Ophthalmic Surgery and Lasers

[About Ophthalmic Surgery and Lasers ] [Table of Contents]

Volume 30 (7) * July/August 1999 * Case Reports (abstract)

Orbital Leiomyosarcoma After Retinoblastoma

Kimberly A. Klippenstein, MD Ralph E. Wesley, MD Alan D. Glick, MD

ABSTRACT

Patients with the inherited, bilateral form of retinoblastoma have an increased incidence of osteogenic sarcoma such that the mortality from the secondary tumor exceeds that of the initial bilateral retinoblastoma. We report a 29-year-old male survivor of bilateral retinoblastomas originally diagnosed at 8 months of age, whose treatment eventually included bilateral enucleation, bilateral orbital radiation, and systemic chemotherapy. At age 26, a tumor removed from his right maxillary sinus was diagnosed as fibroma. At age 29, he developed an inferior orbital mass that extended into the right maxillary sinus. A biopsy and comparison with the previous maxillary sinus mass revealed both lesions to be leiomyosarcoma. Both light and electron microscpy supported the diagnosis. The patient has survived treatment with orbital exenteration and maxillectomy combined with postoperative radiation to the right orbital-maxillary area. This appears to be the fourth case of leiomyosarcoma in the third decade of life in a male patient with a previously irradiated orbit after enucleation for bilateral retinoblastoma. Leiomyosarcoma appears to be another orbital tumor associated with bilateral retinoblastoma. [Ophthalmic Surg Lasers 1999;30:579-583.]

AUTHORS

From the Ophthalmic Plastic and Orbital Service, Department of Ophthalmology (Drs Klippenstein and Wesley) and the Department of Pathology (Dr Glick), Vanderbilt University Medical Center, Nashville, Tennessee.

Accepted for publication April 5, 1999.

Supported in part by a grant from Research to Prevent Blindness, Inc.

Address reprint requests to Ralph E. Wesley, MD, Orbital and Oculoplastic Center, Suite 216, The Atrium, 250 25th Avenue North, Nashville, TN 37203.

* About SLACK Inc.
* Go to ocular resources.
* Go to Ophthalmic Surgery and Lasers.
* Current issue contents.
* Try out a free issue.
* Take out a subscription.

* Copyright 1999, SLACK Incorporated. Revised 17 August 1999.
* Get notification of major content changes.
* Read the instructions to contributors.
* Send a letter to the editor.
* Comment to Webmaster, SLACK Inc.