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Deborah Y. Chong, MD; Hakan Demirci, MD; Shawn M. Ronan, MD; Andrew Flint, MD; Victor M. Elner, MD, PhD

Arch Ophthalmol. 2007;125(4):566-569.

	Since this article does not have an abstract, we have provided the first 150 words of the full text and any section headings.

Rhabdomyosarcoma is the most common malignant orbital neoplasm in children. Li-Fraumeni syndrome (LFS) is an autosomal dominant disorder often resulting from germline mutations impairing the antitumor functions of p53.^1-2 Li-Fraumeni syndrome is characterized by a marked increase in familial disposition to cancers, including rhabdomyosarcoma. We report, to our knowledge, the first clinicopathologic description of orbital rhabdomyosarcoma in LFS in the ophthalmic literature.

Report of a Case

A 23-month-old boy developed progressive left proptosis over 4 days. His medical history was unremarkable, but his family history was positive for cancer in multiple generations. His mother developed Paget disease of the breast at age 31 years, a maternal uncle died of an unknown cancer at age 12 years, a maternal grandmother had bilateral breast cancer at ages 20 and 31 years, and a maternal great-grandmother also had . . . [Full Text of this Article]

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