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Brief Report |

Intraocular Teratoid Medulloepithelioma Presenting With a Completely Rhabdomyosarcomatous Distant Metastasis

Frederick A. Jakobiec, MD, DSc1,2; Durga S. Borkar, MD1; Anna M. Stagner, MD1,2; Nahyoung Grace Lee, MD1,3
[+] Author Affiliations
1Department of Ophthalmology, Harvard Medical School, Boston, Massachusetts
2David G. Cogan Laboratory of Ophthalmic Pathology, Massachusetts Eye and Ear Infirmary, Boston
3Ophthalmic Plastic Surgery, Massachusetts Eye and Ear Infirmary, Boston
JAMA Ophthalmol. 2016;134(8):919-923. doi:10.1001/jamaophthalmol.2016.1515.
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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.

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Figure 1.
Metastatic Intraocular Medulloepithelioma

A, Bone marrow biopsy shows round tumor cells (hematoxylin-eosin, original magnification ×200). B, Desmin positivity (immunoperoxidase reaction, diaminobenzidine chromogen and hematoxylin counterstain, original magnification ×400). C, Top, positron emission tomographic scan with uptake (arrowhead) in left eye only. Bottom, magnetic resonance imaging demonstrates arclike mass posteriorly (arrowhead). D, White pupillary membrane. E, Solid tumor with multiple cysts (arrowheads). F, Freshly enucleated globe with hemorrhagic tumor, glaucoma valve (arrowhead), and inferior cavity of retracted vitreous. T indicates tumor; VR, vitreous retraction.

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Figure 2.
Histopathologic Features of Teratoid Medulloepithelioma

A, Partially hemorrhagic myxoid intravitreal tumor (T) with cysts (black arrowheads) and a fibrous plaque behind the iris (blue arrowhead) (hematoxylin-eosin, original magnification ×12.5). B, Vitreous tumor above invading optic nerve (arrowheads) (hematoxylin-eosin, original magnification ×25). C, Space where part of the valve was located with adjacent surgical wound. Fibrous metaplasia of the lens epithelium has caused a plaque (hematoxylin-eosin, original magnification ×25). The black arrowhead indicates neoplastic membrane; the blue arrowhead highlights distorted ciliary processes. D, Fibrous plaque delimited by lens capsule (black arrowheads) (periodic acid–Schiff, original magnification ×200). Inset: dystrophic calcification under lens capsule (arrowhead) (hematoxylin-eosin, original magnification ×200). E, Round rhabdomyoblasts in the vitreous (hematoxylin-eosin, original magnification ×100). F, Cytologic detail of rhabdomyoblasts (hematoxylin-eosin, original magnification ×200). C indicates cornea; FML, fibrous metaplasia of the lens epithelium; ON, optic nerve; V, valve; VR, vitreous retraction; W, wound.

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Figure 3.
Additional Histopathologic and Immunohistochemical Findings

A, Elongated and slender multipolar tumor cells (periodic acid–Schiff, original magnification ×200). B, Small units of primitive neuroepithelium near pars plana (top panel, hematoxylin-eosin, original magnification ×100; bottom panel, Masson trichrome, original magnification ×100). C, Neuroepithelium breaking up into solid units (arrowheads) and discohesive clusters (inset) with cysts seen above (hematoxylin-eosin, original magnification ×50; inset, hematoxylin-eosin, original magnification ×200). D, Desmin cytoplasmic positivity in round (left) and spindled (right) cells (immunoperoxidase reaction, diaminobenzidine chromogen and hematoxylin counterstain, original magnification ×200). E, Positive nuclear myogenin immunostaining (immunoperoxidase reaction, diaminobenzidine chromogen and hematoxylin counterstain, original magnification ×200). F, Top, pronounced Ki67 nuclear staining in neuroepithelial clusters (immunoperoxidase reaction, diaminobenzidine chromogen and hematoxylin counterstain, original magnification ×200). Bottom, lower proliferation index in mesencyhmal tumor (immunoperoxidase reaction, diaminobenzidine chromogen and hematoxylin counterstain, original magnification ×200). C indicates cysts.

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