Vegetative plaque on the left superior eyelid

Area of Science:

• Dermatology
• Oncology
• Hematology

Background:

• Primary cutaneous anaplastic large cell lymphoma (pcALCL) is a rare CD30+ lymphoproliferative neoplasm.
• Distinguishing pcALCL from other periocular malignancies is crucial for appropriate management.

Observation:

• A 73-year-old female presented with a rapidly progressing, ulcerated, multinodular tumor on her left superior eyelid.
• Initial treatments for suspected infection yielded minimal improvement, necessitating further investigation.

Findings:

• Shave biopsy confirmed pcALCL, a diagnosis supported by the absence of extracutaneous involvement on PET-CT.
• Differential diagnoses considered included Merkel cell carcinoma, sebaceous carcinoma, lymphomatoid papulosis, and extranodal NK/T cell lymphoma.

Implications:

• Localized pcALCL is effectively treated with surgical excision achieving negative margins.
• Systemic therapy, such as brentuximab vedotin, is indicated for widespread, relapsed, or refractory pcALCL.
• Early diagnosis and appropriate treatment are key to favorable outcomes in pcALCL.