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AJNR - American Journal of Neuroradiology

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American Journal of Neuroradiology 24:1765-1768, October 2003
© 2003 American Society of Neuroradiology

Case Report
HEAD AND NECK

Mesectodermal Leiomyosarcoma of the Ciliary Body

Sun-Won Park^a, Hyung-Jin Kim^a, Hee-Seung Chin^b, Kee-Sun Tae^b and Jee-Young Han^c

^a Department of Radiology, Inha University Hospital, Incheon, Korea
^b Department of Opthalmology, Inha University Hospital, Incheon, Korea
^c Department of Pathology, Inha University Hospital, Incheon, Korea

Address correspondence to Dr. Hyung-Jin Kim, Inha University Hospital, Department of Radiology, 7–206, 3rd Street, Shinheung-dong, Joong-gu, Incheon 400–711, Korea

Summary: We report a case of low-grade mesectodermal leiomyosarcoma of the ciliary body, an extremely rare tumor of neural crest origin, occurring in a 12-year-old boy. MR imaging showed a well-marginated, ovoid soft tissue mass at the right temporal ciliary body, accompanied by total retinal detachment. The mass was slightly hyperintense relative to contralateral vitreous on T1-weighted images and markedly hypointense on T2-weighted images and enhanced very well. Initial biopsy of the mass suggested a peripheral nerve sheath tumor. The mass grew rapidly after biopsy, and enucleation was performed. The final diagnosis based on histology and immunohistochemistry was a low-grade mesectodermal leiomyosarcoma. To our knowledge, this is the first reported case of a malignant form of mesectodermal leiomyoma of the ciliary body.