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Dermatology

Cutaneous Squamous Cell Carcinoma With Orbital Extension

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.

1 Credit CME

JAMA Ophthalmology

Published Online: October 6, 2022

JAMA Ophthalmology Clinical Challenge

Article Information and Disclosures

Kathryn S. Park, BA¹;

Theresa Guo, MD²;

Catherine Y. Liu, MD, PhD¹

Author Affiliations

¹Division of Oculofacial Plastic and Reconstructive Surgery, UC San Diego Viterbi Family Department of Ophthalmology, La Jolla, California
²Division of Otolaryngology-Head and Neck Surgery, Department of Surgery, University of California, San Diego

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A 72-year-old homeless man was referred for management of a recurrent cutaneous squamous cell carcinoma (SCC) of the right temple and brow. On review of history, maxillofacial magnetic resonance imaging (MRI) with and without contrast done 5 months prior to presentation showed a 5.9 × 5.3 × 3.7-cm mass centered at the right temple that tracked along the right lateral orbital wall as well as an enhancing right intraparotid lymph node. The patient was lost to follow-up and re-presented 5 months later with an enlarged crusting and ulcerating lesion of the right temple/brow (Figure 1A). On ocular examination, best-corrected visual acuity was 20/100 OD and 20/125 OS due to cataracts, pupils reacted normally without afferent pupillary defect, and ocular motility was full. He had mild proptosis in the right eye and right lower eyelid retraction. Orbicularis strength was intact and symmetric in both eyes. MRI of the orbits, face, and neck with and without contrast showed a 6.1 × 5.7 × 4.0-cm mass with erosion through the right sphenoid wing, involvement of the lacrimal gland, and abutment of the lateral rectus (Figure 1B). In discussing treatment strategies for the orbital component of the disease, the patient was strongly against an orbital exenteration.

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Stage T3N1M0 recurrent cutaneous SCC of the face

B. Neoadjuvant cemiplimab immunotherapy (anti–PD-L1 antibody), globe sparing orbitotomy, and surgical excision of the mass

Advanced cutaneous SCC with orbital involvement has historically been difficult to treat with 10-year survival rates less than 20%.¹ The standard treatment is exenteration, due to difficulty in obtaining clear margins in the orbit. Exenteration has been shown to provide better local control with clear margins obtained in 42.5% to 97% of cases and lower rates of local recurrence with overall survival of 83% and 65% at 1 and 5 years, respectively.² However, recently, the overall survival benefit of exenteration vs conservative surgeries has been questioned.²^- 4 In addition, exenteration causes facial disfigurement, loss of vision, psychological distress, and some, like this patient, may decline this surgery.

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Article Information

Corresponding Author: Catherine Y. Liu, MD, PhD, Division of Oculofacial Plastic and Reconstructive Surgery, UC San Diego Viterbi Family Department of Ophthalmology, 9415 Campus Point Dr, La Jolla, CA 92037 (yul107@health.ucsd.edu).

Published Online: October 6, 2022. doi:10.1001/jamaophthalmol.2022.3919

Conflict of Interest Disclosures: Dr Liu reported grant support from Horizon Therapeutics and royalties from Wolter Kluwers Health. No other disclosures were reported.

Additional Contributions: We thank the patient for granting permission to publish this information.

References

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Martel A , Hamedani M , Lagier J , Bertolotto C , Gastaud L , Poissonnet G . Does orbital exenteration still have a place in 2019? J Fr Ophtalmol. 2020;43(2):152-174. In French. doi:10.1016/j.jfo.2019.04.021 PubMed Google Scholar

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Goldfarb JA , Ferrarotto R , Gross N , et al. Immune checkpoint inhibitors for treatment of periorbital squamous cell carcinoma. Br J Ophthalmol. 2021;bjophthalmol-2021-319417. doi:10.1136/bjophthalmol-2021-319417 PubMed Google Scholar Crossref

Gerring RC , Ott CT , Curry JM , Sargi ZB , Wester ST . Orbital exenteration for advanced periorbital non-melanoma skin cancer: prognostic factors and survival. Eye (Lond). 2017;31(3):379-388. doi:10.1038/eye.2016.218 PubMed Google Scholar Crossref

Migden MR , Rischin D , Schmults CD , et al. PD-1 blockade with cemiplimab in advanced cutaneous squamous-cell carcinoma. N Engl J Med. 2018;379(4):341-351. doi:10.1056/NEJMoa1805131 PubMed Google Scholar Crossref

Migden MR , Khushalani NI , Chang ALS , et al. Cemiplimab in locally advanced cutaneous squamous cell carcinoma: results from an open-label, phase 2, single-arm trial. Lancet Oncol. 2020;21(2):294-305. doi:10.1016/S1470-2045(19)30728-4 PubMed Google Scholar Crossref

Argenziano G , Fargnoli MC , Fantini F , et al. Identifying candidates for immunotherapy with cemiplimab to treat advanced cutaneous squamous cell carcinoma: an expert opinion. Ther Adv Med Oncol. 2022;14:17588359211066272. doi:10.1177/17588359211066272 PubMed Google Scholar Crossref

Gross N , Ferrarotto R , Nagarajan P , et al. LBA74—phase II study of neoadjuvant cemiplimab prior to surgery in patients with stage III/IV (M0) cutaneous squamous cell carcinoma of the head and neck (CSCC-HN). Ann Oncol. 2019;30(5):v910. doi:10.1093/annonc/mdz394.071 Google Scholar Crossref

Chik JYK , Leung CWL , Wong KH . Palliative radiation therapy for patients with orbital and ocular metastases. Ann Palliat Med. 2020;9(6):4458-4466. doi:10.21037/apm.2019.12.02 PubMed Google Scholar Crossref

AMA CME Accreditation Information

Credit Designation Statement: The American Medical Association designates this Journal-based CME activity activity for a maximum of 1.00  AMA PRA Category 1 Credit(s)™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.

Successful completion of this CME activity, which includes participation in the evaluation component, enables the participant to earn up to:

1.00 Medical Knowledge MOC points in the American Board of Internal Medicine's (ABIM) Maintenance of Certification (MOC) program;;
1.00 Self-Assessment points in the American Board of Otolaryngology – Head and Neck Surgery’s (ABOHNS) Continuing Certification program;
1.00 MOC points in the American Board of Pediatrics’ (ABP) Maintenance of Certification (MOC) program;
1.00 Lifelong Learning points in the American Board of Pathology’s (ABPath) Continuing Certification program; and
1.00 CME points in the American Board of Surgery’s (ABS) Continuing Certification program

It is the CME activity provider's responsibility to submit participant completion information to ACCME for the purpose of granting MOC credit.