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Adenocarcinoma arising from congenital hypertrophy of the retinal
pigment epithelium.Graefes
Arch Clin Exp Ophthalmol. 2006
Jan;244(1):125-8. Epub 2005 Jun 28.
BACKGROUND:
Adenocarcinoma of the retinal pigment epithelium (RPE) is a rare
primary malignant intraocular neoplasm. We report a
histopathologically confirmed case of adenocarcinoma of the retinal
pigment epithelium arising from congenital hypertrophy of the retinal
pigment epithelium (CHRPE). METHODS: The clinical features, surgical
management, and histopathological features of a melanotic tumor
arising from CHRPE are presented. The tumor was excised by transcleral
resection. RESULTS: Histopathological and immunohistochemical study of
the tumor showed it to be an adenocarcinoma of the RPE. CONCLUSIONS:
Adenocarcinoma of the RPE arising from CHRPE is extremely rare. Such a
tumor can resemble a choroidal melanoma. In spite of the rarity of
this association, periodic monitoring of CHRPE for development of a
nodular tumor of the RPE is probably warranted.
Adenocarcinoma
arising from congenital hypertrophy of retinal pigment epithelium.Arch
Ophthalmol. 2001 Apr;119(4):597-602.
Congenital
hypertrophy of the retinal pigment epithelium (CHRPE), traditionally
regarded as a benign stationary condition, has recently been shown in
5 cases to give rise to an elevated, solid tumor. However, the
histopathologic nature of the tumor that arises from CHRPE has not
been previously determined. A 65-year-old woman developed a
progressively enlarging peripheral fundus tumor that arose from a
focus of classic CHRPE. The tumor produced a localized exudative
retinal detachment, cystoid macular edema, and surface-wrinkling
retinopathy. The mass was removed by local resection, and
histopathologic examination revealed a low-grade adenocarcinoma of the
retinal pigment epithelium, apparently arising from CHRPE. Although
CHRPE is usually a benign nonprogressive lesion, it can give rise to a
malignant tumor. Congenital hypertrophy of the retinal pigment
epithelium should be observed periodically for development of a
neoplasm.
Adenocarcinoma of
the retinal pigment epithelium: a diagnostic and therapeutic
challenge.Graefes
Arch Clin Exp Ophthalmol. 1996 Aug;234
Suppl 1:S22-7
BACKGROUND:
Adenocarcinoma of the retinal pigment epithelium (RPE) is an uncommon
intraocular tumor which has been rarely if ever diagnosed prior to
enucleation. Our review of the literature suggests that when a
melanotic and tuberous-shaped tumor presents in a woman with signs of
uveitis one should consider the diagnosis of adenocarcinoma of the RPE.
METHODS: We report a case in which a 4.9-mm-tall, mushroom-shaped,
moderately reflective melanotic tumor found to be present in a
50-year-old woman. Other findings included a 12 x 10-mm dense
transillumination shadow and 270 deg of posterior iris synechiae. With
a presumptive diagnosis of uveal melanoma the patient was treated with
palladium-103 plaque radiotherapy. Within 6 months she underwent
enucleation due to uncontrollable ocular pain and progressive tumor
growth. Histopathologic evaluation revealed an adenocarcinoma of the (RPE).
RESULTS: Two months after radiotherapy the eye developed acute
angle-closure glaucoma, secondary retinal detachment, and a painful
scleritis (a result similar to that of Greer, who treated an
intraocular adenoma with radiation). Our diagnosis of adenocarcinoma
of the RPE was made after enucleation. CONCLUSION: Adenocarcinomas of
the RPE are rarely if ever diagnosed on clinical evaluation, should be
suspected in women with a melanotic intraocular tumor and uveitis, and
may respond poorly to ophthalmic plaque radiotherapy. All reported
cases presenting without extrascleral extension have not metastasized.
Presumed
adenocarcinoma of the retinal pigment epithelium in a blind eye with a
staphyloma.Arch
Ophthalmol. 1998 Apr;116(4):525-8.
The retinal pigment epithelium (RPE) can undergo reactive hyperplasia
and metaplasia following a variety of ocular insults. However, true
neoplasms of the RPE are rare. We report a case of a papillary
adenocarcinoma of the RPE arising in the blind staphylomatous right
eye of a 79-year-old woman with a long history of bilateral posterior
staphylomas who was seen with increasing pain and exophthalmos of the
right eye. Findings from ultrasonography and computed tomography
demonstrated linear calcification consistent with osseous metaplasia
of the RPE. Progression of the exophthalmos and worsening exposure
keratitis led to enucleation of the eye. Gross pathology showed a
79-mm-long globe. Histopathologic findings revealed a largely
amelanotic papillary adenocarcinoma arising from the RPE. Positive
immunoreactivity for cytokeratin supported the epithelial origin of
the tumor. Adenocarcinoma of the RPE is rare but may develop in a
blind eye.
Clinicopathologic correlation of a case of adenocarcinoma of the
retinal pigment epithelium.Am
J Ophthalmol. 1995 Feb;119(2):243-5.
PURPOSE/METHODS: We observed a clinicopathologic correlation of a case
of adenocarcinoma of the retinal pigment epithelium.
RESULTS/CONCLUSIONS: The ultrasonographic findings of this tumor
demonstrated a collar button profile with high internal reflectivity.
The ultrasonographic characteristics in this case reflected the
variable vascularity and cystic spaces within the tumor.
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