• My Dashboard My Education Find an Ophthalmologist
  • Home
  • For Ophthalmologists
    • Meetings
      • AAO 2021
        • Meeting Information
          • Past and Future Meetings
          • Contact Information
          • Virtual Meeting Guide
          • Policies and Disclaimers
        • Program
          • Program Search
          • Program Highlights
          • Program Committees
          • CME
          • Meeting Archives
        • Expo
        • Registration
        • Hotels & Travel
          • Hotel Reservation Information
          • New Orleans
          • International Attendees
          • Hotel Meeting Space
        • Presenter Central
          • Presenter Central
          • Abstract Selection Process
          • Submission Policies
          • Subject Classification/Topics
          • Instruction Courses and Skills Transfer Labs
          • Papers and Posters
          • Videos
          • Grand Rounds Symposium
          • Program Participant and Faculty Guidelines
          • Faculty Development Program
        • Exhibitors
          • Exhibitor Central
          • Exhibitor Portal Information
          • New Exhibiting Companies
          • Exhibitor Resources
      • Mid-Year Forum
        • Registration and Travel
        • Congressional Advocacy Day
        • Advocacy Ambassador Program
        • Program
        • Schedule
        • Sponsored Attendees
        • News
      • Ophthalmology Business Summit
      • Codequest
        • Codequest Instructors
        • Claim Codequest CME or CEU Credit
      • Eyecelerator
    • Clinical Education
      • COVID-19
      • Education
        • Browse All Education
        • Courses
        • Cases
        • Learning Plans
        • Interactive
        • Focal Points
        • Wills Eye Manual
        • Disease Reviews
        • Clinical Webinars
        • Diagnose This
        • Self-Assessments
        • Educational Centers
          • Glaucoma Education Center
          • Pediatric Ophthalmology Education Center
          • Laser Surgery Education Center
          • Myopia Resources
          • Oculofacial Plastic Surgery Center
          • Redmond Ethics Center
      • Journals
      • Guidelines
        • Browse All Practice Guidelines
        • Preferred Practice Patterns
        • Clinical Statements
        • Compendium Guidelines
        • Complementary Therapy Assessments
        • Medical Information Technology
        • Ophthalmic Technology Assessments
        • Patient Safety Statements
        • Choosing Wisely
        • Low Vision
        • Eye Care for Older Adults
        • Eye Disease Statistics
        • About the Hoskins Center
      • Multimedia Library
        • Browse All Videos and Audio
        • Clinical and Surgical Videos
        • Presentations and Lectures
        • 1-Minute Videos
        • Master Class Videos
        • Basic Skills Videos
        • Interviews
        • Audio and Podcasts
        • Images
        • Submit an Image
        • Submit a Video
      • News
        • Browse All Clinical News
        • Editors' Choice
        • Headlines
        • Current Insight
      • CME Central
        • Browse All CME Activities
        • Claim CME Credit and View Transcript
        • CME Planning Resources
        • Complete Your Financial Disclosure
        • Joint Sponsorship Portal
        • LEO Continuing Education Recognition Award
        • Safe ER/LA Opioid Prescribing
        • Check Your Industry Payment Records
      • MOC
      • Resident Education
        • All Resident Education
        • OKAP and Board Exam Resources
          • OKAP Exam
          • Board Prep Resources
          • OKAP and Board Review Presentations
          • Study Flashcards
        • Resident Courses
        • Resident Videos
        • Cataract Master
        • Simulation in Resident Education
        • Pediatric Ophthalmology Education Center
        • News and Advice from YO Info
    • Membership
      • Join
      • Renew
      • Current Member
      • Volunteer
      • Physician Wellness
      • Member Directory
      • Member Obituaries
      • AAOE Membership
    • Advocacy
      • Advocacy News
      • Get Involved
        • Ways to Give
        • How to Get Involved
        • Congressional Advocacy
        • Support the Academy's Agenda
        • Research Legislation
        • Find Your Legislators
        • I Am an Advocate
        • Advocacy at Home
        • Advocate Tools
        • Best Practices for Advocating at Home
        • Social Media Toolkit
        • Letter to Editor
        • Town Hall Guide
        • Guide to Engaging With New Lawmakers
        • Resources
        • Attending a Political Fundraiser
      • OPHTHPAC
        • About Us
        • Join OPHTHPAC
        • OPHTHPAC Blog
      • Surgical Scope Fund
        • Support Surgery By Surgeons
        • Surgery By Surgeons Blog
    • Publications
      • EyeNet Magazine
        • Latest Issue
        • Archive
        • Subscribe
        • Advertise
        • Write For Us
        • Corporate Lunches
        • Contact
        • MIPS 2019
        • MIPS 2020
      • Focal Points
      • Ophthalmology
      • Ophthalmology Glaucoma
      • Ophthalmology Retina
      • YO Info
      • Scope
    • Subspecialties
      • Cataract/Anterior Segment
      • Comprehensive Ophthalmology
      • Cornea/External Disease
      • Glaucoma
      • Neuro-Ophthalmology/Orbit
      • Ocular Pathology/Oncology
      • Oculoplastics/Orbit
      • Pediatric Ophthalmology/Strabismus
      • Refractive Management/Intervention
      • Retina/Vitreous
      • Uveitis
    • IRIS Registry
      • About
      • Using the Registry
        • User Guide
        • Medicare Reporting
        • Maintenance of Certification
        • Non-EHR Reporting
      • Sign Up
        • Application Process
        • Why Participate
        • Once You've Applied: Getting Started
        • What Practices Are Saying About the Registry
      • Requirements
        • EHR Systems
        • Data & Technical Needs
      • Research
      • Registry Dashboard
      • News
    • Medicare Information
  • For Practice Management
    • Coding
      • Codequest
      • Ask the Coding Experts
      • Coding Updates and Resources
      • Coding for Injectable Drugs
      • ICD-10-CM
        • News and Advice
      • Ophthalmic Coding Specialist (OCS) Exam
    • Regulatory
      • Medicare Participation Options
      • Audits
      • Medicare Advantage Plans
        • Termination Appeal Letter
      • New Medicare Card
      • Provider Enrollment, Chain and Ownership System (PECOS)
      • HIPAA Resources
    • Practice Operations
      • Practice Management Advice
      • Lean Management
      • Cybersecurity
      • Private Equity
      • EHRs
        • Overview
        • Planning and Preparation
        • Vendor Selection
        • Implementation and Evaluation
        • Patient Portals
        • Resources
        • Ratings
    • Events
      • Annual Meeting
      • Business Summit
      • Codequest Courses
    • Leadership
      • AAOE Board of Directors
      • Leadership Program (OPAL)
    • Listservs
    • Resources
      • Practice Management Resource Library
      • Coronavirus Resources
      • Patient Education
      • Practice Forms Library
        • Practice Forms Library - Examination
        • Practice Forms Library - Financial
        • Practice Forms Library - HIPAA
        • Practice Forms Library - Human Resources
        • Practice Forms Library - Job Descriptions
        • Practice Forms Library - Patient Forms
        • Practice Forms Library - Protocols
        • Practice Forms Library - Surgery
      • Practice Analytics
        • Benchmarking Tool
        • Salary Survey
      • Consultant Directory
      • Ophthalmology Job Center
      • Practice Management for Retina
      • Reopening and Recovery
    • Get Involved
    • Medicare
      • Quality
        • Overview
        • Reporting
        • Measures
      • Promoting Interoperability
        • Overview
        • Measures
        • Attestation
        • Hardships and Exceptions
        • Audits
        • News and Advice
      • Improvement Activities
        • Overview
        • List of Improvement Activities
        • Attestation
        • Audits
      • Cost
      • Avoid a Penalty
      • Resources
        • 2019 to 2020 MIPS Changes
        • MIPS Solo and Small Practice Survival Guide
        • MIPS Glossary
        • MIPS Resources on EyeNet
        • MIPS Extreme Hardship Exceptions
        • Solo and Small-Practice Roadmap
        • MIPS Manual
        • MIPS Large Practice Roadmap
        • IRIS Registry User Guide
        • CMS Websites
        • 2020 MIPS Payments: Understanding Remittance Advice Codes
        • Final Checklist for EHR/Non-EHR 2019 MIPS Reporting
        • MIPS Tips
        • MIPS Archive
    • Membership
  • For Public & Patients
    • Eye Health A-Z
    • Symptoms
    • Glasses & Contacts
    • Tips & Prevention
    • News
    • Ask an Ophthalmologist
    • Patient Stories
    • No Cost Eye Exams
    • Español
      • A - Z de Salud Ocular
      • Síntomas
      • Anteojos y Lentes de Contacto
      • Consejos y Prevención
      • Noticias
      • Relatos de Pacientes
      • Exámenes de la vista sin costo
      • English
  • AAO 2021
    • Meeting Information
      • Past and Future Meetings
      • Contact Information
      • Virtual Meeting Help
      • Virtual Meeting Guide
      • Policies and Disclaimers
    • Program
      • Program Search
      • Program Highlights
      • Program Committees
      • CME
      • Meeting Archives
    • Expo
    • Registration
    • Hotels & Travel
      • Hotel Reservation Information
      • New Orleans
      • International Attendees
      • Hotel Meeting Space
    • Presenter Central
      • Presenter Central
      • Abstract Selection Process
      • Submission Policies
      • Subject Classification/Topics
      • Instruction Courses and Skills Transfer Labs
      • Papers and Posters
      • Videos
      • Grand Rounds Symposium
      • Program Participant and Faculty Guidelines
      • Faculty Development Program
    • Exhibitors
      • Exhibitor Central
      • Exhibitor Portal Information
      • New Exhibiting Companies
      • Exhibitor Resources
  • About
    • Who We Are
      • What We Do
      • About Ophthalmology
      • The Eye Care Team
      • Ethics and the Academy
      • History
      • Museum of Vision
      • Values
    • Governance
      • Council
      • Board of Trustees
      • Committees
      • Academy Past Presidents
      • Secretariats
      • Elections
      • Academy Blog
      • Academy Staff Leadership
    • Leadership Development
    • Awards
      • Laureate Recognition Award
      • Outstanding Advocate Award
      • Outstanding Humanitarian Service Award
      • International Blindness Prevention Award
      • Distinguished Service Award
      • Guests of Honor
      • Secretariat Award
      • Straatsma Award
      • Achievement Award Program
      • Artemis Award
      • EnergEYES Award
      • International Education Award
      • International Scholar Award
      • Commitment to Advocacy Award
      • Visionary Society Award
    • Financial Relationships
    • Policy Statements
    • Related Organizations
      • Subspecialty/Specialized Interest Society Directory
      • State Society Directory
      • Subspecialty/Specialized Interest Society Meetings
      • State Society Meetings
      • Resources for Societies
    • Year in Review
      • 2019 Year in Review
  • Foundation
    • About
      • Funding Allocations and Sources
      • 2019-2020 Annual Report
      • Annual Report Archives
      • News From the Chair
      • Foundation Staff
    • Our Impact
      • Partners for Sight
      • Donor Spotlights
      • Global Ophthalmic Community
      • Sponsorships
      • Patients and the Public
    • Giving Options
      • Our Supporters
      • Estate and Planned Giving
      • Ophthalmic Business Council
    • Orbital Gala
      • Why Attend
      • Photo Recap
      • Corporate Support Opportunities
      • Tribute Gifts
      • Silent Auction
      • Corporate Sponsors
    • Donate
    • Museum of the Eye Campaign
      • Museum Supporters
  • Museum of the Eye
    • Visit
    • Events
    • Explore
      • Research and Resources
      • Collection Search
      • Previous Exhibits
      • Oral Histories
      • Biographies
    • Volunteer
    • Mailing List
    • Donate
    • About the Museum
      • Museum Blog
  • Young Ophthalmologists
    • YO Info
    • Learn to Bill
    • Engage with the Academy
  • Senior Ophthalmologists
    • Scope
    • Practice Transitions
  • International Ophthalmologists
    • Global Programs and Resources for National Societies
    • Awards
    • Global Outreach
  • Residents
  • Medical Students
×
Log In Create an Account
  • For Ophthalmologists
  • For Practice Management
  • For Public & Patients
  • About
  • Foundation
  • Museum of the Eye
  • COVID-19
  • Journals
  • Education
    • Education
    • Courses
    • Cases
    • Learning Plans
    • Interactive
    • Focal Points
    • Wills Eye Manual
    • Disease Reviews
    • Clinical Webinars
    • Diagnose This
    • Self-Assessments
    • Education Centers
      • Glaucoma Education Center
      • Pediatric Ophthalmology Education Center
      • Laser Surgery Education Center
      • Oculofacial Plastic Surgery Center
      • Redmond Ethics Center
      • Myopia Resources
  • Guidelines
    • Practice Guidelines
    • Preferred Practice Patterns
    • Clinical Statements
    • Ophthalmic Technology Assessments
    • Patient Safety Statements
    • Complementary Therapy Assessments
    • Compendium Guidelines
    • Medical Information Technology
    • Low Vision
    • Choosing Wisely
    • Eye Care for Older Adults
    • Eye Disease Statistics
    • About the Hoskins Center
    • Artificial Intelligence
    • Premium IOLs
  • Multimedia
    • Multimedia Library
    • Video
      • 1-Minute Videos
      • Presentations and Lectures
      • Master Class Videos
      • Basic Skills Videos
      • Clinical and Surgical Videos
      • Interviews
      • Resident Lectures
      • Submit a Video
    • Audio and Podcasts
    • Images
      • Submit an Image
  • News
    • Clinical News
    • Editors' Choice
    • Headlines
    • Current Insight
  • CME
    • CME Central
    • Claim CME Credit and View Transcript
    • CME Planning Resources
    • Complete Your Financial Disclosure
    • Joint Sponsorship Portal
    • LEO Continuing Education Recognition Award
    • Safe ER/LA Opioid Prescribing
    • Check Your Industry Payment Records
  • MOC
  • Residents
    • Resident Education
    • OKAP and Board Exam Resources
      • OKAP Exam
      • Board Prep Resources
      • OKAP and Board Review Presentations
      • Study Flashcards
    • Resident Courses
    • Resident Videos
    • Cataract Master
    • Simulation in Resident Education
    • Diversity and Inclusion Education
    • Pediatric Ophthalmology Education Center
    • News and Advice from YO Info
    • Clinical Education /
    • Book Excerpts /
    • Basic and Clinical Science Course - Excerpt
  • 2020–2021 BCSC Basic and Clinical Science Course™

    Go to Academy Store Learn more and Purchase.

    4 Ophthalmic Pathology and Intraocular Tumors

    Part I: Ophthalmic Pathology

    Chapter 14: Orbit and Lacrimal Drainage System

    Neoplasia

    Neoplasms of the orbit may be primary or secondary (extensions from adjacent structures or metastatic disease). Secondary tumors are slightly more common than primary tumors (see the section Secondary Tumors at the end of this chapter). The types of orbital tumors that occur in children differ from those that occur in adults. Developmental tumors are the primary orbital lesions most often encountered in children, whereas vascular and lymphoid tumors are the primary orbital lesions most often seen in adults.

    In children, approximately 90% of orbital tumors are benign. Benign cystic lesions (dermoid or simple epithelial cysts) represent 50% of orbital lesions in childhood. Rhabdomyosarcoma is the most common primary orbital malignant tumor in childhood and represents 3% of all orbital masses. The orbit may be involved secondarily in cases of retinoblastoma, neuroblastoma, and leukemia/lymphoma.

    See BCSC Section 7, Oculofacial Plastic and Orbital Surgery, for additional discussion.

    Lacrimal Gland Neoplasia

    Because the lacrimal gland is very similar to the salivary gland, epithelial lacrimal gland tumors are categorized according to the World Health Organization (WHO) epithelial salivary gland classification system. The most common types of epithelial lacrimal gland tumors are pleomorphic adenoma, adenocarcinoma arising from pleomorphic adenoma (carcinoma ex pleomorphic adenoma), and adenoid cystic carcinoma.

    • von Holstein SL, Rasmussen PK, Heegaard S. Tumors of the lacrimal gland. Semin Diagn Pathol. 2016;33(3):156–163.

    Pleomorphic adenoma

    The most common epithelial tumor of the lacrimal gland is pleomorphic adenoma (also known as benign mixed tumor). Pleomorphic adenoma is slightly more common in men than in women and usually presents in the fourth or fifth decade of life. The tumor is pseudoencapsulated and grows slowly by expansion. This progressive expansive growth may excavate the bone of the lacrimal fossa. Tumor growth stimulates the periosteum to deposit a thin layer of new bone (ie, cortication). The adjacent orbital bone is not eroded. Typically, the patient experiences no pain.

    Histologically, pleomorphic adenoma has a fibrous pseudocapsule and comprises a mixture of duct-derived epithelial and stromal elements, all arising from the lacrimal glandular epithelium. The epithelial component may form nests or tubules lined by 2 layers of cells, the outermost layer blending imperceptibly with the stroma (Fig 14-7). The stroma may appear myxoid and may contain heterologous elements, including cartilage and bone. Results of immunohistochemistry (IHC) reflect the epithelial and myoepithelial components, both derived from epithelium. The tumor cells are usually positive with IHC for keratin and epithelial membrane antigen in the ductal areas and positive with IHC for keratin, actin, myosin, fibronectin, and S-100 protein in the myoepithelial areas. Microscopic foci of carcinoma in situ may be identified in these lesions but are not a significant feature.

    Malignant transformation may take place in a long-standing or incompletely excised pleomorphic adenoma in the form of adenocarcinoma (carcinoma ex pleomorphic adenoma), with relatively rapid growth after a period of relative quiescence. Malignancies, including adenocarcinoma (carcinoma ex pleomorphic adenoma) and adenoid cystic carcinoma, may also develop in pleomorphic adenomas that recur in the orbit.

    Adenoid cystic carcinoma

    As mentioned previously, adenoid cystic carcinoma (ACC) can develop in a pleomorphic adenoma or, more commonly, arise de novo in the lacrimal gland. The tumor is slightly more common in women than in men, and the median age at presentation is about 40 years.

    Unlike pleomorphic adenoma, ACC has no pseudocapsule; it tends to erode bone and invade orbital nerves, accounting for the pain that is frequently reported by patients on presentation. The gross appearance is grayish white, firm, and nodular. Histologically, the tumor cells may grow in a variety of patterns: cribriform (“Swiss cheese”), which is the most common; basaloid (solid nests); comedo; sclerosing; and tubular (ductal) (Fig 14-8). Presence of the basaloid pattern has been associated with a worse prognosis (5-year survival rate of 20%) when compared with absence of a basaloid component (5-year survival rate of 70%). IHC staining for S-100 protein, keratin, and actin is typically positive within areas of myoepithelial differentiation. There is a correlation between expression of bcl-2 and BAX proteins and a more favorable prognosis. Expression of p53 is associated with a poor prognosis. Orbital exenteration is one of the currently accepted treatments for this tumor, but some advocate globe-sparing intra-arterial chemotherapy.

    Figure 14-7 Pleomorphic adenoma (benign mixed tumor) of the lacrimal gland. A, Clinical photograph. A superotemporal orbital mass is present, causing proptosis and downward displacement of the left globe. B, CT scan (coronal view) demonstrates the left orbit tumor. C, Low-magnification photomicrograph shows the circumscribed nature of this pleomorphic adenoma. D, Note both the neoplastic epithelial elements (arrow) and the fibromyxoid stroma (asterisk).E, Frequent well-differentiated glandular structures with lumina (asterisk) are seen. The outer myoepithelial layer can become metaplastic and form other mesenchymal tissue (eg, bone, cartilage [arrow]).

    (Parts A and B courtesy of Sander Dubovy, MD; parts C–E courtesy of Heather Potter, MD.)

    Figure 14-8 Adenoid cystic carcinoma of the lacrimal gland. A, Low-magnification photomicrograph shows numerous invasive tumor lobules. B, Note the characteristic cribriform (“Swiss cheese”) pattern of growth. C, The solid lobules of tumor represent the basaloid pattern, which is associated with a less favorable prognosis. D, Perineural invasion of tumor. Blue tumor invades the perineurium and surrounds a peripheral nerve (asterisk).

    (Part A courtesy of Heather Potter, MD; parts B–D courtesy of Nasreen A. Syed, MD.)

    • Ahmad SM, Esmaeli B, Williams M, et al. American Joint Committee on Cancer classification predicts outcome of patients with lacrimal gland adenoid cystic carcinoma. Ophthalmology. 2009;116(6):1210–1215.

    • von Holstein SL, Coupland SE, Briscoe D, Le Tourneau C, Heegaard S. Epithelial tumours of the lacrimal gland: a clinical, histopathological, surgical and oncological survey. Acta Ophthalmol. 2013;91(3):195–206.

    Excerpted from BCSC 2020-2021 series: Section 4 - Ophthalmic Pathology and Intraocular Tumors. For more information and to purchase the entire series, please visit https://www.aao.org/bcsc.

  • Most Commented
    Loading, please wait...
    There are no comments available.
    Most Viewed
    Loading, please wait...
    Most Viewed content is not available.
  • The Academy Store
    2021 North Carolina Codequest (Recording)
    2021 Tennessee Codequest (Recording)
    2021 Codequest (Recording)
    2021 Michigan Codequest (Recording)
    Focal Points 2020 Complete Set
    2021 Coding Coach: Complete Ophthalmic Coding Reference 
    2021 CPT: Complete Pocket Ophthalmic Reference
    2021 Retina Coding: Complete Reference Guide
    2021 Fundamentals of Ophthalmic Coding
    2021 CPT Professional Edition 
    2021 HCPCS Level ll Professional Edition
    2021 Coding Assistant: Cataract and Anterior Segment
    2021 Coding Assistant: Cornea
    2021 Coding Assistant: Glaucoma
    2021 Coding Assistant: Oculofacial
    2021 Coding Assistant: Pediatrics/Strabismus
    2021 Coding Assistant for Subspecialties 
    Recorded Webinar: 2021 Ophthalmology Coding Update
    Webinar: 2021 Ophthalmology Coding Update
    Ophthalmology Journal
    Ophthalmology Retina Journal
    Focal Points Member Benefit
    2021 ICD-10-CM for Ophthalmology: The Complete Reference 
    Conquering New E-M Documentation Guidelines for Ophthalmology
    2020-2021 Basic and Clinical Science Course Complete Set
    2020-2021 Basic and Clinical Science Course Residency Set
    2020-2021 Basic and Clinical Science Course, Section 01: Update on General Medicine
    2020-2021 Basic and Clinical Science Course, Section 02: Fundamentals and Principles of Ophthalmology
    2020-2021 Basic and Clinical Science Course, Section 03: Clinical Optics
    2020-2021 Basic and Clinical Science Course, Section 04: Ophthalmic Pathology and Intraocular Tumors
 
  • Contact Us
  • About the Academy
  • Jobs at the Academy
  • Financial Relationships with Industry
  • Medical Disclaimer
  • Privacy Policy
  • Terms of Service
  • Help
  • For Advertisers
  • For Media
  • Ophthalmology Job Center

OUR SITES

  • EyeWiki
  • International Society of Refractive Surgery

FOLLOW THE ACADEMY

Medical Professionals

  • Facebook
  • Twitter
  • LinkedIn
  • YouTube

Public & Patients

  • Facebook
  • Twitter
  • Instagram
  • YouTube

Museum of the Eye

  • Facebook
  • Twitter
  • Instagram
  • Trip Advisor
  • Yelp
© American Academy of Ophthalmology 2021