Periorbital Merkel Cell Carcinoma: Management, outcomes and the role of immune checkpoint inhibitors
Author: Li Teng Kok
Base Hospital / Institution: Moorfields Eye Hospital
ePoster presentation
Abstract ID: 25-182
Purpose
Merkel cell carcinoma (MCC) is a rare neuroendocrine tumour associated with aggressive disease. This case series describes the clinical presentations, management and outcomes of patients with periorbital MCC. We also describe the use of immune checkpoint inhibitors as treatment in three patients.
Methods
A retrospective review of primary periorbital MCC cases from a tertiary referral centre between 2013 and 2023 was conducted. Clinical presentation, management and outcomes were analysed.
Results
Nine patients with primary periorbital MCC presented between 2013 to 2023. The median age of presentation was 83 years (range 61-95) and median tumour size was 18.5mm (range 6-30mm). TNM staging ranged from T2b N0 M0 to T4 N1 M0. Five (56%) presented with lymph node involvement and none with distant metastases. Eight (89%) underwent wide local excision (WLE) with 5-10 mm margin control. Five (56%) underwent adjuvant radiotherapy and one (11%) received chemotherapy. Avelumab was initiated in three (33%) as treatment in primary metastatic tumours (n=2) and in refractory metastatic disease (n=1) with a median progression free survival of 30.1 months. Progression and recurrence occurred in 33% with median time to recurrence of 37.5 months. All-cause mortality was 33% and MCC disease-specific mortality was 11% occurring at 15 months. Five (56%) patients are disease free at last median follow-up of 44 months.
Conclusion
Periorbital MCC is rare but aggressive and usually presents late with a large tumour size. WLE is the mainstay treatment with 78% receiving adjuvant treatment in the form of radiotherapy, immunotherapy and chemotherapy. Immune checkpoint inhibitors have an important role as periorbital surgical excision is often challenging. It is a suitable life-extending treatment option for metastatic periorbital MCC.
Additional Authors
| First name | Last name | Base Hospital / Institution |
|---|---|---|
| Allan | Nghiem | Moorfields Eye Hospital |
| Jimmy | Uddin | Moorfields Eye Hospital |
| Tarang | Gupta | Moorfields Eye Hospital |
| Clare | Daniel | Moorfields Eye Hospital |
