Dermatology Online Journal

Primary cutaneous signet-ring cell carcinoma (PCSRCC) is a rare but aggressive tumor. Our case highlights a 60-year-old man who presented with eyelid ptosis, for which he underwent multiple surgical procedures over a 3-year period prior to referral to our clinic. These procedures were complicated by scarring, delayed healing, and poor cosmetic outcome.  In addition, the patient was noted to develop progressive enophthalmos.  These concerning signs led to a CT scan and subsequent eyelid biopsy, which revealed a diagnosis of PCSRCC.  Further management has involved an MRI and orbitotomy with biopsy revealing widespread extension of the carcinoma.  Exenteration was performed to reduce the likelihood of metastasis.

There are few documented case reports of PCSRCC of the eyelid in the literature.  Of the 33 published cases of PCSRCC, 27 cases involve the eyelids and the other 6 cases involve the axilla. The unique clinical features of this case will be discussed, in particular the presentation as ptosis, an otherwise commonplace complaint in the oculoplastics clinic.  The surgical course and histopathologic findings will be presented.  The literature regarding PCSRCC will be reviewed including demographics, management, and prognosis. Although rare, PCSRCC follows an aggressive course with characteristically delayed diagnosis.  Early identification and treatment likely offer a better prognosis.  Thus, description of the clinical presentation of this rare tumor may aid in recognition and earlier treatment.