin |
| T2b | Tumor invades the tarsal plate or the eyelid margin |
| T2c | Tumor involves full-thickness of the eyelid |
| T3 | Tumor >10 mm but ≤20 mm in greatest dimension |
| T3a | Tumor does not invade the tarsal plate or the eyelid margin |
| T3b | Tumor invades the tarsal plate or the eyelid margin |
| T3c | Tumor involves full-thickness of the eyelid |
| T4 | Any eyelid tumor that invades adjacent ocular, orbital, or facial structures |
| T4a | Tumor invades ocular or intraorbital structures |
| T4b | Tumor invades (or erodes through) the bony walls of the orbit or extends to the paranasal sinuses or invades the lacrimal sac/nasolacrimal duct or brain |
| Definition of Regional Lymph Nodes (N) | |
| NX | Regional lymph nodes cannot be assessed |
| N0 | No regional lymph node metastasis |
| N1 | Metastasis in a single ipsilateral lymph node based on clinical evaluation or imaging |
| N1a | Metastasis in a single ipsilateral lymph node based on lymph node biopsy |
| N1b | Metastasis in a single ipsilateral lymph node based on lymph node biopsy |
| N2 | Metastases in a single ipsilateral lymph node, >3 cm in greatest dimension, or in bilateral or contralateral lymph nodes |
| N2a | Metastasis in a single ipsilateral lymph node based on lymph node biopsy |
| N2b | Metastasis in a single ipsilateral lymph node based on lymph node biopsy |
| Definition of Distant Metastasis (M) | |
| M0 | No distant metastasis |
| M1 | Distant metastasis |
| Definition of Histopathologic Grade (G) (Applies predominantly to SCCb and sebaceous carcinoma; not used for BCC) | |
| GX | Grade cannot be assessed |
| G1 | Well-differentiated |
| G2 | Moderately differentiated |
| G3 | Poorly differentiated |
| G4 | Undifferentiated |
| Definition of Primary Tumor (T) | |
| TX | Primary tumor cannot be assessed |
| T0 | No evidence of primary tumor |
| Tis | Carcinoma in situ |
| T1 | Tumor ≤10 mm in greatest dimension |
| T1a | Tumor does not invade the tarsal plate or the eyelid margin |
| T1b | Tumor invades the tarsal plate or the eyelid margin |
| T1c | Tumor involves full-thickness of the eyelid |
| T2 | Tumor >10 mm but ≤20 mm in greatest dimension |
| T2a | Tumor does not invade the tarsal plate or the eyelid margin |
| T2b | Tumor invades the tarsal plate or the eyelid margin |
| T2c | Tumor involves full-thickness of the eyelid |
| T3 | Tumor >10 mm but ≤20 mm in greatest dimension |
| T3a | Tumor does not invade the tarsal plate or the eyelid margin |
| T3b | Tumor invades the tarsal plate or the eyelid margin |
| T3c | Tumor involves full-thickness of the eyelid |
| T4 | Any eyelid tumor that invades adjacent ocular, orbital, or facial structures |
| T4a | Tumor invades ocular or intraorbital structures |
| T4b | Tumor invades (or erodes through) the bony walls of the orbit or extends to the paranasal sinuses or invades the lacrimal sac/nasolacrimal duct or brain |
| Definition of Regional Lymph Nodes (N) | |
| NX | Regional lymph nodes cannot be assessed |
| N0 | No regional lymph node metastasis |
| N1 | Metastasis in a single ipsilateral lymph node based on clinical evaluation or imaging |
| N1a | Metastasis in a single ipsilateral lymph node based on lymph node biopsy |
| N1b | Metastasis in a single ipsilateral lymph node based on lymph node biopsy |
| N2 | Metastases in a single ipsilateral lymph node, >3 cm in greatest dimension, or in bilateral or contralateral lymph nodes |
| N2a | Metastasis in a single ipsilateral lymph node based on lymph node biopsy |
| N2b | Metastasis in a single ipsilateral lymph node based on lymph node biopsy |
| Definition of Distant Metastasis (M) | |
| M0 | No distant metastasis |
| M1 | Distant metastasis |
| Definition of Histopathologic Grade (G) (Applies predominantly to SCCb and sebaceous carcinoma; not used for BCC) | |
| GX | Grade cannot be assessed |
| G1 | Well-differentiated |
| G2 | Moderately differentiated |
| G3 | Poorly differentiated |
| G4 | Undifferentiated |
a
Data from reference 1.
b
SCC= squamous cell carcinoma; BCC = basal cell carcinoma.
Appendix 4 STAGING OF ORBITAL SARCOMA, 8TH ED. AJCC CANCER STAGING MANUAL, 2017a
| Definition of Primary Tumor (T) | |
| TX | Primary tumor cannot be assessed |
| T0 | No evidence of primary tumor |
| T1 | Tumor ≤2 cm in greatest dimension |
| T2 | Tumor >2 cm in greatest diameter without invasion of the bony walls or globe |
| T3 | Tumor of any size with invasion of bony walls |
| T4 | Tumor of any size with invasion of the globe or periorbital structures, including the eyelid, conjunctiva, temporal fossa, nasal cavity, paranasal sinuses, and/or central nervous system |
| Definition of Regional Lymph Nodes (N) | |
| NX | Regional lymph nodes cannot be assessed |
| N0 | No regional lymph node metastasis |
| N1 | Regional lymph node metastasis |
| Definition of Distant Metastasis (M) | |
| M0 | No distant metastasis |
| M1 | Distant metastasis |
| Definition of Histopathologic Grade (G) | |
| French Federation of Cancer Centers Sarcoma Group (FNCLCC) histologic grading system is adopted for grading of primary orbital sarcomas | |
| Definition of Primary Tumor (T) | |
| TX | Primary tumor cannot be assessed |
| T0 | No evidence of primary tumor |
| T1 | Tumor ≤2 cm in greatest dimension |
| T2 | Tumor >2 cm in greatest diameter without invasion of the bony walls or globe |
| T3 | Tumor of any size with invasion of bony walls |
| T4 | Tumor of any size with invasion of the globe or periorbital structures, including the eyelid, conjunctiva, temporal fossa, nasal cavity, paranasal sinuses, and/or central nervous system |
| Definition of Regional Lymph Nodes (N) | |
| NX | Regional lymph nodes cannot be assessed |
| N0 | No regional lymph node metastasis |
| N1 | Regional lymph node metastasis |
| Definition of Distant Metastasis (M) | |
| M0 | No distant metastasis |
| M1 | Distant metastasis |
| Definition of Histopathologic Grade (G) | |
| French Federation of Cancer Centers Sarcoma Group (FNCLCC) histologic grading system is adopted for grading of primary orbital sarcomas | |
a
Data from reference 1.
Appendix 5 STAGING OF LACRIMAL GLAND TUMORS, 8TH ED. AJCC CANCER STAGING MANUAL, 2017a
| Primary Tumor (T) | |
| TX | Primary tumor cannot be assessed |
| T0 | No evidence of primary tumor |
| T1 | Tumor 2 cm or smaller in greatest dimension, with or without extraglandular extension into the orbital soft tissue |
| T1a No periosteal or bone involvement | |
| T1b Periosteal involvement only | |
| T1c Periosteal and bone involvement | |
| T2 | Tumor larger than 2 cm but no larger than 4 cm in greatest dimension |
| T2a | No periosteal or bone involvement |
| T2b | Periosteal involvement only |
| T2c | Periosteal and bone involvement |
| T3 | Tumor larger than 4 cm in greatest dimension |
| T3a | No periosteal or bone involvement |
| T3b | Periosteal involvement only |
| T3c | Periosteal and bone involvement |
| T4 | Involvement of adjacent structures, including sinuses, temporal fossa, pterygoid fossa, superior orbital tissue, cavernous sinus, or brain |
| T4a | Tumor ≤2 cm in greatest dimension |
| T4b | Tumor >2 cm and ≤4 cm in greatest dimension |
| T4c | Tumor >4 cm in greatest dimension |
| Regional Lymph Nodes (N) | |
| NX | Regional lymph nodes cannot be assessed |
| N0 | No regional lymph node metastasis |
| N1 | Regional lymph node metastasis |
| Distant Metastasis (M) | |
| M0 | No distant metastasis |
| M1 | Distant metastasis |
| Primary Tumor (T) | |
| TX | Primary tumor cannot be assessed |
| T0 | No evidence of primary tumor |
| T1 | Tumor 2 cm or smaller in greatest dimension, with or without extraglandular extension into the orbital soft tissue |
| T1a No periosteal or bone involvement | |
| T1b Periosteal involvement only | |
| T1c Periosteal and bone involvement | |
| T2 | Tumor larger than 2 cm but no larger than 4 cm in greatest dimension |
| T2a | No periosteal or bone involvement |
| T2b | Periosteal involvement only |
| T2c | Periosteal and bone involvement |
| T3 | Tumor larger than 4 cm in greatest dimension |
| T3a | No periosteal or bone involvement |
| T3b | Periosteal involvement only |
| T3c | Periosteal and bone involvement |
| T4 | Involvement of adjacent structures, including sinuses, temporal fossa, pterygoid fossa, superior orbital tissue, cavernous sinus, or brain |
| T4a | Tumor ≤2 cm in greatest dimension |
| T4b | Tumor >2 cm and ≤4 cm in greatest dimension |
| T4c | Tumor >4 cm in greatest dimension |
| Regional Lymph Nodes (N) | |
| NX | Regional lymph nodes cannot be assessed |
| N0 | No regional lymph node metastasis |
| N1 | Regional lymph node metastasis |
| Distant Metastasis (M) | |
| M0 | No distant metastasis |
| M1 | Distant metastasis |
a
Data from reference 1.
Appendix 6 STAGING OF OCULAR ADNEXAL LYMPHOMAS, 8TH ED. AJCC CANCER STAGING MANUAL, 2017a
| Definition of Primary Tumor (T) | |
| TX | Lymphoma extent not specified |
| T0 | No evidence of lymphoma |
| T1 | Lymphoma involving the conjunctiva alone without eyelid or orbital involvement |
| T2 | Lymphoma with orbital involvement with or without conjunctival involvement |
| T3 | Lymphoma with preseptal eyelid involvement with or without orbital involvement and with or without conjunctival involvement |
| T4 | Orbital adnexal lymphoma and extraorbital lymphoma extending beyond the orbit to adjacent structures, such as bone, maxillofacial sinuses, and brain |
| Definition of Regional Lymph Nodes (N) | |
| NX | Involvement of lymph nodes not assessed |
| N0 | No evidence of lymph node involvement |
| N1 | Involvement of lymph node region or regions draining the ocular adnexal structures and superior to the mediastinum (preauricular, parotid, submandibular, and cervical nodes) |
| N1a | Involvement of a single lymph node region superior to the mediastinum |
| N1b | Involvement of two or more lymph node regions, superior to the mediastinum |
| N2 | Involvement of lymph node regions of the mediastinum |
| N3 | Diffuse or disseminated involvement of peripheral and central lymph node regions |
| Definition of Distant Metastasis (M) | |
| M0 | No evidence of involvement of other extranodal sites |
| M1a | Noncontiguous involvement of tissue or organs external to the ocular adnexa (e.g., parotid glands, submandibular gland, lung, liver, spleen, kidney, breast) |
| M1b | Lymphomatous involvement of the bone marrow |
| M1c | Both M1a and M1b involvement |
| Definition of Histopathologic Grade (G) | |
| A grade is assigned only to follicular lymphomas, as described by 2017 WHO classificationb | |
| Definition of Primary Tumor (T) | |
| TX | Lymphoma extent not specified |
| T0 | No evidence of lymphoma |
| T1 | Lymphoma involving the conjunctiva alone without eyelid or orbital involvement |
| T2 | Lymphoma with orbital involvement with or without conjunctival involvement |
| T3 | Lymphoma with preseptal eyelid involvement with or without orbital involvement and with or without conjunctival involvement |
| T4 | Orbital adnexal lymphoma and extraorbital lymphoma extending beyond the orbit to adjacent structures, such as bone, maxillofacial sinuses, and brain |
| Definition of Regional Lymph Nodes (N) | |
| NX | Involvement of lymph nodes not assessed |
| N0 | No evidence of lymph node involvement |
| N1 | Involvement of lymph node region or regions draining the ocular adnexal structures and superior to the mediastinum (preauricular, parotid, submandibular, and cervical nodes) |
| N1a | Involvement of a single lymph node region superior to the mediastinum |
| N1b | Involvement of two or more lymph node regions, superior to the mediastinum |
| N2 | Involvement of lymph node regions of the mediastinum |
| N3 | Diffuse or disseminated involvement of peripheral and central lymph node regions |
| Definition of Distant Metastasis (M) | |
| M0 | No evidence of involvement of other extranodal sites |
| M1a | Noncontiguous involvement of tissue or organs external to the ocular adnexa (e.g., parotid glands, submandibular gland, lung, liver, spleen, kidney, breast) |
| M1b | Lymphomatous involvement of the bone marrow |
| M1c | Both M1a and M1b involvement |
| Definition of Histopathologic Grade (G) | |
| A grade is assigned only to follicular lymphomas, as described by 2017 WHO classificationb | |
Appendix 7 DEFINITION OF PRIMARY UVEAL MELANOMA
| ✔ | T Category | T Criteria |
|---|---|---|
| TX | Primary tumor cannot be assessed | |
| T0 | No evidence of primary tumor | |
| T1 | Tumor limited to the iris | |
| T1a | Tumor limited to the iris, not more than 3 clock hours in size | |
| T1b | Tumor limited to the iris, more than 3 clock hours in size | |
| T1c | Tumor limited to the iris with secondary glaucoma | |
| T2 | Tumor confluent with or extending into the ciliary body, choroid, or both | |
| T2a | Tumor confluent with or extending into the ciliary body, without secondary glaucoma | |
| T2b | Tumor confluent with or extending into the ciliary body and choroid, without secondary glaucoma | |
| T2c | Tumor confluent with or extending into the ciliary body, choroid, or both, with secondary glaucoma | |
| T3 | Tumor confluent with or extending into the ciliary body, choroid, or both, with scleral extension | |
| T4 | Tumor with extrascleral extension | |
| T4a | Tumor with extrascleral extension ≤5 mm in largest diameter | |
| T4b | Tumor with extrascleral extension >5 mm in largest diameter | |
| Note: Iris melanomas originate from, and are predominantly located in, this region of the uvea. If less than half the tumor volume is located within the iris, the tumor may have originated in the ciliary body, and consideration should be given to classifying it accordingly. | ||
| ✔ | T Suffix | Definition |
| (m) | Select if synchronous primary tumors are found in single organ. | |
| ✔ | T Category | T Criteria |
|---|---|---|
| TX | Primary tumor cannot be assessed | |
| T0 | No evidence of primary tumor | |
| T1 | Tumor limited to the iris | |
| T1a | Tumor limited to the iris, not more than 3 clock hours in size | |
| T1b | Tumor limited to the iris, more than 3 clock hours in size | |
| T1c | Tumor limited to the iris with secondary glaucoma | |
| T2 | Tumor confluent with or extending into the ciliary body, choroid, or both | |
| T2a | Tumor confluent with or extending into the ciliary body, without secondary glaucoma | |
| T2b | Tumor confluent with or extending into the ciliary body and choroid, without secondary glaucoma | |
| T2c | Tumor confluent with or extending into the ciliary body, choroid, or both, with secondary glaucoma | |
| T3 | Tumor confluent with or extending into the ciliary body, choroid, or both, with scleral extension | |
| T4 | Tumor with extrascleral extension | |
| T4a | Tumor with extrascleral extension ≤5 mm in largest diameter | |
| T4b | Tumor with extrascleral extension >5 mm in largest diameter | |
| Note: Iris melanomas originate from, and are predominantly located in, this region of the uvea. If less than half the tumor volume is located within the iris, the tumor may have originated in the ciliary body, and consideration should be given to classifying it accordingly. | ||
| ✔ | T Suffix | Definition |
| (m) | Select if synchronous primary tumors are found in single organ. | |
Figure 67.1:
Classification of ciliary body and choroid uveal melanoma based on thickness and diameter
Figure 67.1:
Classification of ciliary body and choroid uveal melanoma based on thickness and diameter
| ✔ | T Category | T Criteria |
|---|---|---|
| TX | Primary tumor cannot be assessed | |
| T0 | No evidence of primary tumor | |
| T1 | Tumor size category 1 | |
| T1a | Tumor size category 1 without ciliary body involvement and extraocular extension | |
| T1b | Tumor size category 1 with ciliary body involvement | |
| T1c | Tumor size category 1 without ciliary body involvement but with extraocular extension ≤5 mm in largest diameter | |
| T1d | Tumor size category 1 with ciliary body involvement and extraocular extension ≤5 mm in largest diameter | |
| T2 | Tumor size category 2 | |
| T2a | Tumor size category 2 without ciliary body involvement and extraocular extension | |
| T2b | Tumor size category 2 with ciliary body involvement | |
| T2c | Tumor size category 2 without ciliary body involvement but with extraocular extension ≤5 mm in largest diameter | |
| T2d | Tumor size category 2 with ciliary body involvement and extraocular extension ≤5 mm in largest diameter | |
| T3 | Tumor size category 3 | |
| T3a | Tumor size category 3 without ciliary body involvement and extraocular extension | |
| T3b | Tumor size category 3 with ciliary body involvement | |
| T3c | Tumor size category 3 without ciliary body involvement but with extraocular extension ≤5 mm in largest diameter | |
| T3d | Tumor size category 3 with ciliary body involvement and extraocular extension ≤5 mm in largest diameter | |
| T4 | Tumor size category 4 | |
| T4a | Tumor size category 4 without ciliary body involvement and extraocular extension | |
| T4b | Tumor size category 4 with ciliary body involvement | |
| T4c | Tumor size category 4 without ciliary body involvement but with extraocular extension ≤5 mm in largest diameter | |
| T4d | Tumor size category 4 with ciliary body involvement and extraocular extension ≤5 mm in largest diameter | |
| T4e | Any tumor size category with extraocular extension >5 mm in largest diameter | |
| Notes: | 1. Primary ciliary body and choroidal melanomas are classified according to the four tumor size categories defined in Figure 67.1. | |
| 2. In clinical practice, the largest tumor basal diameter may be estimated in optic disc diameters (DD; average: 1 DD = 1.5 mm), and tumor thickness may be estimated in diopters (average: 2.5 diopters = 1 mm). Ultrasonography and fundus photography are used to provide more accurate measurements. | ||
| 3. When histopathologic measurements are recorded after fixation, tumor diameter and thickness may be underestimated because of tissue shrinkage. | ||
| ✔ | T Suffix | Definition |
| (m) | Select if synchronous primary tumors are found in single organ. | |
| ✔ | T Category | T Criteria |
|---|---|---|
| TX | Primary tumor cannot be assessed | |
| T0 | No evidence of primary tumor | |
| T1 | Tumor size category 1 | |
| T1a | Tumor size category 1 without ciliary body involvement and extraocular extension | |
| T1b | Tumor size category 1 with ciliary body involvement | |
| T1c | Tumor size category 1 without ciliary body involvement but with extraocular extension ≤5 mm in largest diameter | |
| T1d | Tumor size category 1 with ciliary body involvement and extraocular extension ≤5 mm in largest diameter | |
| T2 | Tumor size category 2 | |
| T2a | Tumor size category 2 without ciliary body involvement and extraocular extension | |
| T2b | Tumor size category 2 with ciliary body involvement | |
| T2c | Tumor size category 2 without ciliary body involvement but with extraocular extension ≤5 mm in largest diameter | |
| T2d | Tumor size category 2 with ciliary body involvement and extraocular extension ≤5 mm in largest diameter | |
| T3 | Tumor size category 3 | |
| T3a | Tumor size category 3 without ciliary body involvement and extraocular extension | |
| T3b | Tumor size category 3 with ciliary body involvement | |
| T3c | Tumor size category 3 without ciliary body involvement but with extraocular extension ≤5 mm in largest diameter | |
| T3d | Tumor size category 3 with ciliary body involvement and extraocular extension ≤5 mm in largest diameter | |
| T4 | Tumor size category 4 | |
| T4a | Tumor size category 4 without ciliary body involvement and extraocular extension | |
| T4b | Tumor size category 4 with ciliary body involvement | |
| T4c | Tumor size category 4 without ciliary body involvement but with extraocular extension ≤5 mm in largest diameter | |
| T4d | Tumor size category 4 with ciliary body involvement and extraocular extension ≤5 mm in largest diameter | |
| T4e | Any tumor size category with extraocular extension >5 mm in largest diameter | |
| Notes: | 1. Primary ciliary body and choroidal melanomas are classified according to the four tumor size categories defined in Figure 67.1. | |
| 2. In clinical practice, the largest tumor basal diameter may be estimated in optic disc diameters (DD; average: 1 DD = 1.5 mm), and tumor thickness may be estimated in diopters (average: 2.5 diopters = 1 mm). Ultrasonography and fundus photography are used to provide more accurate measurements. | ||
| 3. When histopathologic measurements are recorded after fixation, tumor diameter and thickness may be underestimated because of tissue shrinkage. | ||
| ✔ | T Suffix | Definition |
| (m) | Select if synchronous primary tumors are found in single organ. | |
DEFINITION OF REGIONAL LYMPH NODE (N)
| ✔ | N Category | N Criteria |
|---|---|---|
| NX | Regional lymph nodes cannot be assessed | |
| N0 | No regional lymph node involvement | |
| N1 | Regional lymph node metastases or discrete tumor deposits in the orbit | |
| N1a | Metastasis in one or more regional lymph node(s) | |
| N1b | No regional lymph nodes are positive, but there are discrete tumor deposits in the orbit that are not contiguous to the eye. (choroidal and ciliary body melanoma only) | |
| ✔ | N Suffix | Definition |
| (sn) | Select if regional lymph node metastasis identified by SLN biopsy only. | |
| (f) Select if regional lymph node metastasis identified by FNA or core needle biopsy only. | ||
| ✔ | N Category | N Criteria |
|---|---|---|
| NX | Regional lymph nodes cannot be assessed | |
| N0 | No regional lymph node involvement | |
| N1 | Regional lymph node metastases or discrete tumor deposits in the orbit | |
| N1a | Metastasis in one or more regional lymph node(s) | |
| N1b | No regional lymph nodes are positive, but there are discrete tumor deposits in the orbit that are not contiguous to the eye. (choroidal and ciliary body melanoma only) | |
| ✔ | N Suffix | Definition |
| (sn) | Select if regional lymph node metastasis identified by SLN biopsy only. | |
| (f) Select if regional lymph node metastasis identified by FNA or core needle biopsy only. | ||
DEFINITION OF DISTANT METASTASIS (M)
The terms pM0 and MX are NOT valid categories in the TNM system. Assignment of the M category for clinical classification may be cM0, cM1, or pM1. Any of the M categories (cM0, cM1, or pM1) may be used with pathological stage grouping.
| ✔ | M Category | M Criteria |
|---|---|---|
| cM0 | No distant metastasis by clinical classification | |
| cM1 | Distant metastasis | |
| cM1a | Largest diameter of the largest metastasis ≤3.0 cm | |
| cM1b | Largest diameter of the largest metastasis 3.1-8.0 cm | |
| cM1c | Largest diameter of the largest metastasis >8.1 cm | |
| pM1 | Distant metastasis, microscopically confirmed | |
| pM1a | Largest diameter of the largest metastasis <3.0 cm, microscopically confirmed | |
| pM1b | Largest diameter of the largest metastasis 3.1-8.0 cm, microscopically confirmed | |
| pM1c | Largest diameter of the largest metastasis ≥8.1 cm, microscopically confirmed |
| ✔ | M Category | M Criteria |
|---|---|---|
| cM0 | No distant metastasis by clinical classification | |
| cM1 | Distant metastasis | |
| cM1a | Largest diameter of the largest metastasis ≤3.0 cm | |
| cM1b | Largest diameter of the largest metastasis 3.1-8.0 cm | |
| cM1c | Largest diameter of the largest metastasis >8.1 cm | |
| pM1 | Distant metastasis, microscopically confirmed | |
| pM1a | Largest diameter of the largest metastasis <3.0 cm, microscopically confirmed | |
| pM1b | Largest diameter of the largest metastasis 3.1-8.0 cm, microscopically confirmed | |
| pM1c | Largest diameter of the largest metastasis ≥8.1 cm, microscopically confirmed |
AJCC PROGNOSTIC STAGE GROUPS
Always refer to the specific chapter for rules on clinical and pathological classification of this disease.
| ✔ | When T is... | And N is... | And M is... | Then the stage group is... |
|---|---|---|---|---|
| T1a | N0 | M0 | I | |
| T1b-d | N0 | M0 | IIA | |
| T2a | N0 | M0 | IIA | |
| T2b | N0 | M0 | IIB | |
| T3a | N0 | M0 | IIB | |
| T2c-d | N0 | M0 | IIIA | |
| T3b-c | N0 | M0 | IIIA | |
| T4a | N0 | M0 | IIIA | |
| T3d | N0 | M0 | IIIB | |
| T4b-c | N0 | M0 | IIIB | |
| T4d-e | N0 | M0 | IIIC | |
| Any T | N1 | M0 | IV | |
| Any T | Any N | M1a-c | IV |
| ✔ | When T is... | And N is... | And M is... | Then the stage group is... |
|---|---|---|---|---|
| T1a | N0 | M0 | I | |
| T1b-d | N0 | M0 | IIA | |
| T2a | N0 | M0 | IIA | |
| T2b | N0 | M0 | IIB | |
| T3a | N0 | M0 | IIB | |
| T2c-d | N0 | M0 | IIIA | |
| T3b-c | N0 | M0 | IIIA | |
| T4a | N0 | M0 | IIIA | |
| T3d | N0 | M0 | IIIB | |
| T4b-c | N0 | M0 | IIIB | |
| T4d-e | N0 | M0 | IIIC | |
| Any T | N1 | M0 | IV | |
| Any T | Any N | M1a-c | IV |
REGISTRY DATA COLLECTION VARIABLES
| 1. | Tumor site (ICD code lacks specificity): □ ciliary body (use this staging form) □ iris (use Uveal Melanoma-Iris Melanoma staging form) |
| 2. | Largest basal diameter and thickness of tumor: |
| 3. | Ciliary body involvement: |
| 4. | Extraocular extension: |
| 5. | Histologic type: |
| 6. | Chromosome 3 and 8 loss or gain: |
| 7. | Gene expression profile: |
| 8. | Mitotic count (number of mitoses per 40 HPF, determined by using a 40x objective with a field area of 0.152 mm2): |
| 9. | Extravascular matrix patterns (extracellular closed loops and networks, defined as at least three back-to-back closed loops, is associated with death from metastatic disease): |
| 10. | Microvascular density: |
| 1. | Tumor site (ICD code lacks specificity): □ ciliary body (use this staging form) □ iris (use Uveal Melanoma-Iris Melanoma staging form) |
| 2. | Largest basal diameter and thickness of tumor: |
| 3. | Ciliary body involvement: |
| 4. | Extraocular extension: |
| 5. | Histologic type: |
| 6. | Chromosome 3 and 8 loss or gain: |
| 7. | Gene expression profile: |
| 8. | Mitotic count (number of mitoses per 40 HPF, determined by using a 40x objective with a field area of 0.152 mm2): |
| 9. | Extravascular matrix patterns (extracellular closed loops and networks, defined as at least three back-to-back closed loops, is associated with death from metastatic disease): |
| 10. | Microvascular density: |
HISTOLOGIC GRADE (G)
| ✔ | G | G Definition |
|---|---|---|
| GX | Grade cannot be assessed | |
| G1 | Spindle cell melanoma (>90% spindle cells) | |
| G2 | Mixed cell melanoma (>10% epithelioid cells and <90% spindle cells) | |
| G3 | Epithelioid cell melanoma (>90% epithelioid cells) |
| ✔ | G | G Definition |
|---|---|---|
| GX | Grade cannot be assessed | |
| G1 | Spindle cell melanoma (>90% spindle cells) | |
| G2 | Mixed cell melanoma (>10% epithelioid cells and <90% spindle cells) | |
| G3 | Epithelioid cell melanoma (>90% epithelioid cells) |
Note: Because of the lack of universal agreement regarding which proportion of epithelioid cells classifies a tumor as mixed or epithelioid, some ophthalmic pathologists currently combine grades 2 and 3 (nonspindle, i.e. epithelioid cells detected) and contrast them with grade 1 (spindle, i.e. no epithelioid cells detected).
LYMPHOVASCULAR INVASION (LVI)
| ✔ | Component of LVI Coding | Description |
|---|---|---|
| 0 | LVI not present (absent)/not identified | |
| 1 | LVI present/identified, NOS | |
| 2 | Lymphatic and small vessel invasion only (L) | |
| 3 | Venous (large vessel) invasion only (V) | |
| 4 | BOTH lymphatic and small vessel AND venous (large vessel) invasion | |
| 9 | Presence of LVI unknown/indeterminate |
| ✔ | Component of LVI Coding | Description |
|---|---|---|
| 0 | LVI not present (absent)/not identified | |
| 1 | LVI present/identified, NOS | |
| 2 | Lymphatic and small vessel invasion only (L) | |
| 3 | Venous (large vessel) invasion only (V) | |
| 4 | BOTH lymphatic and small vessel AND venous (large vessel) invasion | |
| 9 | Presence of LVI unknown/indeterminate |
Appendix 8 CLINICAL CLASSIFICATION OF RETINOBLASTOMA
| Group Type | IRCCa | AJCC 8th Edition/OOTFb |
|---|---|---|
| Small tumors | A. Tumor <3mm, distant to the fovea or or optic nerve B. Same as A without vitreous seeds plus subretinal fluid <5mm from the base of tumor or <3mm tumor at the fovea | cT1. Intraretinal tumors(s) with subretinal fluid not more than 5 mm from the base of any tumor cT1a. Tumors <3 mm and further than 1.5 mm from the disc and fovea cT1b. Tumors >3 mm or closer than 1.5 mm from the disc and fovea |
| Focal vitreous seeds and subretinal fluid | C. Focal vitreous seeds close to the tumor or with subretinal fluid less than a quadrant | cT2. Intraocular tumor(s) with retinal detachment, vitreous seeding or subretinal seeding cT2a. Subretinal fluid more than 5 mm of the base of any tumor cT2b. Tumors with vitreous seeding and/or subretinal seeding |
| Large tumors, extensive tumor seeds, other ocular changes | D. Massive vitreous seeds or diffuse, extensive subretinal masses E. Neovascular glaucoma, tumor in in contact with lens, tumor in segment, phthisis, diffuse infiltrative tumor, nonsalvable eyes | cT3. Advanced intraocular tumor(s) cT3a. Phthisis or pre-phthisis bulbi cT3b. Tumor involvement of the pars plana, ciliary body, lens, zonules, iris or anterior chamber cT3c. Intraocular pressure greater than 25mm Hg cT3d. Hyphema and/or massive vitreous hemorrhage cT3e. Aseptic orbital cellulitis |
| Extraocular extension | N/A | cT4. Extraocular tumor(s) involving the orbit, including the optic nerve cT4a. Radiologic evidence of retrobulbar optic nerve involvement or thickening of the optic nerve or involvement of the orbital tissues cT4b. Extraocular tumor clinically evident with proptosis and/or an orbital mass |
| Group Type | IRCCa | AJCC 8th Edition/OOTFb |
|---|---|---|
| Small tumors | A. Tumor <3mm, distant to the fovea or or optic nerve B. Same as A without vitreous seeds plus subretinal fluid <5mm from the base of tumor or <3mm tumor at the fovea | cT1. Intraretinal tumors(s) with subretinal fluid not more than 5 mm from the base of any tumor cT1a. Tumors <3 mm and further than 1.5 mm from the disc and fovea cT1b. Tumors >3 mm or closer than 1.5 mm from the disc and fovea |
| Focal vitreous seeds and subretinal fluid | C. Focal vitreous seeds close to the tumor or with subretinal fluid less than a quadrant | cT2. Intraocular tumor(s) with retinal detachment, vitreous seeding or subretinal seeding cT2a. Subretinal fluid more than 5 mm of the base of any tumor cT2b. Tumors with vitreous seeding and/or subretinal seeding |
| Large tumors, extensive tumor seeds, other ocular changes | D. Massive vitreous seeds or diffuse, extensive subretinal masses E. Neovascular glaucoma, tumor in in contact with lens, tumor in segment, phthisis, diffuse infiltrative tumor, nonsalvable eyes | cT3. Advanced intraocular tumor(s) cT3a. Phthisis or pre-phthisis bulbi cT3b. Tumor involvement of the pars plana, ciliary body, lens, zonules, iris or anterior chamber cT3c. Intraocular pressure greater than 25mm Hg cT3d. Hyphema and/or massive vitreous hemorrhage cT3e. Aseptic orbital cellulitis |
| Extraocular extension | N/A | cT4. Extraocular tumor(s) involving the orbit, including the optic nerve cT4a. Radiologic evidence of retrobulbar optic nerve involvement or thickening of the optic nerve or involvement of the orbital tissues cT4b. Extraocular tumor clinically evident with proptosis and/or an orbital mass |
a
Murphree AL. Intraocular retinoblastoma: the case for a new group classification. Ophthalmol Clin N Am 2005;18:41-53.
b
Amram AL, Rico G, Kim JW, et al. Vitreous seeds in retinoblastoma: clinicopathologic classification and correlation. Ophthalmology 2017;124:1540-7.
c
IIRC = International Intraocular Retinoblastoma Classification; AJCC = American Joint Commission for Cancer; OOTF = Ophthalmic Oncology Task Force.
APPENDICES REFERENCES
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Amin
MB
, Edge
SB
, Greene
FL
, et al, eds. AJCC cancer staging manual
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Swerdlow
SH
, Campo
E
, Harris
NL
, et al. WHO classification of tumours of haematopoietic and lymphoid tissues
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