Congenital Malignant Teratoid Neoplasm of the Eye and Orbit

Steinkuller, Paul G.; Font, Ramon L.

doi:10.1016/s0161-6420(97)30364-9

Cited by 27 publications

(13 citation statements)

References 31 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Aggressiveness is related mainly to extraocular extension 2 . The prognosis is poor if extrascleral extension occurs in ocular medulloepitheliomas 18 . In our case, a follow‐up period of 1 year did not reveal any evidence of recurrence but the time is too short to give a decision about the prognosis of our patient.…”

Section: Discussionmentioning

confidence: 56%

“…Typical symptoms of medulloepithelioma are pain and poor vision, leukocoria and mass in the iris or ciliary body 2 . Glaucoma, cataracts, uveitis, rubeosis iridis and retinal detachment may also be observed 2,18 …”

Section: Discussionmentioning

confidence: 99%

“…So enucleation is generally recommended 2,7 . The role of radiotherapy and chemotherapy are unknown 18 …”

Section: Discussionmentioning

confidence: 99%

See 2 more Smart Citations

Malignant non‐teratoid medulloepithelioma of ciliary body with retinoblastic differentiation: A case report and review of literature

Al-Salam

Algawi²,

Al-Ashari

2008

Neuropathology

View full text Add to dashboard Cite

We present a 6-year-old child with intraocular and extraocular mass and high intraocular pressure. The tumor mass involved a disorganized anterior segment and extended through the medial cornea and sclera. A preliminary diagnosis of retinoblastoma with extraocular extension was made. An exenteration of the left globe and orbital tissue was performed. Histological examination showed that the lesion, which occupied the posterior chamber, involved the ciliary body, extending into the iris, sclera and cornea, projecting beyond the cornea anteriorly and extending to the retina posteriorly. The tumor cells were diffusely immunoreactive to vimentin, neuron specific enolase and CD 138. The medulloepithelioma cells were focally positive to cytokeratin (AE1/AE3), cytokeratin 18, CD56,CD57, S100, HMB-45 and bcl2 while areas of retinoblastic differentiation showed diffuse immunoreactivity to synaptophysin, neurofilament and CD138 with focal immunoreactivity to calretinin. All tumor cells showed no immunoreactivity to cytokeratin 7, cytokeratin 20, epithelial membrane antigen, carcinoembryonic antigen, desmin, GFAP, and chromogranin. Nuclear staining for P53 was seen in 80% of tumor cells. The ki-67 index was 90%. The tumor was described as malignant intraocular non-teratoid medulloepithelioma with retinoblastic differentiation arising from the ciliary body. Tumor satellites were seen in the adjacent periocular soft tissue. The treatment involved exenteration of the left globe and orbital tissue with secondary skin graft following chemotherapy. The patient is well and has no recurrence after 1 year of treatment. We report that medulloepithelioma can present as a case of infantile glaucoma, can show signs of intraocular calcifications and can show retinoblastic differentiation.

show abstract

Section: Discussionmentioning

confidence: 56%

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Malignant non‐teratoid medulloepithelioma of ciliary body with retinoblastic differentiation: A case report and review of literature

Al-Salam

Algawi²,

Al-Ashari

2008

Neuropathology

View full text Add to dashboard Cite

show abstract

“…The present case had numerous foci with immature cartilage differentiation in the ocular tumor, and is thus classified as a teratoid ocular medulloepithelioma. Ocular medulloepitheliomas with teratoid elements have also been described in humans, 18,22,23 dogs 11,12 and a llama 17 …”

Section: Discussionmentioning

confidence: 96%

“…Extrabulbar extension to the orbit has been reported in cockatiels and horses 10,14,21 . In humans metastases rarely occur and, if present, local invasion of the orbit seems to be most common 18,22,23 . However, spread to the parotid gland has been reported 22 .…”

Section: Discussionmentioning

confidence: 99%