Conjunctival Perivascular Epithelioid Cell Neoplasm With RBM10-TFE3 Fusion Presenting as Recurrent Subconjunctival...

Subconjunctival hemorrhages commonly present to eye care professionals and are frequently regarded as benign self-limited conditions. In selected cases, subconjunctival hemorrhages can be a harbinger of more severe disease. Perivascular epithelioid cell tumors, or PEComas, are rare mesenchymal neoplasms believed to originate from perivascular myoid cells and are rarely present in ocular structures. We present a rare case of a conjunctival perivascular epithelioid cell tumor that initially presented with recurrent subconjunctival hemorrhage. To our knowledge, this is the first description of a PEComa with a RBM10-TFE3 gene fusion, only previously seen with renal cell carcinoma. Physicians should be aware of this rare condition, its location in the fornix and its presentation as a recurrent subconjunctival hemorrhage.