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Orbital leiomyosarcoma after retinoblastoma.
K A Klippenstein1, R E Wesley, A D Glick
1Department of Ophthalmology, Vanderbilt University Medical Center, Nashville, Tennessee, USA.
Ophthalmic Surgery and Lasers
|August 10, 2000
Summary
Survivors of bilateral retinoblastoma (RB) treated with radiation have an increased risk of secondary cancers. This case highlights leiomyosarcoma as a potential orbital tumor in these patients.
Area of Science:
- Oncology
- Ophthalmology
- Pathology
Background:
- Bilateral retinoblastoma (RB) survivors treated with enucleation and radiation face elevated risks of secondary malignancies, including osteogenic sarcoma.
- The long-term oncologic outcomes for bilateral RB survivors require continued investigation.
Observation:
- A 29-year-old male with a history of bilateral RB, treated with enucleation, orbital radiation, and chemotherapy, developed recurrent tumors.
- Initial diagnosis of a maxillary sinus tumor was fibroma; however, a subsequent orbital mass extending into the sinus was diagnosed as leiomyosarcoma.
Findings:
- Histopathological analysis confirmed both the fibroma and the subsequent mass as leiomyosarcoma.
- This represents the fourth reported case of leiomyosarcoma in a male patient in his third decade with a history of treated bilateral RB and orbital radiation.
Implications:
- Leiomyosarcoma may be an underrecognized secondary malignancy associated with bilateral retinoblastoma treatment.
- This case underscores the importance of long-term surveillance for secondary cancers in bilateral RB survivors.