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Orbital Tumors

Determining the Stage of the Cancer

The final step before discussing treatment options is a determination of the stage of the cancer. As with all cancers of the head and neck, doctors in the U.S. use the AJCC Cancer Staging Manual (7th Ed) to determine the stage based on three factors.

Factors that go into determining the stage of the cancer
T Characteristics of the main tumor mass
N Status of the lymph nodes in the neck (i.e., evidence of cancer spread)
M Status of cancer spread to parts of the body outside of the head and neck

At first, you will be given a clinical stage based on all of the available information.

  • Clinical staging (cTNM) is determined from any information your doctor might have about how extensive the cancer is BEFORE starting any treatment. Stage is determined based on your doctor’s physical exam, imaging studies, laboratory work and biopsies. Classification of clinical stage is described using the lower case prefix c (e.g., cT, cN, cM).

If there is surgical removal of the cancer as part of your treatment, a pathologist will analyze the tumor and any lymph nodes that may have been removed. You will then be assigned a pathologic stage.

  • Pathologic staging (pTNM) provides more data. Classification of pathology stage is described using the lower case prefix p (e.g., pT, pN, pM). This may or may not differ from the clinical stage.

There are also a number of other lower-case prefixes that might be used in the staging of your cancer.

  • The subscript y (yTNM) is used to assign a cancer stage after some sort of medical, systemic or radiation treatment is given (Posttherapy or Postneoadjuvant Stage). It is typically combined with either a clinical or pathologic stage. For example, ycT2N0M0 indicates that after some sort of non-surgical therapy, the new clinical stage is T2N0M0.
  • The subscript r (rTNM) is used when the tumor has recurred after some period of time in which it was gone. This is called Retreatment Classification Stage. Your doctor will use all the available information to assign you a retreatment stage.

However, cancers of the orbit are a little different from other cancers in the head and neck. Though we can assign them a TNM category, there is no good staging system. This is likely because they are so rare, so scientists have not been able to separate patients into different groups in a scientifically sound way. Of the eye tumors staged in the AJCC manual, only carcinoma of the eyelid and malignant melanoma of the uvea have a stage associated with different TNM categories.

The TNM staging of different cancers of the orbit are as follows:

  • Staging of Carcinoma of the Lacrimal Gland
  • Staging of Sarcoma of the Orbit
  • Staging of Ocular Adnexal Lymphoma
  • Staging of Carcinoma of the Eyelid

Carcinoma of the lacrimal gland staging:

T stage: the main tumor mass (carcinoma of the lacrimal gland)

Based on a physical examination and review of any imaging, your doctor should be able to give you a T stage that falls within one of the following categories.

52lacrimal_Tstage

Tx The primary tumor cannot be evaluated.
T0 There is no evidence of a primary tumor.
T1 The tumor is 2 centimeters or less, whether or not it has extended into the orbital soft tissue outside of the gland.
T2 The size of the tumor is between 2 and 4 centimeters.
T3 The tumor is more than 4 centimeters at its largest dimension.
T4a The tumor has invaded into the layer of tissue covering the bone (periosteum).
T4b The tumor invades into the bones of the orbit.
T4c The tumor has invaded structures nearby such as the brain, sinus, pterygoid fossa or temporal fossa.

N stage: spread of cancer to the lymph nodes in the neck (carcinoma of the lacrimal gland)

Next, your doctor will use all the available information and assign you an N stage. This is based on the assessment as to whether the cancer has spread to lymph nodes in the neck.

 

Nx The neck lymph nodes cannot be assessed.
N0 There is no evidence of any spread to the nodes.
N1 There is spread to lymph nodes in the region.

M stage: spread of cancer outside the head and neck (carcinoma of the lacrimal gland)

Finally, based on an assessment on the entire body, you will be assigned an M stage.

M0 No evidence of distant (outside the head and neck) spread.
M1 There is evidence of spread outside of the head and neck (i.e., in the lungs, bone, brain, etc.).

There is no staging or prognostic group associated with this tumor.

Sarcoma of the orbit staging:

T stage: the main tumor mass (sarcoma of the orbit)

Based on a physical examination and review of any imaging, your doctor should be able to give you a T stage that falls within one of the following categories.

53orbit_sarc_Tstages

Tx The primary tumor cannot be evaluated.
T0 There is no evidence of a primary tumor.
T1 The tumor is 15 millimeters or less at its largest dimension.
T2 The tumor is larger than 15 millimeters at its largest point, but it has not invaded into the globe or bones of the eye socket.
T3 The tumor can be of any size, but it has invaded the orbital tissues and/or the bones of the eye socket.
T4 The tumor has invaded the globe or structures around the orbit (such as eyelids, temporal fossa, nasal cavity, sinuses or the brain).

N stage: spread of cancer to the lymph nodes in the neck (sarcoma of the orbit)

Next, your doctor will use all the available information and assign you an N stage. This is based on his or her assessment as to whether the cancer has spread to lymph nodes in the neck.

Nx The neck lymph nodes cannot be assessed.
N0 There is no evidence of any spread to the nodes.
N1 There is spread to lymph nodes in the region.

M stage: spread of cancer outside the head and neck (sarcoma of the orbit)

Finally, based on an assessment on the entire body, you will be assigned an M stage.

M0 No evidence of distant (outside the head and neck) spread.
M1 There is evidence of spread outside of the head and neck (i.e., in the lungs, bone, brain, etc.).

There is no staging or prognostic group associated with this tumor.

Ocular Adnexnal Lymphoma Staging:

T stage: the main tumor mass (ocular adnexnal lymphoma)

Based on a physical examination and review of any imaging, your doctor should be able to give you a T stage that falls within one of the following categories.

Tx The primary tumor cannot be evaluated.
T0 There is no evidence of any lymphoma.
T1 The lymphoma involves the conjunctiva only.
T1a The lymphoma involves the conjunctiva over the eyeball only.
T1b The lymphoma involves the eyelid portion of the conjunctiva (palpebral conjunctiva), with or without involvement of the fornix or caruncle.
T1c The lymphoma has extensive involvement of the conjunctiva.
T2 The lymphoma has involved some part of the orbit (with or without conjunctival involvement).
T2a The front part of the orbit is involved (with or without conjunctival involvement).
T2b The front part of the orbit and the lacrimal gland are involved (with or without conjunctival involvement).
T2c The back part of the orbit is involved (with or without front of the orbit involvement, with or without conjunctival involvement, with or without extraocular muscle involvement).
T2d The nasolacrimal duct is involved.
T3 The lymphoma involves the eyelid (in front of the orbital septum).
T4 The lymphoma extends beyond the orbit.
T4a Involvement of the nasopharynx.
T4b Involvement of the bone (including the covering over the bones called the periosteum).
T4c The maxillary, ethmoid or frontal sinuses are involved.
T4d The lymphoma involves the brain.

N stage: spread of cancer to the lymph nodes in the neck (ocular adnexnal lymphoma)

Next, your doctor will use all the available information and assign you an N stage. This is based on his or her assessment as to whether the cancer has spread to lymph nodes in the neck.

Nx The neck lymph nodes cannot be assessed.
N0 There is no evidence of any spread to the nodes.
N1 There is spread to lymph nodes in the region on the same side as the primary tumor. This includes nodes in the parotid gland, submandibular area and the side of the neck.
N2 There is spread to lymph nodes in the region on the opposite side or both sides. This includes nodes in the parotid gland, submandibular area and the side of the neck.
N3 Lymph nodes that are on the periphery of the body but not draining from the eye area are involved.
N4 The central lymph nodes in the body are involved.

M stage: spread of cancer outside the head and neck (ocular adnexnal lymphoma)

Finally, based on an assessment on the entire body, you will be assigned an M stage.

M0 No evidence of distant (outside the head and neck) spread.
M1a Areas that are not directly connected to the orbit are involved (parotid gland, submandibular gland, lung, liver, spleen, kidney, breast, etc).
M1b The bone marrow is involved by lymphoma.
M1 Both M1a and M1b.

There is no staging or prognostic group associated with this tumor.

Carcinoma of the Eyelid Staging:

Because tumors of the eyelid can behave differently than the same tumor in a different location, a separate staging system has been created. Eyelid carcinomas that use this staging system include:

  • Basal cell carcinoma
  • Squamous cell carcinoma
  • Mucoepidermoid carcinoma
  • Sebaceous carcinoma
  • Primary eccrine adenocarcinoma
  • Primary apocrine adenocarcinoma
  • Adenoic cystic carcinoma
  • Merkel cell carcinoma

T stage: the main tumor mass (carcinoma of the eyelid)

58eyelid_Tstages

Based on a physical examination and review of any imaging, your doctor should be able to give you a T stage that falls within one of the following categories.

Tx The primary tumor cannot be evaluated.
T0 There is no evidence of a primary tumor.
Tis This is carcinoma in situ (has not invaded deeply past the basement membrane layer).
T1 The tumor is less than 5 millimeters and does not invade the eyelid margin or the tarsal plate.
T2a The tumor is more than 5 millimeters but not more than 10 millimeters, OR any tumor that invades to the margin of the eyelid or the tarsal plate in the eyelid.
T2b The tumor is more than 10 millimeters but less than 20 millimeters, OR the tumor involves the full thickness of the eyelid (outer skin to inner conjunctiva).
T3a The tumor is more than 20 millimeters at its largest point, OR any tumor of the eyelid that invades ocular or orbital structures nearby.
T3b To get the tumor out would involve removal of the eyeball (with or without removal of additional parts of the orbit).
T4 The tumor cannot be removed surgically because it has grown into the brain or extensive invasion of the skull base/face, orbit or ocular structures.

N stage: spread of cancer to the lymph nodes in the neck (carcinoma of the eyelid)

Next, your doctor will use all the available information and assign you an N stage. This is based on his or her assessment as to whether the cancer has spread to lymph nodes in the neck.

Nx The neck lymph nodes cannot be assessed.
cN0 There is no evidence of any spread to the nodes based on clinical evaluation or imaging tests.
pN0 There is spread to lymph nodes in the region based on biopsy of the lymph nodes.
N1 There is spread to lymph nodes in the region.

M stage: spread of cancer outside the head and neck (sarcoma of the orbit)

Finally, based on an assessment on the entire body, you will be assigned an M stage.

M0 No evidence of distant (outside the head and neck) spread.
M1 There is evidence of spread outside of the head and neck (i.e., in the lungs, bone, brain, etc.).

Finally, based on the TNM staging, you will be given a cancer stage based on the following chart:

T N M
Stage 0 Tis N0 M0
Stage IA T1 N0 M0
Stage IB T2a N0 M0
Stage IC T2b N0 M0
Stage II T3a N0 M0
Stage IIIA T3b N0 M0
Stage IIIB Any T N1 M0
Stage IIIC T4 Any N M0
Stage IV Any T Any N M1
References

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