Malignant medulloepithelioma mimicking retinoblastoma — clinical and morphological case analysis and cell culture experience

Saakian S.V.

Moscow Helmholtz Research Institute of Eye Diseases, Moscow, Russia

Tsygankov A.Iu.

FGBU "Moskovskiĭ NII glaznykh bolezneĭ im. Gel'mgol'tsa" Minzdrava Rossii

Ivanova O.A.

Russian Scientific Center of Rentgenoradiology of the Ministry of Health of Russia, Moscow, Russia

Zakharova G.P.

FGBU "Moskovskiĭ NII glaznykh bolezneĭ im. Gel'mgol'tsa" Minzdrava Rossii

Isaeva R.Т.

Moscow Helmholtz Research Institute of Eye Diseases, Ministry of Health of the Russian Federation, 14/19 Sadovaya-Chernogryazskaya St., Moscow, Russian Federation, 105062

Moiseeva N.I.

N.N. Blokhin Russian Cancer Research Center, Russian Academy of Medical Sciences, Moscow

Karamysheva А.F.

Russian Oncological Scientific Center named after Nikolay Blokhin, 23 Kashirskoe Shosse, Moscow, 115478, Russian Federation

Malignant medulloepithelioma mimicking retinoblastoma — clinical and morphological case analysis and cell culture experience

Authors:

Saakian S.V., Tsygankov A.Iu., Ivanova O.A., Zakharova G.P., Isaeva R.Т., Moiseeva N.I., Karamysheva А.F.

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Journal: Russian Annals of Ophthalmology. 2016;132(6): 64‑69

DOI: 10.17116/oftalma2016132664-69

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To cite this article:

Saakian SV, Tsygankov AIu, Ivanova OA, Zakharova GP, Isaeva RТ, Moiseeva NI, Karamysheva АF. Malignant medulloepithelioma mimicking retinoblastoma — clinical and morphological case analysis and cell culture experience. Russian Annals of Ophthalmology. 2016;132(6):64‑69. (In Russ.)
https://doi.org/10.17116/oftalma2016132664-69

Подписка 2025 (Russian Annals of Ophthalmology)

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The paper presents clinical and morphological case analysis of primary intraocular malignant medulloepitelioma (medulloblastoma) of rare localization (central part of the retina with optic nerve involvement) that simulated retinoblastoma in a 2-year-old child. Histological features of the tumor are given in details (tubular and mesh structures of the tumor, rosettes, ribbons, cells with hyperchromic nuclei, and cellular polymorphism). An experience of creating a primary intraocular malignant medulloepitelioma cell culture, as yet exclusive in the Russian Federation, is described. Culture sensitivity for particular drugs (oxaliplatin, irinotecan, ifosfamide, and ascorbic acid at different concentrations) was evaluated by MTT-assay. Of the four products, IC50 (3.3 mg/ml) was obtained only for ascorbic acid. Despite the relative rarity of primary intraocular malignant medulloepitelioma, its differential diagnosis should be carried out, with retinoblastoma in mind in the first place. The obtained data on the effectiveness of ascorbic acid against intraocular malignant medulloepitelioma cells can be used to supplement the existing chemotherapeutic protocols in pediatric ocular and neuro-oncology.

Keywords:

intraocular tumor

medulloepithelioma

cell culture

МТТ-assay

retinoblastoma

Authors:

Saakian S.V.

Moscow Helmholtz Research Institute of Eye Diseases, Moscow, Russia

Tsygankov A.Iu.

FGBU "Moskovskiĭ NII glaznykh bolezneĭ im. Gel'mgol'tsa" Minzdrava Rossii

Ivanova O.A.

Russian Scientific Center of Rentgenoradiology of the Ministry of Health of Russia, Moscow, Russia

Zakharova G.P.

FGBU "Moskovskiĭ NII glaznykh bolezneĭ im. Gel'mgol'tsa" Minzdrava Rossii

Isaeva R.Т.

Moscow Helmholtz Research Institute of Eye Diseases, Ministry of Health of the Russian Federation, 14/19 Sadovaya-Chernogryazskaya St., Moscow, Russian Federation, 105062

Moiseeva N.I.

N.N. Blokhin Russian Cancer Research Center, Russian Academy of Medical Sciences, Moscow

Karamysheva А.F.

Russian Oncological Scientific Center named after Nikolay Blokhin, 23 Kashirskoe Shosse, Moscow, 115478, Russian Federation

List of references:

Saunders T, Margo CE. Intraocular medulloepithelioma. Arch Pathol Lab Med. 2012;136(2):212-216. doi: 10.5858/arpa.2010-0669-RS
Vajaranant TS, Mafee MF, Kapur R, Rapoport M, Edward DP. Medulloepithelioma of the ciliary body and optic nerve: clinicopathologic, CT, and MR imaging features. Neuroimaging Clin N Am. 2005;15(1):69-83. doi:10.1016/j.nic.2005.02.008
Foster RE, Murray T, Byrne SF, Hughes JR, Gendron BK, Ehlies FJ, Nicholson DH. Echographic features of medulloepithelioma. Am J Ophthalmol. 2000;130:364-366. doi:10.1016/s0002-9394(00)00542-0
Chavez M, Mafee MF, Castillo B, Kaufman LM, Johnstone H, Edward DP. Medulloepithelioma of the optic nerve. J Pediatr Ophthalmol Strabismus. 2004;41(1):48-52.
Pastora-Salvador N, Abelairas-Gómez J, Peralta-Calvo J, García-Fernández E, Morales-Bastos C, Asencio-Durán M, Carceller-Benito F. Atypical clinical presentation and long-term survival in a patient with optic nerve medulloepithelioma: a case report. J Med Case Rep. 2012;6:123. doi: 10.1186/1752-1947-6-123
Jakobiec FA, Trief D, Rashid A, Rose MF, Minckler D, Vanderveen D, Mukai S. New insights into the development of infantile intraocular medulloepithelioma. Am J Ophthalmol. 2014;158(6):1275-1296.e1. doi: 10.1016/j.ajo.2014.08.036
Horoshilova-Maslova I, Saakyan S, Nabieva M, Zaharova G, Amiryan A. Adenocarcinoma of retinal pigment epithelium (a clinical and histopathological case report). Rossijskij oftal'mologicheskij zhurnal. 2011;4(2):64-68 (In Russ.)
Saakyan S, Amiryan A, Tsygankov A, Zhil'cova M, Zaharova G, Andreeva L. Intraocular schwannoma case report: clinical and pathomorphological analysis. Vestnik oftal'mologii. 2014;130(2):54-58. (In Russ.)
Alkatan H, Al-Amry M, Al-Hussain H, Al-Dhibi H, Al-Mesfer S. Medulloepithelioma of the ciliary body: the delay in diagnosis and frequent initial mismanagement. Can J Ophthalmol. 2011;46(5):431-438. doi: 10.1016/j.jcjo.2011.07.007
Solov'jov A, Morgun V, Pahol'chuk A. Medulloblastomas in children. Onkopediatrija. 2015;2(2):158-161. (In Russ.) doi: 10.15690/onco.v2i2.1349)
Konoplja N, Strongin Ju, Talabaev M, Alejnikova O. Effective potentiation of chemotherapy for pediatric neuroectodermal medulloblastoma. Voprosy onkologii. 2008;54(2):157-163. (In Russ.)
Ushakova T, Pavlovskaja A, Poljakov V. Morphological prognostic factors in children with unilateral retinoblastoma after initial enucleation. Onkohirurgija. 2011;3(4):4-8. (In Russ.)

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