Orbital Perivascular Epithelioid Cell Tumor in a Case of Tuberous Sclerosis.

ABSTRACT: The aim of the study was to report a case of orbital perivascular epithelioid cell tumor (PEComa) in a known diagnosed patient of tuberous sclerosis and retinal astrocytic hamartoma. 43-year-old female presented with rapid progressive painful proptosis in the left eye, also reported new mass growing in her upper back. The patient past medical history is significant for left renal angiomyolipoma and multiple bilateral lung cysts of which she underwent right nephrectomy and lung biopsy, respectively. The lung biopsy turned diagnostic for lymphangiomyomatosis. On external examination, the left eye was grossly proptotic with hypoglobus. A typical butterfly distribution of sebaceous adenoma was noted across the patient cheeks and nose. Visual acuity in the right eye was 20/20 and the left eye,

SUBMITTER: Aljneibi SH 

PROVIDER: S-EPMC10359681 | biostudies-literature | 2023 Jan-Dec

REPOSITORIES: biostudies-literature