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Transscleral resection of a ciliary body leiomyoma in a child: case report and review of the literature.
Graefes Arch Clin Exp Ophthalmol 2003; 241(11):953-7GA

Abstract

PURPOSE

To present the case of a patient with leiomyoma of the ciliary body and discuss the histological features and treatment of this rare intraocular tumor. METHODS/CASE REPORT: A 13-year-old boy presented with an asymptomatic tumor of the right eye. Visual acuity was 20/20 in both eyes. Ophthalmoscopy revealed an amelanotic, vascularized ciliary body tumor with exudative retinal detachment and partial transillumination. On ultrasound examination the tumor height was 8 mm and a low internal reflectivity was found. T2-weighted MRI scans showed a hypointense and T1-weighted scans a hyperintense intraocular mass with significant Gd-TPA enhancement. On the assumption that the diagnosis was consistent with an amelanotic ciliary body melanoma, a transscleral resection with adjuvant ruthenium-106 brachytherapy was performed. Visual acuity was 20/40 at 6 months after the operation.

RESULTS

Routine stains revealed a pleomorphic tumor composed mainly of spindle cells with palisading in some areas and a prominent intercellular fibrillary background. Immunohistochemistry showed positivity for desmin, vimentin and actin. No reactivity with S-100 and HMB-45 was seen. Intracytoplasmatic filaments and micropinocytotic vesicles were detected by transmission electron microscopy. These findings were consistent with the diagnosis of a ciliary body leiomyoma.

CONCLUSION

Typical clinical features of leiomyoma include a dome-shaped configuration and translucency, but the final diagnosis can only be confirmed by histology with the aid of immunohistochemistry and electron microscopy. Though rare, leiomyoma should be considered in the differential diagnosis of amelanotic uveal tumors. Transscleral resection is the treatment of choice of anterior uveal leiomyomas, with a fairly good visual prognosis.

Authors+Show Affiliations

Augenklinik, Universitätsklinikum Benjamin Franklin, Freie Universität Berlin, Hindenburgdamm 30, 12200, Berlin, Germany. miriam.richter@medizin.fu-berlin.deNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Review

Language

eng

PubMed ID

14595565

Citation

Richter, Miriam N., et al. "Transscleral Resection of a Ciliary Body Leiomyoma in a Child: Case Report and Review of the Literature." Graefe's Archive for Clinical and Experimental Ophthalmology = Albrecht Von Graefes Archiv Fur Klinische Und Experimentelle Ophthalmologie, vol. 241, no. 11, 2003, pp. 953-7.
Richter MN, Bechrakis NE, Stoltenburg-Didinger G, et al. Transscleral resection of a ciliary body leiomyoma in a child: case report and review of the literature. Graefes Arch Clin Exp Ophthalmol. 2003;241(11):953-7.
Richter, M. N., Bechrakis, N. E., Stoltenburg-Didinger, G., & Foerster, M. H. (2003). Transscleral resection of a ciliary body leiomyoma in a child: case report and review of the literature. Graefe's Archive for Clinical and Experimental Ophthalmology = Albrecht Von Graefes Archiv Fur Klinische Und Experimentelle Ophthalmologie, 241(11), pp. 953-7.
Richter MN, et al. Transscleral Resection of a Ciliary Body Leiomyoma in a Child: Case Report and Review of the Literature. Graefes Arch Clin Exp Ophthalmol. 2003;241(11):953-7. PubMed PMID: 14595565.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Transscleral resection of a ciliary body leiomyoma in a child: case report and review of the literature. AU - Richter,Miriam N, AU - Bechrakis,Nikolaos E, AU - Stoltenburg-Didinger,G, AU - Foerster,Michael H, Y1 - 2003/11/01/ PY - 2003/06/23/received PY - 2003/08/09/revised PY - 2003/08/11/accepted PY - 2003/11/5/pubmed PY - 2004/3/6/medline PY - 2003/11/5/entrez SP - 953 EP - 7 JF - Graefe's archive for clinical and experimental ophthalmology = Albrecht von Graefes Archiv fur klinische und experimentelle Ophthalmologie JO - Graefes Arch. Clin. Exp. Ophthalmol. VL - 241 IS - 11 N2 - PURPOSE: To present the case of a patient with leiomyoma of the ciliary body and discuss the histological features and treatment of this rare intraocular tumor. METHODS/CASE REPORT: A 13-year-old boy presented with an asymptomatic tumor of the right eye. Visual acuity was 20/20 in both eyes. Ophthalmoscopy revealed an amelanotic, vascularized ciliary body tumor with exudative retinal detachment and partial transillumination. On ultrasound examination the tumor height was 8 mm and a low internal reflectivity was found. T2-weighted MRI scans showed a hypointense and T1-weighted scans a hyperintense intraocular mass with significant Gd-TPA enhancement. On the assumption that the diagnosis was consistent with an amelanotic ciliary body melanoma, a transscleral resection with adjuvant ruthenium-106 brachytherapy was performed. Visual acuity was 20/40 at 6 months after the operation. RESULTS: Routine stains revealed a pleomorphic tumor composed mainly of spindle cells with palisading in some areas and a prominent intercellular fibrillary background. Immunohistochemistry showed positivity for desmin, vimentin and actin. No reactivity with S-100 and HMB-45 was seen. Intracytoplasmatic filaments and micropinocytotic vesicles were detected by transmission electron microscopy. These findings were consistent with the diagnosis of a ciliary body leiomyoma. CONCLUSION: Typical clinical features of leiomyoma include a dome-shaped configuration and translucency, but the final diagnosis can only be confirmed by histology with the aid of immunohistochemistry and electron microscopy. Though rare, leiomyoma should be considered in the differential diagnosis of amelanotic uveal tumors. Transscleral resection is the treatment of choice of anterior uveal leiomyomas, with a fairly good visual prognosis. SN - 0721-832X UR - https://www.unboundmedicine.com/medline/citation/14595565/Transscleral_resection_of_a_ciliary_body_leiomyoma_in_a_child:_case_report_and_review_of_the_literature_ L2 - https://dx.doi.org/10.1007/s00417-003-0766-x DB - PRIME DP - Unbound Medicine ER -