Fall 2017 Newsletter

Follow our latest updates in our Fall 2017 Newsletter

Fall 2017 Newsletter
7th Residency Announcement

Exciting Residency Announcement!

ACGME approves seventh resident complement; first increase in over 30 years!

Grand Rounds

Grand Rounds and Case Studies

Check out our weekly presentations

OTEP

OTEP

Ophthalmic Technician Education Program

LARGEST STUDY ON CHINESE AMERICANS PUBLISHED

LARGEST STUDY ON CHINESE
AMERICANS PUBLISHED

USC Ophthalmology Researchers Find More
Effective Treatments For Blinding Eye Diseases

EDUCATION

Case Study: Hello Jell-O

Mustafi Berry
Presenter: Debarshi Mustafi, MD, PhD Discussant: Jesse L. Berry, MD
 

History

  • 73-year-old male presented to urgent care with a few day history of foreign body sensation in his right eye
  • Previous ocular history remarkable for “no-touch technique” excision with cryotherapy OD and exenteration OS one year prior to current presentation for squamous cell carcinoma invasive to the orbit OS

Exam Findings

Figure 1
Figure 1: (A) New gelatinous lesion OD at nasal limbus with intrinsic vascularity, (B) Previous exam one year prior showed white and quiet sclera.

 

Differential Diagnosis

  • Benign Lesions
    • Papilloma
    • Pseudoepitheliomatous hyperplasia
    • Benign hereditary intraepithelial dyskeratosis
  • Pre-Invasive Lesions
    • Conjunctival and corneal intraepithelial neoplasia (CIN)
      • Graded as mild, moderate or severe based on thickness involvement (partial vs full)
Figure 1
See above

 

  • Malignant Lesions
    • Squamous cell carcinoma
    • Mucoepidermoid carcinoma

Additional Investigations

  • Excisional biopsies at initial visits, during exenteration and at recurrence one year later
Figure 2
Figure 2: Biopsy of lesion at initial presentation revealing keratinization, hyperplasia, nuclear hyperchromasia and pleomorphism, altered cell polarity with full thickness involvement and areas of micro-invasion.

 

Figure 3
Figure 3: Histopathological slides following exenteration show involvement of the globe with invasion of instrascleral vascular channel, Schlemm’s canal/trabecular meshwork and with focal involvement of ciliary body and choroid as shown in high resolution in slide B. Of note: mucicarmine and alcian blue stains were negative.

 

Figure 4
Figure 4: Histopathological slide of the recurrent lesion at one year revealed
dysplastic epithelium with increased cellularity and nuclear hyperchromasia.

 

Diagnosis

  • Ocular surface squamous neoplasia (OSSN), which is an encompassing term for precancerous and cancerous epithelial lesions of the conjunctiva and cornea. It includes the spectrum of dysplasia, CIN and invasive squamous cell carcinoma.
  • Staging is dependent on biopsy as outlined by the 8th edition of the American Joint Committee on Cancer
Figure 4
T criteria key

 

Pathophysiology

  • Factors associated with the development of OSSN are:
    • Exposure to sunlight
    • HPV type 16 infections
    • Immunocompromised status
    • Systemic associations of the development of OSSN include xeroderma pigmentosum
  • The nasal limbal area may be particularly vulnerable to solar UV radiation and has a high concentration of stem cells in the basal epithelium
Figure 5
Figure 5: DNA damage from UV radiation may trigger disease in individuals with reduced DNA repair mechanisms in place or disruption or tumor suppressor activity (p53).

 

Treatment

  • Paradigms as detailed below consist of drop therapy vs surgical excision
Figure 2
Drop therapy vs surgical excision

 

Prognosis and Future Directions

  • Patient “no-touch technique” dissection with cryotherapy of new lesion of the right eye
  • POD1 BCVA 20/40 OD with sclera and conjunctiva amniotic membrane in place with vicryl sutures over conjunctival excision
  • Mutant TTN could be a potential prognostic biomarker for OSSN response to IFNα-2b treatment
  • The advent of next generation sequencing has started to provide insight into the genetic players that may drive treatment paradigms:
Figure 6
Figure 6: The four samples carrying TTN mutations (100%) had previously failed treatment with IFNα-2b. The other three tumors, which were not found to carry TTN mutations, had been excised primarily.

 

References

  • Usai Y, et al.Bilateral ocular surface squamous neoplasia: a clinicopathological case report. Br J Ophthalmol. 2004; 88:595-596.
  • Giuchuhi S, et al. Pathophysiology of ocular surface squamous neoplasia. Exp Eye Res. 2014 Dec;129:172-182.
  • Lee GA, Hirst LW.Ocular surface squamous neoplasia. Surv Ophthalmol. 1995 May-Jun;39(6): 429-450.
  • Shields CL, Shields JA.Tumors of the conjunctiva and cornea. Surv Ophthalmol. 2004 Jan-Feb; 49(1): 3-24.
  • Honavar SG, et al.Tumors of the ocular surface: A review. Indian J Ophthalmol. 2015;63:187-203.
  • Galor A, et al. Whole exome profiling of ocular surface squamous neoplasia. Ophthalmology. 2016 Jan;123(1):216-217.

Contact

Section Editors

 

Produced by: Monica Chavez, John Daniel, Joseph Yim and Dr. Vivek Patel
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