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Intraocular Teratoid Medulloepithelioma Presenting With a Completely Rhabdomyosarcomatous Distant Metastasis.

Publication ,  Journal Article
Jakobiec, FA; Borkar, DS; Stagner, AM; Lee, NG
Published in: JAMA Ophthalmol
August 1, 2016

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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Published In

JAMA Ophthalmol

DOI

EISSN

2168-6173

Publication Date

August 1, 2016

Volume

134

Issue

8

Start / End Page

919 / 923

Location

United States

Related Subject Headings

  • Rhabdomyosarcoma
  • Ophthalmology & Optometry
  • Neuroectodermal Tumors, Primitive
  • Neoplasm Metastasis
  • Middle Aged
  • Magnetic Resonance Imaging
  • Humans
  • Female
  • Eye Neoplasms
  • Eye Enucleation
 

Citation

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MLA
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Jakobiec, F. A., Borkar, D. S., Stagner, A. M., & Lee, N. G. (2016). Intraocular Teratoid Medulloepithelioma Presenting With a Completely Rhabdomyosarcomatous Distant Metastasis. JAMA Ophthalmol, 134(8), 919–923. https://doi.org/10.1001/jamaophthalmol.2016.1515
Jakobiec, Frederick A., Durga S. Borkar, Anna M. Stagner, and Nahyoung Grace Lee. “Intraocular Teratoid Medulloepithelioma Presenting With a Completely Rhabdomyosarcomatous Distant Metastasis.JAMA Ophthalmol 134, no. 8 (August 1, 2016): 919–23. https://doi.org/10.1001/jamaophthalmol.2016.1515.
Jakobiec FA, Borkar DS, Stagner AM, Lee NG. Intraocular Teratoid Medulloepithelioma Presenting With a Completely Rhabdomyosarcomatous Distant Metastasis. JAMA Ophthalmol. 2016 Aug 1;134(8):919–23.
Jakobiec, Frederick A., et al. “Intraocular Teratoid Medulloepithelioma Presenting With a Completely Rhabdomyosarcomatous Distant Metastasis.JAMA Ophthalmol, vol. 134, no. 8, Aug. 2016, pp. 919–23. Pubmed, doi:10.1001/jamaophthalmol.2016.1515.
Jakobiec FA, Borkar DS, Stagner AM, Lee NG. Intraocular Teratoid Medulloepithelioma Presenting With a Completely Rhabdomyosarcomatous Distant Metastasis. JAMA Ophthalmol. 2016 Aug 1;134(8):919–923.

Published In

JAMA Ophthalmol

DOI

EISSN

2168-6173

Publication Date

August 1, 2016

Volume

134

Issue

8

Start / End Page

919 / 923

Location

United States

Related Subject Headings

  • Rhabdomyosarcoma
  • Ophthalmology & Optometry
  • Neuroectodermal Tumors, Primitive
  • Neoplasm Metastasis
  • Middle Aged
  • Magnetic Resonance Imaging
  • Humans
  • Female
  • Eye Neoplasms
  • Eye Enucleation