Case Study: July 2015

History

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.

Questions:

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?

 

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1. Neoplasm composed of spindle and epithelioid cells.

2. Extraocular spread uveal melanoma, conjunctival melanoma, spindle cell carcinoma, sarcoma (various types), metastasis.

3. HHV-8

4. Kaposi sarcoma of the orbit. KS can occur very rarely in CVID scenarios.