UNUSUAL PRIMARY OCULAR NEOPLASM IN A CHILD: LEIOMYOSARCOMA OF THE CILIARY BODY

Object. The authors report their experience using gamma knife radiosurgery (GKS) to treat uveal melanomas. Methods. Between 1992 and 1998, 60 patients were treated with GKS at a prescription dose between 45 Gy and 80 Gy. The mean diameter of the tumor base was 12.2 mm (range 3–22 mm). The mean height of the tumor prominence was 6.7 mm (range 3–12 mm). The eye was immobilized. The follow-up period ranged from 16 to 94 months. Tumor regression was achieved in 56 (93%) of 60 patients. There were four recurrences followed by enucleation. The severe side effect of neovascular glaucoma developed in 21 (35%) patients in a high-dose group with larger tumors and in proximity to the ciliary body. A reduction in the prescription dose to 40 Gy or less and excluding treatment to tumors near the ciliary body decreased the rate of glaucoma without affecting the rate of tumor control. Conclusions. Gamma knife radiosurgery at a prescription dose of 45 Gy or more can achieve tumor regression in 85% of the uveal melanomas treated. Neovascular glaucoma can develop in patients when using this dose in tumors near the ciliary body. It is advised that such tumors be avoided and that the prescription dose be reduced to 40 Gy.

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Notch Signaling Controls Ciliary Body Morphogenesis and Secretion by Directly Regulating Nectin Protein Expression

SSRN Electronic Journal ◽

10.2139/ssrn.3418423 ◽

2019 ◽

Author(s):

Ji Pang ◽

Liang Le ◽

Yi Zhou ◽

Qiang Hou ◽

Marina Thexton ◽

...

Keyword(s):

Protein Expression ◽

Notch Signaling ◽

Ciliary Body

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Eye Tumors in Childhood as First Sign of Tumor Predisposition Syndromes: Insights from an Observational Study Conducted in Germany and Austria

Cancers ◽

10.3390/cancers13081876 ◽

2021 ◽

Vol 13 (8) ◽

pp. 1876

Author(s):

Madlen Reschke ◽

Eva Biewald ◽

Leo Bronstein ◽

Ines B. Brecht ◽

Sabine Dittner-Moormann ◽

...

Keyword(s):

Optic Nerve ◽

Observational Study ◽

Ciliary Body ◽

Prospective Observational Study ◽

Genetic Data ◽

Detailed History ◽

Cancer Early Detection ◽

Physical Examinations ◽

Eye Tumors ◽

Multicenter Prospective Observational Study

Retinoblastoma and other eye tumors in childhood are rare diseases. Many eye tumors are the first signs of a genetic tumor predisposition syndrome and the affected children carry a higher risk of developing other cancers later in life. Clinical and genetic data of all children with eye tumors diagnosed between 2013–2018 in Germany and Austria were collected in a multicenter prospective observational study. In five years, 300 children were recruited into the study: 287 with retinoblastoma, 7 uveal melanoma, 3 ciliary body medulloepithelioma, 2 retinal astrocytoma, 1 meningioma of the optic nerve extending into the eye. Heritable retinoblastoma was diagnosed in 44% of children with retinoblastoma. One child with meningioma of the optic nerve extending into the eye was diagnosed with neurofibromatosis 2. No pathogenic constitutional variant in DICER1 was detected in a child with medulloepithelioma while two children did not receive genetic analysis. Because of the known association with tumor predisposition syndromes, genetic counseling should be offered to all children with eye tumors. Children with a genetic predisposition to cancer should receive a tailored surveillance including detailed history, physical examinations and, if indicated, imaging to screen for other cancer. Early detection of cancers may reduce mortality.

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Patients presenting with metastases: stage IV uveal melanoma, an international study

British Journal of Ophthalmology ◽

10.1136/bjophthalmol-2020-317949 ◽

2021 ◽

pp. bjophthalmol-2020-317949

Author(s):

Gaurav Garg ◽

Paul T Finger ◽

Tero T Kivelä ◽

E Rand Simpson ◽

Brenda L Gallie ◽

...

Keyword(s):

Uveal Melanoma ◽

Ciliary Body ◽

Regional Lymph Node ◽

Subcutaneous Tissue ◽

Primary Tumour ◽

Stage Iv ◽

International Study ◽

Whole Body ◽

Retrospective Data Analysis ◽

Synchronous Metastases

ObjectiveTo analyse ocular and systemic findings of patients presenting with systemic metastasis.Methods and analysisIt is an international, multicentre, internet-enabled, registry-based retrospective data analysis. Patients were diagnosed between 2001 and 2011. Data included: primary tumour dimensions, extrascleral extension, ciliary body involvement, American Joint Committee on Cancer (AJCC)-tumour, node, metastasis staging, characteristics of metastases.ResultsOf 3610 patients with uveal melanoma, 69 (1.9%; 95% CI 1.5 to 2.4) presented with clinical metastasis (stage IV). These melanomas originated in the iris, ciliary body and choroid in 4%, 16% and 80% of eyes, respectively. Using eighth edition AJCC, 8 (11%), 20 (29%), 24 (35%), and 17 (25%) belonged to AJCC T-categories T1–T4. Risk of synchronous metastases increased from 0.7% (T1) to 1.5% (T2), 2.6% (T3) and 7.9% (T4). Regional lymph node metastases (N1a) were detected in 9 (13%) patients of whom 6 (67%) had extrascleral extension. Stage of systemic metastases (known for 40 (59%) stage IV patients) revealed 14 (35%), 25 (63%) and 1 (2%) had small (M1a), medium-sized (M1b) and large-sized (M1c) metastases, respectively. Location of metastases in stage IV patients were liver (91%), lung (16%), bone (9%), brain (6%), subcutaneous tissue (4%) and others (5%). Multiple sites of metastases were noted in 24%. Compared with the 98.1% of patients who did not present with metastases, those with synchronous metastases had larger intraocular tumours, more frequent extrascleral extension, ciliary body involvement and thus a higher AJCC T-category.ConclusionsThough higher AJCC T-stage was associated with risk for metastases at diagnosis, even small T1 tumours were stage IV at initial presentation. The liver was the most common site of metastases; however, frequent multiorgan involvement supports initial whole-body staging.

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Identification of beta-adrenergic receptors in the pigmented mammalian iris-ciliary body diaphragm

Experimental Eye Research ◽

10.1016/0014-4835(82)90017-3 ◽

1982 ◽

Vol 34 (1) ◽

pp. 153-157 ◽

Cited By ~ 15

Author(s):

Graham E. Trope ◽

Barry Clark ◽

S.J.S. Titinchi

Keyword(s):

Adrenergic Receptors ◽

Ciliary Body ◽

Beta Adrenergic Receptors ◽

Beta Adrenergic

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High-Intensity Focused Ultrasound Circular Cyclocoagulation in Glaucoma: A Step Forward for Cyclodestruction?

Journal of Ophthalmology ◽

10.1155/2017/7136275 ◽

2017 ◽

Vol 2017 ◽

pp. 1-14 ◽

Cited By ~ 5

Author(s):

Rodolfo Mastropasqua ◽

Vincenzo Fasanella ◽

Alessandra Mastropasqua ◽

Marco Ciancaglini ◽

Luca Agnifili

Keyword(s):

High Intensity ◽

Ciliary Body ◽

Focused Ultrasound ◽

Current Knowledge ◽

High Intensity Focused Ultrasound ◽

High Rate ◽

Dose Effect ◽

Computer Assisted ◽

Refractory Glaucoma ◽

High Level

The ciliary body ablation is still considered as a last resort treatment to reduce the intraocular pressure (IOP) in uncontrolled glaucoma. Several ablation techniques have been proposed over the years, all presenting a high rate of complications, nonselectivity for the target organ, and unpredictable dose-effect relationship. These drawbacks limited the application of cyclodestructive procedures almost exclusively to refractory glaucoma. High-intensity focused ultrasound (HIFU), proposed in the early 1980s and later abandoned because of the complexity and side effects of the procedure, was recently reconsidered in a new approach to destroy the ciliary body. Ultrasound circular cyclocoagulation (UC3), by using miniaturized transducers embedded in a dedicated circular-shaped device, permits to selectively treat the ciliary body in a one-step, computer-assisted, and non-operator-dependent procedure. UC3 shows a high level of safety along with a predictable and sustained IOP reduction in patients with refractory glaucoma. Because of this, the indication of UC3 was recently extended also to naïve-to-surgery patients, thus reconsidering the role and timing of ciliary body ablation in the surgical management of glaucoma. This article provides a review of the most used cycloablative techniques with particular attention to UC3, summarizing the current knowledge about this procedure and future possible developments.

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XVIII. On the accommodation of vision, and the anatomy of the ciliary body

Proceedings of the Royal Society of London ◽

10.1098/rspl.1872.0086 ◽

1873 ◽

Vol 21 (139-147) ◽

pp. 423-425

Keyword(s):

Ciliary Body ◽

Ciliary Muscle ◽

Near Vision ◽

Interior Surface ◽

Ciliary Processes ◽

Pass Through ◽

Human Eyes

This paper is to show that the increase in the convexity of the lens, when accommodated for near vision, is effected by compression of the equator of the lens by an erectile cushion composed of the ciliary processes turgid with blood, the ciliary muscle being the motor agent; also that the iris aids accommodation by increasing its rapidity, but that accommodation of vision can be effected slowly without the assistance of the iris. The author states that, by dissection of human eyes, he has determined the existence of an erectile mass attached to the interior surface of the ciliary muscle from which the ciliary processes proceed, and that in dissections of injected specimens the vessels of the ciliary processes and of the erectile mass can be seen to pass through and between the fibres of the ciliary muscle near to the apex of that muscle.

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