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Anaplastic Large Cell Lymphoma With Intraocular or Ocular Adnexal Involvement: A Case Report and Systematic Review
Seyed Mohsen Rafizadeh1, Hossein Ghahvehchian1,2, Mohammad Taher Rajabi1
1Department of Oculofacial Plastic and Orbital Surgery, Farabi Comprehensive Center of Excellence in Ophthalmology, Tehran University of Medical Sciences, Tehran, Iran.
Ophthalmic Plastic and Reconstructive Surgery
|September 18, 2023
Summary
Anaplastic large cell lymphoma (ALCL) affecting the eye is rare and aggressive. Early biopsy and chemotherapy are crucial for managing this malignant tumor, though outcomes remain poor for many patients.
Area of Science:
- Ophthalmology
- Oncology
- Pathology
Background:
- Anaplastic large cell lymphoma (ALCL) is a rare, aggressive non-Hodgkin lymphoma.
- Ocular involvement, including the globe and adnexa, presents unique diagnostic and therapeutic challenges.
Purpose of the Study:
- To conduct a literature review of ALCL affecting the ocular structures.
- To report a case of ALCL involving the globe and ocular adnexa.
Main Methods:
- Systematic literature search of PubMed, Scopus, and Google Scholar (inception to May 2023).
- Inclusion of all reported cases of ALCL involving intraocular or adnexal ocular structures.
- Addition of a newly reported case to the reviewed data.
Main Results:
- Nine patients with ocular ALCL were analyzed (mean age 29.7 years).
- Common manifestations included periorbital swelling, chemosis, and decreased vision; 3 patients experienced misdiagnosis.
- CD30 expression was universal; ALK positivity was noted in 6/9 patients. Chemotherapy and radiation were common treatments, with a 55.6% mortality rate.
Conclusions:
- Ocular ALCL is a rare, highly malignant tumor often mimicking benign conditions.
- Prompt diagnosis via biopsy and aggressive multimodal treatment, including chemotherapy (e.g., CHOP) and radiation, are recommended.
- Despite treatment, the prognosis for ocular ALCL remains guarded.
