Summary Stage 2018: Retinoblastoma
Summary Stage 2018
Notes
**Retinoblastoma**
**C690-C699**: 9510-9514
C690: Conjunctiva
C691: Cornea, NOS
C692: Retina
C693: Choroid
C694: Ciliary Body
C695: Lacrimal Gland
C696: Orbit, NOS
C698: Overlapping lesion of eye and adnexa
C699: Eye, NOS
**Note 1:** **Sources used in the development of this chapter**
* SEER Extent of Disease 1988: Codes and Coding Instructions (3rd Edition, 1998) (https://seer.cancer.gov/archive/manuals/EOD10Dig.3rd.pdf)
* SEER Summary Staging Manual-2000: Codes and Coding Instructions (https://seer.cancer.gov/tools/ssm/ssm2000/)
* Collaborative Stage Data Collection System, version 02.05: https://cancerstaging.org/cstage/Pages/default.aspx
* Chapter 68 *Retinoblastoma*, in the AJCC Cancer Staging Manual, Eighth Edition (2017) published by Springer International Publishing. Used with permission of the American College of Surgeons, Chicago, Illinois.
**Note 2:** **Not applicable codes**
* Code 0 is not applicable for this chapter.
**Note 3:** **Enucleation**
* Pathological staging information from an enucleation always takes precedence over clinical staging
* *Exception*: Cases with neoadjuvant treatment where clinical disease is as extensive as or more extensive than disease at surgery.
| SS2018 |
Description |
| 1 |
**Localized only (localized, NOS)**
* Confined to retina, NOS
* Intraocular tumor(s) WITH local invasion, NOS
- Choroid (concomitant focal invasion)
- Pre- or intralaminar involvement of optic nerve head
- Retinal detachment
- Schlemm's canal
- Stromal invasion iris
- Subretinal seeding
- Trabecular meshwork
- Vitreous seeding
* Advanced intraocular tumor(s) WITH significant local invasion
- Anterior chamber
- Aseptic orbital cellulitis
- Buphthalmos
- Choroid (multiple foci, focal, full-thickness involvement)
- Ciliary body
- Emissary channels
- Hyphema AND/OR massive vitreous hemorrhage
- Iris
- Lens
- Pars plana
- Phthisis or pre-phthisis bulbi
- Raised intraocular pressure with neovascularization
- Retrolaminar invasion of optic nerve head
- Sclera
- Zonules |
| 2 |
**Regional by direct extension only**
* Extraocular tumor
- Adjacent adipose tissue
- Bone
- Conjunctiva
- Episclera
- Extraocular muscle
- Eyelids
- Meningeal spaces (around optic nerve)
- Optic nerve (clinical thickening)
- Optic nerve (retrobulbar, extraocular)
- Orbit
- Orbital tissue (includes clinical orbital tissue thickening) |
| 3 |
**Regional lymph node(s) involved only**
* Cervical, NOS
* Mandibular, NOS
- Submandibular (submaxillary)
* Parotid
- Infra-auricular
- Preauricular
* Regional lymph node(s), NOS
- Lymph node(s), NOS |
| 4 |
**Regional by BOTH direct extension AND regional lymph node(s) involved**
* Codes (2) + (3) |
| 7 |
**Distant site(s)/lymph node(s) involved**
* Distant site(s) (including further contiguous extension)
- Cerebrospinal fluid
- CNS parenchyma
* Distant lymph node(s), NOS
* Distant metastasis, NOS
- Carcinomatosis
- Distant metastasis WITH or WITHOUT distant lymph node(s) |
| 9 |
Unknown if extension or metastasis |
(1) Fritz AG, Ries LAG (eds). **SEER Extent of Disease 1988: Codes and Coding Instructions (3rd Edition, 1998)**, National Cancer Institute, NIH Pub. No. 98-2313, Bethesda, MD, 1998
(2) Young JL Jr, Roffers SD, Ries LAG, Fritz AG, Hurlbut AA (eds.). **SEER Summary Staging Manual-2000: Codes and Coding Instructions**, National Cancer Institute, NIH Pub. No. 01-4969, Bethesda, MD, 2001.
(3) Collaborative Stage Work Group of the American Joint Committee on Cancer. **Collaborative Stage Data Collection System User Documentation and Coding Instructions, version 02.05**. American Joint Committee on Cancer (Chicago, IL)
(4) Gress, D.M., Edge, S.B., Gershenwald, J.E., et al. **Principles of Cancer Staging**. In: Amin, M.B., Edge, S.B., Greene, F.L., et al. (Eds.) AJCC Cancer Staging Manual. 8th Ed. New York: Springer; 2017
(5) Mallipatna, A.C., Finger, P.T., et al. **Retinoblastoma**. In: Amin, M.B., Edge, S.B., Greene, F.L., et al. (Eds.) AJCC Cancer Staging Manual. 8th Ed. New York: Springer; 2017
