Ruthenium-106 Plaque Brachytherapy in the Primary Management of Ocular Medulloepithelioma

Poon, Daniel; Reich, Ehud; Smith, Victoria M.; Kingston, J. E.; Reddy, M. Ashwin; Hungerford, J. L.; Sagoo, Mandeep S.

doi:10.1016/j.ophtha.2015.03.002

Cited by 14 publications

(19 citation statements)

References 5 publications

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“…15 In large series of 41 patients including children and adults, conservative treatment by brachytherapy was performed in only in 3 cases (9%) with an apex dose of 40 ECG. 16 We preferred use brachytherapy in the first treatment line, which is reported to be an optimal treatment for both, malignant melanoma and ME. 16 We preferred use brachytherapy in the first treatment line, which is reported to be an optimal treatment for both, malignant melanoma and ME.…”

Section: Discussionmentioning

confidence: 99%

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Adult ocular medulloepithelioma diagnosed by transscleral fine needle aspiration: A case report

Mahdjoubi

Cassoux

Lévy-Gabriel

et al. 2017

Diagnostic Cytopathology

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Ocular medulloepithelioma (ME) is a rare congenital tumor which occurs usually during childhood but is also reported in adults. They have seen an intraocular tumor in an 89 years-old female with a history of small cell lung carcinoma. Transscleral fine needle aspiration was performed. Aspirates were rich and composed of two distinctive populations of cells. The first consisted of epithelioid large cohesive cells with rare rosettes. Nuclei were oval and chromatin was delicate with small nucleoli. The second population consisted of smaller and dispersed cells with regular nuclei and dusty chromatin. Immunohistochemistry performed on paraffin-embedded cell block sections showed that the larger cells and rosettes were cytokeratin AE1/AE3, Synaptophysin, Chromogranin A, CD56, NSE, and EMA positive, whereas the smaller cells were always negative. Interestingly smaller cells expressed only weak nuclear positivity for TTF1, whereas larger cells were TTF1 negative. Melanocytic markers were negative in both populations. Morphological patterns and immunohistochemical staining confirmed ocular ME and allowed to exclude pulmonary metastasis or primary malignant melanoma. The patient was successfully treated by brachytherapy alone and is alive and well 10 months after treatment. Diagn. Cytopathol. 2017;45:561-564. © 2017 Wiley Periodicals, Inc.

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Section: Discussionmentioning

confidence: 99%

“…2 In a recent series using a Ruthenium-106 Plaque in children, ME was well controlled and 80% of patients notes a tumor regression at 26 months' follow-up. 16 We preferred use brachytherapy in the first treatment line, which is reported to be an optimal treatment for both, malignant melanoma and ME.…”

Section: Discussionmentioning

confidence: 99%

Adult ocular medulloepithelioma diagnosed by transscleral fine needle aspiration: A case report

Mahdjoubi

Cassoux

Lévy-Gabriel

et al. 2017

Diagnostic Cytopathology

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“…Though systemic chemotherapy with a combination of vincristine, carboplatin, and etoposide has been successful in treating an aggressive medulloepithelioma with extraocular extension and metastasis, there is no standardized treatment regimen and the chemosensitivity in this case cannot be generalized to all types of medulloepithelioma [16,17]. Plaque brachytherapy has shown promise in inducing tumor regression in a small British case series of pediatric medulloepithelioma and in an adult case reported by Poon et al [18]. In atypical presentations, primary enucleation remains a useful diagnostic and therapeutic tool, and should be considered in these cases.…”

Section: Discussionmentioning

confidence: 99%

Nonteratoid Medulloepithelioma Presenting in a 78-Year-Old Male

et al. 2016

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Background: Medulloepithelioma is a rare congenital neoplasm derived from precursors of the nonpigmented ciliary epithelium of the ciliary body. The average patient age at clinical presentation is 3.8 years. Case Presentation: We present the case of a 78-year-old male with progressive lens subluxation and ocular hypertension who was found to have a ciliary body mass. After enucleation for presumed ciliary body melanoma, histopathology showed a nonteratoid medulloepithelioma. Cytogenetic analysis revealed abnormalities in chromosomes 3 and 8. Conclusion: Medulloepithelioma is often initially misdiagnosed. Though congenital in nature, it can exhibit rapid growth, have chromosomal abnormalities, and must be considered in all age groups.

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“…Treatment options for ciliary body medulloepithelioma include cryotherapy, plaque radiotherapy, external beam radiotherapy, local resection, and enucleation. [912174748495051] Enucleation is a standard treatment for advanced intraocular medulloepithelioma with large tumors and neovascular glaucoma. [91217] Enucleation must be performed with minimal manipulation with due care taken to prevent accidental perforation and spillage of tumor cells into the orbit.…”

Section: Managementmentioning

confidence: 99%

“…There have been reports of successful treatment with I-125 and Ru-106 plaque radiotherapy. [495051] The procedure is shorter, technically less demanding and produces better results than local resection. Ang et al [50] reported on 6 cases of medulloepithelioma treated with plaque brachytherapy, as primary treatment in 5 cases and secondary treatment in 1 case.…”

Section: Managementmentioning

confidence: 99%

Intraocular medulloepithelioma – A review of clinical features, DICER 1 mutation, and management

Tadepalli

Shields

et al. 2019

Indian J Ophthalmol

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Intraocular medulloepithelioma is a nonhereditary neoplasm of childhood arising from primitive medullary epithelium. It most often involves the ciliary body. Most patients present between 2 and 10 years of age with loss of vision, pain, leucocoria, or conjunctival congestion. The mass appears as a grey-white ciliary body lesion with intratumoral cysts. Presence of a neoplastic cyclitic membrane with extension to retrolental region is characteristic. Secondary manifestations like cataract and neovascular glaucoma may be present in up to 50% and 60% patients, respectively. These could be the first signs for which, unfortunately, about 50% patients undergo surgery before recognition of the hidden tumor. Systemic correlation with pleuropulmonary blastoma (DICER1 gene) has been documented in 5% cases. Histopathology shows primitive neuroepithelial cells arranged as cords closely resembling the primitive retina. Histopathologically, the tumor is classified as teratoid (containing heteroplastic elements) and nonteratoid (containing medullary epithelial elements), each of which are further subclassified as benign or malignant. Retinoblastoma-like and sarcoma-like areas may be seen within the tissue. The treatment modality depends on tumor size and extent of invasion. For small localized tumors (≤3-4 clock hours), conservative treatments with cryotherapy, plaque radiotherapy, or partial lamellar sclerouvectomy (PLSU) have been used. Plaque brachytherapy is generally preferred for best tumor control. Advanced and extensive tumors require enucleation. Rare use of intra-arterial and intravitreal chemotherapy has been employed. Systemic prognosis is favorable, but those with extraocular extension and orbital involvement show risk for local recurrence and metastatic disease, which can lead to death.

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Ruthenium-106 Plaque Brachytherapy in the Primary Management of Ocular Medulloepithelioma

Cited by 14 publications

References 5 publications

Adult ocular medulloepithelioma diagnosed by transscleral fine needle aspiration: A case report

Adult ocular medulloepithelioma diagnosed by transscleral fine needle aspiration: A case report

Nonteratoid Medulloepithelioma Presenting in a 78-Year-Old Male

Intraocular medulloepithelioma – A review of clinical features, DICER 1 mutation, and management

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