Sir,

We report a case of xanthomatous tumor presenting as uveitis, which improved after surgery and immunosuppressive treatment.

Case report

A 52-year-old male with normal serum lipoproteins has blurred vision and dull pain for 5 months OS. His best-corrected visual acuity (BCVA) was 6/600. Hypopyon and posterior synechiae occurred. Intravenous methylprednisolone was given. After posterior synechiae was lysed, a retrolental mass was found and confirmed by magnetic resonance imaging (Figure 1). Phacoemulsification, external transscleral tumor resection and partial lamellar cyclectomy were performed. GEN-PROBE amplified Mycobacterium Tuberculosis direct test and Mantoux test were negative. The pathology (Figure 2) showed negative staining of synaptophysin and chromogranin-A, which excluded paraganglioma. The negative staining of S-100 protein and melan-A eliminated melanocytic lineage. The negative staining of melan-A and desmin precluded perivascular epithelioid cell tumor. Granular cell tumor was unlikely due to the absence of typical granular eosinophilic cytoplasms and negative S-100 protein staining. The positive staining of CD68 confirmed its histiocytic lineage. Differential diagnosis included xanthoma,1 juvenile xanthogranuloma,2 reticulohistiocytoma,3 Malakoplakia or Rosai–Dorfman disease.4 No specific pathogen is identified under periodic Acid–Schiff and acid-fast stains. A xanthomatous tumor was diagnosed. After treatment, his BCVA maintained 6/6.

Figure 1
figure 1

After posterior synechiae was lysed, a retrolental mass was found. A lobulated lesion about 7 mm in length behind the iris and lens was confirmed by magnetic resonance imaging.

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Figure 2
figure 2

(a) The hematoxylin and eosin stain. (b) The negative staining of desmin. (c) The negative staining of synaptophysin. (d) The negative staining of chromogranin-A. (e) The negative staining of S-100 protein. (f) The negative staining of melan-A. (g) The negative staining of cytokeratin. (h) The positive staining of CD68.

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Comment

Xanthomas are usually related to abnormal metabolism of lipids. Histology of xanthoma showed a diffuse proliferation of foamy histiocytes with occasional Tuton giant cell.1 The reported non-malignant tumors causing uveitis include juvenile xanthogranuloma2 and adenoma of the ciliary body.5 Juvenile xanthogranuloma is a benign non-Langerhans cell histiocytic proliferation of skin and soft tissue.2 It can be differentiated from xanthoma by the distribution of lesion and the absence of lipid abnormalities. Reticulohistiocytoma usually presents as a firm, skin colored, yellowish or reddish papule or nodule.3 Malakoplakia is an inflammatory condition presenting as a plaque or a nodule that usually affects the genitourinary tract. Rosai–Dorfman disease is an idiopathic non-neoplastic histioproliferative disease characterized clinically by massive painless cervical lymphadenopathy.4 The pathological diagnosis of our case rendered as xanthomatous tumor because no features of the above mentioned diseases were presented.

This is the first case of xanthomatous tumor presenting as uveitis. Radiologically and clinically, xanthomatous tumor can mimic ciliary body tumor and cause inflammation.