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Mesectodermal Suprauveal Iridociliary Leiomyoma: Transscleral Excision Without Postoperative Iris Defect

Mesectodermal Suprauveal Iridociliary Leiomyoma: Transscleral Excision Without Postoperative Iris... Mesectodermal leiomyoma of the ciliary body is a rare tumor originating from smooth muscle, having both muscular and neural differentiation.1 The first case was reported in 1977, and so far 24 cases have been reported.1 It should be considered in the differential diagnosis of an amelanotic melanoma, especially in young people. Herein, we report a case of mesectodermal iridociliary leiomyoma in a young man, showing the results of ultrasound biomicroscopy, 3-T magnetic resonance imaging, histology, and immunohistochemistry. The tumor was excised en bloc by a transscleral approach and later treated with ruthenium 106 plaque brachytherapy. Report of a Case A 20-year-old man was referred to our department because a slow-growing red mass had been visible on the right iris for 3 months. Visual acuity was 20/20 OU. Slitlamp examination revealed a vascularized mass extending from the 3-o’clock position to the 5-o’clock position on the iris, pushing the iris root centrally. The lesion measured 4.5 × 2.0 mm (Figure 1A). Gonioscopy showed the mass extending into the open anterior chamber angle. Transillumination showed enhanced transmission of light within the mass extending into the ciliary body. The intraocular pressure and fundus were normal. The left eye examination findings were unremarkable. Ultrasound biomicroscopy revealed a low reflective iridociliary mass measuring 9.7 × 6.8 mm with a prominence of 5.6 mm including the sclera (Figure 1B). A well-defined mass located in the ciliary body and iris was seen on 3-T magnetic resonance imaging; it was hyperintense on T1-weighted imaging with marked enhancement after gadolinium administration (Figure 1C and D). Although the clinical diagnosis was a leiomyoma, a biopsy was performed based on the patient's and his family's wish. This established the smooth muscle nature of the tumor. The therapeutic options were discussed with the patient and his family, and excision was chosen. Transscleral en bloc excision of the tumor was performed by one of us (G.P.M.L.) by peeling the suprauveal tumor off the ciliary body under a large deep scleral flap, thus sparing the iris, ciliary body stroma, and choroidal tissue.2 Histopathology revealed bundles of elongated spindle cells with oval nuclei and eosinophilic cytoplasm composed of nonbranching fibrils. Immunohistochemistry showed positive results for α–smooth muscle actin, desmin, caldesmon, CD56, and neuron-specific enolase and negative results for S-100 protein and HMB-45 (Figure 2A-C). Therefore, diagnosis of an iridociliary mesectodermal leiomyoma was confirmed. The postoperative result was excellent, with a normal appearance of the iris (Figure 2D). To avoid any recurrences given that small remnants of the tumor were left in situ, a ruthenium 106 CCB plaque delivering 108 Gy (to convert gray to rad, multiply by 100) at 2.4 mm was applied over the excised area. View LargeDownload Figure 1. Preoperative slitlamp photograph, ultrasound biomicroscopy image, and magnetic resonance images. A, Slitlamp photograph shows a vascular lesion in the nasal iris of the right eye pushing the normal iris tissue centrally. B, Ultrasound biomicroscopy shows a low reflective solid mass arising from the ciliary body and involving the iris. C, Axial magnetic resonance image shows a well-defined mass (arrow) in the ciliary body and iris of the right eye. The lesion is hyperintense to the vitreous on the T1-weighted image with fat suppression. D, A T1-weighted magnetic resonance image shows the same lesion (arrow) with marked enhancement after gadolinium administration. View LargeDownload Figure 2. Histologic findings and postoperative slitlamp photograph. A, The tumor removed in this case had elongated spindle cells with oval nuclei (hematoxylin-eosin, original magnification ×40). B, Tumor cells had immunoreactivity for CD56 antigen (original magnification ×40). C, Tumor cells had positive staining with neuron-specific enolase (original magnification ×40). D, Slitlamp photograph of the same eye 20 days after the excision of tumor shows no iris defect. Comment Mesectodermal leiomyoma of the ciliary body originates from the mesectodermal smooth muscle cells derived embryologically from the neural crest origin. Differential diagnoses include amelanotic melanoma, metastases, schwannomas, and adenocarcinoma of ciliary body. It was impossible to distinguish intraocular leiomyoma from amelanotic melanomas without histochemistry.3 Now, the correlation of marked enhancement of leiomyoma demonstrated after gadolinium administration on T1-weighted imaging as compared with moderate enhancement in melanoma can give a diagnostic clue for leiomyoma as shown in our case.2,4,5 To date, immunohistochemistry has found only 3 cases positive for CD56 and caldesmon, while only 2 were positive for neuron-specific enolase.1,6 Our case also showed positive results for all of these markers. Although mesectodermal leiomyoma is a benign lesion, it can grow and cause complications such as lens subluxation and secondaryglaucoma and can extend extrasclerally. For these reasons and for diagnostic confirmation, surgery is necessary. Transscleral resection as performed in our case allows excision of the tumor without any postoperative iris defect, avoiding potential photophobia, astigmatism, or other aberrations or decreased vision, and leads to good cosmetic results. Back to top Article Information Correspondence: Dr Luyten, Department of Ophthalmology, Leiden University Medical Center, Albinusdreef 2, 2300 RC, Leiden, the Netherlands (g.p.m.luyten@lumc.nl). Financial Disclosure: None reported. References 1. Koletsa T, Karayannopoulou G, Dereklis D, Vasileiadis I, Papadimitriou CS, Hytiroglou P. Mesectodermal leiomyoma of the ciliary body: report of a case and review of the literature. Pathol Res Pract. 2009;205(2):125-13018930601PubMedGoogle ScholarCrossref 2. Shields JA, Shields CL, Eagle RC Jr, De Potter P. Observations on seven cases of intraocular leiomyoma: the 1993 Byron Demorest Lecture. Arch Ophthalmol. 1994;112(4):521-5288155052PubMedGoogle ScholarCrossref 3. Foss AJ, Pecorella I, Alexander RA, Hungerford JL, Garner A. Are most intraocular “leiomyomas” really melanocytic lesions? Ophthalmology. 1994;101(5):919-9248190481PubMedGoogle Scholar 4. Richter MN, Bechrakis NE, Stoltenburg-Didinger G, Foerster MH. 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):953-95714595565PubMedGoogle ScholarCrossref 5. Potter DP, Shields JA, Shields CL. Tumors of the uvea. In: Potter DP, Shields JA, Shields CL, eds. MRI of the Eye and Orbit. Philadelphia, PA: JB Lippincott; 1995:58 6. Odashiro AN, Fernandes BF, Al-Kandari A, Gregoire FJ, Burnier MN Jr. Report of two cases of ciliary body mesectodermal leiomyoma: unique expression of neural markers. Ophthalmology. 2007;114(1):157-16117070579PubMedGoogle ScholarCrossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Ophthalmology American Medical Association

Mesectodermal Suprauveal Iridociliary Leiomyoma: Transscleral Excision Without Postoperative Iris Defect

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Publisher
American Medical Association
Copyright
Copyright © 2011 American Medical Association. All Rights Reserved.
ISSN
0003-9950
eISSN
1538-3687
DOI
10.1001/archophthalmol.2011.342
Publisher site
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Abstract

Mesectodermal leiomyoma of the ciliary body is a rare tumor originating from smooth muscle, having both muscular and neural differentiation.1 The first case was reported in 1977, and so far 24 cases have been reported.1 It should be considered in the differential diagnosis of an amelanotic melanoma, especially in young people. Herein, we report a case of mesectodermal iridociliary leiomyoma in a young man, showing the results of ultrasound biomicroscopy, 3-T magnetic resonance imaging, histology, and immunohistochemistry. The tumor was excised en bloc by a transscleral approach and later treated with ruthenium 106 plaque brachytherapy. Report of a Case A 20-year-old man was referred to our department because a slow-growing red mass had been visible on the right iris for 3 months. Visual acuity was 20/20 OU. Slitlamp examination revealed a vascularized mass extending from the 3-o’clock position to the 5-o’clock position on the iris, pushing the iris root centrally. The lesion measured 4.5 × 2.0 mm (Figure 1A). Gonioscopy showed the mass extending into the open anterior chamber angle. Transillumination showed enhanced transmission of light within the mass extending into the ciliary body. The intraocular pressure and fundus were normal. The left eye examination findings were unremarkable. Ultrasound biomicroscopy revealed a low reflective iridociliary mass measuring 9.7 × 6.8 mm with a prominence of 5.6 mm including the sclera (Figure 1B). A well-defined mass located in the ciliary body and iris was seen on 3-T magnetic resonance imaging; it was hyperintense on T1-weighted imaging with marked enhancement after gadolinium administration (Figure 1C and D). Although the clinical diagnosis was a leiomyoma, a biopsy was performed based on the patient's and his family's wish. This established the smooth muscle nature of the tumor. The therapeutic options were discussed with the patient and his family, and excision was chosen. Transscleral en bloc excision of the tumor was performed by one of us (G.P.M.L.) by peeling the suprauveal tumor off the ciliary body under a large deep scleral flap, thus sparing the iris, ciliary body stroma, and choroidal tissue.2 Histopathology revealed bundles of elongated spindle cells with oval nuclei and eosinophilic cytoplasm composed of nonbranching fibrils. Immunohistochemistry showed positive results for α–smooth muscle actin, desmin, caldesmon, CD56, and neuron-specific enolase and negative results for S-100 protein and HMB-45 (Figure 2A-C). Therefore, diagnosis of an iridociliary mesectodermal leiomyoma was confirmed. The postoperative result was excellent, with a normal appearance of the iris (Figure 2D). To avoid any recurrences given that small remnants of the tumor were left in situ, a ruthenium 106 CCB plaque delivering 108 Gy (to convert gray to rad, multiply by 100) at 2.4 mm was applied over the excised area. View LargeDownload Figure 1. Preoperative slitlamp photograph, ultrasound biomicroscopy image, and magnetic resonance images. A, Slitlamp photograph shows a vascular lesion in the nasal iris of the right eye pushing the normal iris tissue centrally. B, Ultrasound biomicroscopy shows a low reflective solid mass arising from the ciliary body and involving the iris. C, Axial magnetic resonance image shows a well-defined mass (arrow) in the ciliary body and iris of the right eye. The lesion is hyperintense to the vitreous on the T1-weighted image with fat suppression. D, A T1-weighted magnetic resonance image shows the same lesion (arrow) with marked enhancement after gadolinium administration. View LargeDownload Figure 2. Histologic findings and postoperative slitlamp photograph. A, The tumor removed in this case had elongated spindle cells with oval nuclei (hematoxylin-eosin, original magnification ×40). B, Tumor cells had immunoreactivity for CD56 antigen (original magnification ×40). C, Tumor cells had positive staining with neuron-specific enolase (original magnification ×40). D, Slitlamp photograph of the same eye 20 days after the excision of tumor shows no iris defect. Comment Mesectodermal leiomyoma of the ciliary body originates from the mesectodermal smooth muscle cells derived embryologically from the neural crest origin. Differential diagnoses include amelanotic melanoma, metastases, schwannomas, and adenocarcinoma of ciliary body. It was impossible to distinguish intraocular leiomyoma from amelanotic melanomas without histochemistry.3 Now, the correlation of marked enhancement of leiomyoma demonstrated after gadolinium administration on T1-weighted imaging as compared with moderate enhancement in melanoma can give a diagnostic clue for leiomyoma as shown in our case.2,4,5 To date, immunohistochemistry has found only 3 cases positive for CD56 and caldesmon, while only 2 were positive for neuron-specific enolase.1,6 Our case also showed positive results for all of these markers. Although mesectodermal leiomyoma is a benign lesion, it can grow and cause complications such as lens subluxation and secondaryglaucoma and can extend extrasclerally. For these reasons and for diagnostic confirmation, surgery is necessary. Transscleral resection as performed in our case allows excision of the tumor without any postoperative iris defect, avoiding potential photophobia, astigmatism, or other aberrations or decreased vision, and leads to good cosmetic results. Back to top Article Information Correspondence: Dr Luyten, Department of Ophthalmology, Leiden University Medical Center, Albinusdreef 2, 2300 RC, Leiden, the Netherlands (g.p.m.luyten@lumc.nl). Financial Disclosure: None reported. References 1. Koletsa T, Karayannopoulou G, Dereklis D, Vasileiadis I, Papadimitriou CS, Hytiroglou P. Mesectodermal leiomyoma of the ciliary body: report of a case and review of the literature. Pathol Res Pract. 2009;205(2):125-13018930601PubMedGoogle ScholarCrossref 2. Shields JA, Shields CL, Eagle RC Jr, De Potter P. Observations on seven cases of intraocular leiomyoma: the 1993 Byron Demorest Lecture. Arch Ophthalmol. 1994;112(4):521-5288155052PubMedGoogle ScholarCrossref 3. Foss AJ, Pecorella I, Alexander RA, Hungerford JL, Garner A. Are most intraocular “leiomyomas” really melanocytic lesions? Ophthalmology. 1994;101(5):919-9248190481PubMedGoogle Scholar 4. Richter MN, Bechrakis NE, Stoltenburg-Didinger G, Foerster MH. 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):953-95714595565PubMedGoogle ScholarCrossref 5. Potter DP, Shields JA, Shields CL. Tumors of the uvea. In: Potter DP, Shields JA, Shields CL, eds. MRI of the Eye and Orbit. Philadelphia, PA: JB Lippincott; 1995:58 6. Odashiro AN, Fernandes BF, Al-Kandari A, Gregoire FJ, Burnier MN Jr. Report of two cases of ciliary body mesectodermal leiomyoma: unique expression of neural markers. Ophthalmology. 2007;114(1):157-16117070579PubMedGoogle ScholarCrossref

Journal

Archives of OphthalmologyAmerican Medical Association

Published: Dec 1, 2011

References

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