Summary Stage 2018: Retinoblastoma
Summary Stage 2018
Notes
**Retinoblastoma**
9510-9514
C690-C696, C698-C699
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:** The following sources were 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:** Code 0 is not applicable for this chapter.
**Note 3:** Pathological staging information from an enucleation always takes precedence over clinical staging, except in 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: 3-30
(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: 819-831
