Primary Signet Ring Cell/Histiocytoid Carcinoma of the Eyelid : Two Cases of Subclinical Conjunctival Involvement and a Review of the Literature
Author: Steffie Arès
Base Hospital / Institution: Hôpital Maisonneuve-Rosemont, Université de Montréal, Montreal, Quebec, Canada
ePoster presentation
Abstract ID: 25-447
Purpose
To review the epidemiology, clinical presentation, treatment, and outcomes of two novel cases of primary signet ring cell/histiocytoid carcinomas (PSRCHC) of the eyelid, as well as previously reported cases. The authors emphasize the critical role of conjunctival and cutaneous mapping biopsies in assessing tumoral spread and guiding management.
Methods
A systematic literature review of PubMed and Medline captured all well documented cases of PSRCHC.
Results
A total of 59 cases of PSRCHC of the eyelid were included (90% male, median age 72). All cases presented with primary eyelid involvement, with orbital involvement in 61% and metastasis eventually occurring in 48% of cases. Most common primary treatments included exenteration (16%) or exenteration with radiotherapy (28%), and local excision (20%) or excision with radiotherapy (10%). Over their clinical course, 57% required exenteration, with exenteration alone having the lowest recurrence rates (25%), followed by exenteration with radiotherapy (43%). Secondary recurrence or progression occurred in 54% of cases. At final follow-up, 50% had achieved disease-free status while disease-related mortality occurred in three cases (6%).
Of the 18 cases commenting on conjunctival involvement, 67% demonstrated clinical or pathological spread. Conjunctival involvement was associated with poorer outcomes, with 75% of those affected developing orbital involvement.
The authors are the first to report initial subclinical conjunctival invasion of PSRCHC and the critical role of conjunctival mapping biopsies in its management. In both novel cases, conjunctival and cutaneous mapping revealed extensive subclinical spread of seemingly normal tissue, leading to exenteration with no recurrence over 3 years.
Conclusion
PSRCHC of the eyelid are rare and aggressive tumors with high rates of orbital involvement, recurrence, and metastasis. Pre-operative conjunctival mapping biopsies are essential for accurate staging and surgical planning. Primary orbital exenteration may be a useful first-line therapy, especially in cases with conjunctival involvement.
Additional Authors
| First name | Last name | Base Hospital / Institution |
|---|---|---|
| Anne | Xuan-Lan Nguyen | Toronto Western Hospital, University of Toronto, Toronto, Ontario, Canada |
| Victoria | Leung | Michael Garron Hospital, Toronto, Ontario, Canada |
| Marie-Jo | Olivier | Hôpital Maisonneuve Rosemont, Université de Montréal, Montreal, Quebec, Canada |
| Patrick | Boulos | Hôpital Maisonneuve Rosemont, Université de Montréal, Montreal, Quebec, Canada |
| Isabelle | Hardy | Hôpital Maisonneuve Rosemont, Université de Montréal, Montreal, Quebec, Canada |
