Recommendations for the Reporting of Tissues Removed as Part of the Surgical Treatment of Common Malignancies of the Eye and its Adnexa Robert Folberg, M.D., Diva Salomao, M.D., Hans E. Grossniklaus, M.D., Alan D. Proia, M.D., Ph.D., Narsing A. Rao, M.D., J. Douglas Cameron, M.D. University of Illinois at Chicago (RF), Chicago, Illinois; The Mayo Clinic (DS, JDC), Rochester, Minnesota; Emory University (HEG), Atlanta, Georgia; Duke University (ADP), Durham, North Carolina; and The Keck School of Medicine (NAR)-University of Southern California, Los Angeles, California The purpose of these recommendations is to pro- The Association of Directors of Anatomic and Sur- vide an informative report for the clinician. The gical Pathology developed recommendations for the recommendations are intended as suggestions, and surgical pathology report for common malignant adherence to them is completely voluntary. In spe- tumors. The recommendations for tumors of the cial circumstances, the recommendations may not eye and its adnexa are reported. be applicable. The recommendations are intended as an educational resource rather than a mandate. KEY WORDS: Basal cell carcinoma, Conjunctiva, Eyelid, Melanoma, Orbit Retinoblastoma, Seba- ceous carcinoma, Squamous cell carcinoma, Uvea. BACKGROUND Mod Pathol 2003;16(7):725–730 Before the public awareness of AIDS and Alzhei- The Association of Directors of Anatomic and mer’s disease as health problems, the disease Surgical Pathology named several committees to feared most by Americans was cancer; the second develop recommendations about the content of the most feared condition was blindness (The Gallup surgical pathology report for common malignant Organization, Inc., Public knowledge and attitudes tumors. A committee of persons with special inter- concerning blindness—a survey sponsored by Re- est and expertise write the recommendations, search to Prevent Blindness, Inc., New York, Octo- which are reviewed and approved by the council of ber 1965 and April 1976, unpublished data). Pa- Association of Directors of Anatomic and Surgical tients who are confronted with a diagnosis of ocular Pathology and subsequently by the entire cancer, therefore, face two of their most principal membership. fears: shortening of their lifespan and loss of vision. The recommendations have been divided into Ophthalmologists who manage most patients with four major areas: 1) items that provide an informa- ocular malignancies often try to balance the pa- tive gross description; 2) additional diagnostics fea- tient’s desire to preserve vision with the goal of tures recommended for inclusion in every report if eradicating the cancer. In general, the pathologist’s possible; 3) optional features that may be included report should catalog not only those features ap- in the final report; and 4) a checklist. propriate for estimating the natural history of the patient’s disease (prognosis), but also those fea- tures that might compromise vision. Copyright © 2003 by The United States and Canadian Academy of In general, it is recommended that pathologists Pathology, Inc. report on malignancies of the orbit using formats VOL. 16, NO. 7, P. 725, 2003 Printed in the U.S.A. Date of acceptance: February 26, 2003. either published or in development for the coun- The authors were the members of an ad hoc committee of the Association terpart lesion elsewhere in the body. For example, of Directors of Anatomic and Surgical Pathology chaired by Robert Fol- berg, M.D., Chicago, Illinois. lymphomas of the orbit should be reported accord- Address reprint requests to: Robert Folberg, M.D., University of Illinois at ing to generalized recommendations for reporting Chicago, Department of Pathology (MC 847), 1819 West Polk Street, Room 446, Chicago, IL 60612-7335. lymphomas. It is reasonable for the report for rhab- Note: Ophthalmic pathologists use the term “primary acquired melanosis domyosarcoma (the most common primary malig- with atypia” in place of the following terms: intraepithelial atypical mela- nocytic hyperplasia, malignant melanoma in situ, Level I malignant nancy of the orbit in childhood in the United melanoma. States) to follow recommendations for reporting DOI: 10.1097/01.MP.0000076978.06324.EE rhabdomyosarcomas in general. The lacrimal gland 725 may be considered to be a minor salivary gland for 7. Orientation of the lesion if provided by the the purposes of reporting malignancies in this re- surgeon gion, and pathology reports dealing with lacrimal 1. Some surgeons will identify surgical margins of gland malignancies (principally adenoid cystic car- interest by applying a suture to an edge of the cinoma) should follow recommendations for re- specimen, by painting certain margins with dyes, or porting this tumor as described for the salivary attaching the specimen to a piece of filter paper and gland. making notations on the specimen mount Recommendations are therefore offered for three B. Iridectomy/iridocyclectomy classes of ocular malignancies: conjunctival neo- 1. Dimensions of the specimen (length, width, plasms (including those affecting the limbus—the thickness) junction between the cornea and sclera), sebaceous 2. Description of tissue received (iris only, iris and carcinoma (a common malignancy of the eyelid), ciliary body, iris, ciliary body and peripheral cornea and the two major intraocular neoplasms (retino- and/or sclera including location by clock hour) blastoma and malignant melanoma). 3. Dimensions of lesion (length, width, height) 1. General –the Association recommends that the 4. Measurement of minimum distance between following features be included in the final report edge of lesion and surgical margin (minimum because they are generally accepted as being of clearance) prognostic importance, of visual importance, re- 1. Relevant surgical margins include the lateral quired for staging or therapy, and/or traditionally margins and the posterior margin (the anterior expected. margin is the pupillary border and is not a true A. How the specimen was received (e.g., fresh or surgical margin) in fixative) C. Enucleation B. How the specimen was identified (e.g., labeled 1. Dimensions of the eye (anterior-posterior, hor- with the name, medical record number, and sur- izontal, vertical) geon’s name) 2. Length of optic nerve attached C. Laterality of the lesion (e.g., originating from 3. Examination of the surface of the eye for gross the right or left eye) evidence of extraocular extension of tumor D. The exact anatomic location of the tumor 4. Dimensions of the cornea (horizontal and 1. Conjunctiva: bulbar (by quadrant –superior, vertical) inferior, nasal, temporal), palpebral (superior or in- 5. Clarity of the cornea ferior), fornix (superior or inferior) 6. Color of the iris (describe lesions if present) 2. Limbus (by clock hour) 7. Shape and diameter of the pupil 3. Caruncle or plica semilunaris 8. Transillumination of the eye with dimensions 4. Eyelid (upper, lower, medial canthus, lateral of any shadows canthus) 1. Transillumination of the eye may be performed 5. Intraocular tissue (iris, ciliary body –by clock with a fiberoptic light source to locate a tumor hour) within the eye by the shadow that it casts during E. The type of surgical procedure this procedure 1. Incisional biopsy, excisional biopsy, shave bi- 1. Location of transillumination shadow(s) rela- opsy (conjunctiva, eyelids) tive to the limbus and optic nerve (distance of 2. Iridectomy (removal of iris tissue), iridocyclec- shadow borders from limbus and optic nerve) tomy (removal of iris and ciliary body tissue) 2. Location of the shadow relative to clock hour 3. Enucleation (removal of eye) 9. Describe the section plane used to open the 4. Exenteration (removal of the eye and orbital eye contents, with or without eyelids, not covered in 10. Obtain cross section of the optic nerve this report) 1. Retinoblastoma 2. Gross Description 1. Obtain a section from either the surgical mar- A. Conjunctival and Eyelid Biopsy gin of the optic nerve (the transected edge) or the 1. Dimensions of the specimen (length, width, cut surface of the optic nerve as it inserts in the eye thickness) 1. If the cut section of the optic nerve adjacent to 2. Maximum diameter of any visible lesion the eye is negative for tumor, then one may con- 3. Measurement of minimum distance between clude that there is no involvement of the nerve edge of lesion and surgical margin (minimum posterior to the eye (tumor extends through the clearance) nerve without skip lesions) 4. Presence or absence of ulceration 2. Frequently, the surgical margin of the optic 5. Color of the lesion and adjacent tissue nerve (the cut edge of the nerve) is crushed by 6. Description of attached tissue (episclera, enucleation scissors cornea) 11. Describe the cut surface of the eye 726 Modern Pathology 1. Retinoblastoma modification of the Breslow method using a cali- 1. Number and size of lesions brated ocular micrometer. The tumor thickness is 2. Location of the lesion(s) measured from the top of the epithelium (there is 3. Gross evidence of choroidal invasion or ex- no granular layer in the normal conjunctival traocular extension epithelium). 4. Presence or absence of vitreous seeding 1. Some pathologists consider a depth of invasion 5. Presence or absence of retinal detachment of 0.8 mm to be significant in separating patients at 2. Uveal melanoma high risk for metastasis from those at low risk. 1. Tissues involved (choroid only, choroid and 4. Intralymphatic invasion by tumor ciliary body, ciliary body only, iris and ciliary body, 5. Optional criteria iris only) 1. Mitotic figures/mm2; proliferation indices us- 2. Location of the melanoma relative to clock ing markers such as MIB-1 or Ki67 hour C. Sebaceous carcinoma of the eyelid 3. Dimensions from cut surface (maximum zone 1. Location of the tumor (upper eyelid versus of scleral contact, dimension perpendicular to max- lower eyelid) imum zone of scleral contact, elevation measured 2. Size in millimeters from top of lesion to interface with sclera) 1. Some pathologists consider this measurement 4.