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

Abstract

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.

The 5th Edition of the World Health Organization Classification of Tumours of the Eye and Orbit

“…Novel molecular genetic methods, like liquid biopsies of tumor-derived cellfree DNA from aqueous fluid for prognostication and from plasma for prenatal testing and detection of nonocular primary malignancies in children with retinoblastoma, are discussed [50][51][52]. Coverage of diagnostic cytopathology for tumors of the retina and neuroepithelium is a notable addition (Table 10) [2,[53][54][55][56][57][58][59][60].…”

Ciliary body medulloepithelioma treated with intra-operative brachytherapy after fine needle aspiration biopsy

“…Although rare, the possibility of seeding malignant cells outside the eye after tumor's biopsy is of special concern, and some authors suggested that the risk of tumor seeding in medulloepithelioma biopsy should default to the risk-equivalent of retinoblastoma, until more information is available. Nonetheless, in some previous cases, FNAB was used to provide histologic confirmation of medulloepithelioma diagnosis [6,18,[22][23][24][25]. Additionally, it is well-known that both resection with safety margins and radiotherapy or radiofrequency application after biopsy, may prevent the risk of malignant cell seeding in some extra-ocular tumors [26][27][28].…”

Intraocular Medulloepitheliomae (Clinical Case Series)

“…В нашем исследовании у четырех пациентов из девяти опухоль выявлена в возрасте старше 10 лет. МЭ у взрослых пациентов (старше 20 лет) относится к крайне редким образованиям, и в литературе описаны лишь единичные случаи данной патологии во взрослом возрасте [15][16][17][18][19]. МЭ необходимо дифференцировать от приобретенной эпителиомы (аденомы или аденокарциномы) беспигментного цилиарного эпителия, развивающейся во взрослом возрасте и не имеющей признаков эмбрионального новообразования [21].…”

“…Средний возраст манифестации заболевания составил 44 года (в диапазоне 23-79 лет), пятерым пациентам диагноз был поставлен на пятой декаде их жизни. В большинстве случаев был отмечен существенный временной промежуток между проявлением первых симптомов и установлением диагноза [7,8,[15][16][17][18][19].…”