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Uncorrectable ptosis: primary cutaneous signet-ring cell carcinoma
Mark S Hansen1, Sulene L Chi, Thomas Cummings
1Duke University.
Dermatology Online Journal
|September 21, 2013
Summary
Primary cutaneous signet-ring cell carcinoma (PCSRCC) is a rare, aggressive eyelid tumor. Delayed diagnosis is common, emphasizing the need for early recognition of subtle signs like ptosis for better outcomes.
Area of Science:
- Oncology
- Ophthalmology
- Dermatology
Background:
- Primary cutaneous signet-ring cell carcinoma (PCSRCC) is an uncommon and aggressive malignancy.
- Eyelid involvement is the most frequent presentation of PCSRCC.
Observation:
- A 60-year-old male presented with eyelid ptosis, initially managed with multiple surgeries over three years.
- Progressive enophthalmos and poor surgical outcomes prompted further investigation.
- CT scan and eyelid biopsy confirmed PCSRCC, with subsequent MRI revealing extensive orbital invasion.
Findings:
- Histopathology confirmed PCSRCC with widespread orbital extension.
- Exenteration was performed to manage the advanced disease and reduce metastasis risk.
- Literature review indicates 27 of 33 PCSRCC cases involve the eyelids.
Implications:
- PCSRCC, though rare, presents aggressively with typically delayed diagnosis.
- Recognizing subtle clinical signs, such as ptosis, is crucial for early detection.
- Prompt diagnosis and treatment are vital for improving prognosis in PCSRCC.
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