A 35 year old female with combined variable immunodeficiency (CVID) presents with a slowly growing anterior orbital mass, with a visible lump present under the upper lid. This is biopsied and the figures illustrate the salient histopathological features.
1. What do figures 1 and 2 show?
2. What is the histological differential diagnosis?
3. Which immunohistochemical stain has been used in Fig 3?
4. What’s the diagnosis?
1. Neoplasm composed of spindle and epithelioid cells.
2. Extraocular spread uveal melanoma, conjunctival melanoma, spindle cell carcinoma, sarcoma (various types), metastasis.
4. Kaposi sarcoma of the orbit. KS can occur very rarely in CVID scenarios.