Related Experiment Video
Updated: Mar 20, 2026
Implantation and Evaluation of Melanoma in the Murine Choroid via Optical Coherence Tomography
Published on: December 2, 2022
Intraocular Teratoid Medulloepithelioma Presenting With a Completely Rhabdomyosarcomatous Distant Metastasis
Frederick A Jakobiec1, Durga S Borkar2, Anna M Stagner1
1Department of Ophthalmology, Harvard Medical School, Boston, Massachusetts2David G. Cogan Laboratory of Ophthalmic Pathology, Massachusetts Eye and Ear Infirmary, Boston.
Importance:
Medulloepithelioma is the second most common primary neuroepithelial tumor of the eye. The full range of its morphologic expressions and appearances in metastases have not been fully explored.
Observations:
A patient in her 50s with glaucoma for decades had undergone multiple filtering surgical procedures, including the placement of a glaucoma drainage device. A paraspinal mass was discovered, and tumor and bone marrow biopsies disclosed rhabdomyosarcoma. This led to the discovery of a multicystic intraocular tumor. A metastatic rhabdomyosarcoma to the eye was considered unlikely because, to our knowledge, this event had never been reported. An enucleation was performed, and an intraocular tumor composed almost entirely of rhabdomyoblasts (desmin- and myogenin-positive) was discovered along with rare clusters of persistent neuroepithelial cells.
Conclusions And Relevance:
To our knowledge, this is the first case of a medulloepithelioma in which teratoid rhabdomyoblasts effaced all but trace amounts of neuroepithelium and generated a distant metastasis entirely composed of rhabdomyoblasts. The prolonged history and filtering procedures probably led to these 2 phenomena.
Insights
This study reports the first known case of medulloepithelioma with teratoid rhabdomyoblasts causing a metastasis composed entirely of rhabdomyoblasts. Glaucoma filtering procedures may have contributed to this rare ocular tumor presentation.
Area of Science:
- Ophthalmology
- Oncology
- Pathology
Background:
- Medulloepithelioma is the second most frequent primary neuroepithelial eye tumor.
- The diverse morphology of medulloepithelioma, especially in metastatic forms, remains incompletely understood.
Observation:
- A patient with a history of chronic glaucoma and multiple filtering surgeries presented with a paraspinal mass.
- Biopsies revealed rhabdomyosarcoma, prompting investigation that uncovered a multicystic intraocular tumor.
- Enucleation revealed an intraocular tumor predominantly composed of rhabdomyoblasts with minimal residual neuroepithelium.
Findings:
- This case represents the first documented instance of medulloepithelioma where teratoid rhabdomyoblasts obscured most neuroepithelium.
- The tumor generated a distant metastasis exclusively composed of rhabdomyoblasts.
- Rhabdomyoblasts were confirmed positive for desmin and myogenin.
Implications:
- The findings suggest a potential link between prior glaucoma filtering procedures and the development of this unusual medulloepithelioma presentation.
- This case expands the known spectrum of medulloepithelioma, highlighting its capacity for aggressive, rhabdomyoblastic differentiation and metastasis.
Related Concept Videos
01:20The Retinoblastoma Gene
The first-ever tumor suppressor gene called Rb was identified in retinoblastoma - a rare eye tumor in children. In inherited forms of the disease, a child inherits one defective copy of the Rb gene, which predisposes them to retinoblastoma. However,...
01:20The Retinoblastoma Gene