Ocular perivascular epithelioid cell tumor: report of 2 cases with distinct clinical presentations

Furusato, Emiko; Cameron, J. Douglas; Newsom, Roger W.; Fujishiro, Takashi; Kojima, Takayoshi; Specht, Charles S.; Fetsch, John F.; Furusato, Bungo; Sesterhenn, Isabell A.; Rushing, Elisabeth J.

doi:10.1016/j.humpath.2009.12.006

« PreviousHuman Pathology
Volume 41, Issue 5 , Pages 768-772, May 2010

Ocular perivascular epithelioid cell tumor: report of 2 cases with distinct clinical presentations

Emiko Furusato, MD
- Department of Neuropathology and Ophthalmic Pathology, Armed Forces Institute of Pathology, Washington, DC 20306-6000, USA
J. Douglas Cameron, MD
- Department of Neuropathology and Ophthalmic Pathology, Armed Forces Institute of Pathology, Washington, DC 20306-6000, USA
Roger W. Newsom, MD
- Tidewater Eye Centers, Portsmouth, VA 23707, USA
Takashi Fujishiro, MD
- Department of Ophthalmology, Saitama Red Cross Hospital, 338-8553, Japan
Takayoshi Kojima, MD, PhD
- Department of Ophthalmology, Saitama Red Cross Hospital, 338-8553, Japan
Charles S. Specht, MD
- Department of Pathology, Ophthalmology, and Neurosurgery, Penn State Hershey, Hershey, PA 17033, USA
John F. Fetsch, MD
- Department of Soft Tissue Pathology, Armed Forces Institute of Pathology, Washington, DC 20306-6000, USA
Bungo Furusato, MD
- Department of Genitourinary Pathology, Armed Forces Institute of Pathology, Washington, DC 20306-6000, USA
Isabell A. Sesterhenn, MD
- Department of Genitourinary Pathology, Armed Forces Institute of Pathology, Washington, DC 20306-6000, USA
Elisabeth J. Rushing, MD
- Department of Neuropathology and Ophthalmic Pathology, Armed Forces Institute of Pathology, Washington, DC 20306-6000, USA
- Corresponding author.

Received 20 October 2009; received in revised form 17 December 2009; accepted 18 December 2009. published online 18 March 2010.

Summary

Perivascular epithelioid cell tumors comprise a rare and recently described family of neoplasms that characteristically coexpress melanocytic and myoid markers. We describe the clinicopathologic features of 2 ocular cases. Case 1 occurred in a 26-year-old woman with a recurrent left upper eyelid tumor, and case 2 was diagnosed in a 7-year-old boy with a left ciliary body mass. This is the first report of perivascular epithelioid cell tumor arising in the ciliary body or eyelid. Neither patient in our series had documented evidence of the tuberous sclerosis complex. Despite its rarity, perivascular epithelioid cell tumor should be considered in the differential diagnosis of ocular melanocytic lesions. Although most examples appear cytologically bland, experience is limited regarding their malignant potential; and therefore, complete surgical resection and close follow-up are recommended.

Keywords: Ciliary body, Eyelid, Immunohistochemistry, Perivascular epithelioid cell tumor