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Vegetative plaque on the left superior eyelid.

Colin G Wikholm1, Sach Thakker2, Pegah R Bakhshi2

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|October 6, 2023
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Summary

A rapidly growing eyelid lesion was diagnosed as primary cutaneous anaplastic large cell lymphoma (pcALCL), a rare CD30+ lymphoproliferative disorder. This indolent lymphoma has a good prognosis with localized surgical excision or systemic treatment for advanced disease.

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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.