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R. Trespalacios, J.B. Dickson, J.P. Tao; Adenocarcinoma of Ciliary Body Pigment Epithelium: A Rare Tumor With Extraorbital and Extracranial Extension Responds to Gleevec® (Imatinib Mesylate) . Invest. Ophthalmol. Vis. Sci. 2005;46(13):3362.
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Adenocarcinoma of the Ciliary Body Pigment Epithelium (CPE) is an exceedingly rare intraocular tumor with an estimated incidence of one new case per year in the United States. These tumors behave primarily in a locally invasive manner; into the sensory retina and choroid. The authors report a case with histologically proven regional metastasis and distant metastasis.
An 81–year–old pseudophakic white male presents with a blind painful left eye after a retinal detachment and ensuing pthisis. An enucleation was performed, during which the globe was found to ruptured 180 degrees, several millimeters posterior to and parallel to the limbus. Otherwise, the enucleation proceeded uneventfully. Pathological specimen was reported as pleomorphic carcinoma of the ciliary or retinal pigment epithelial origin (AFIP).
Initial work up was negative for metastatic disease. However, routine post operative enucleation care was complicated by recurrent extrusion of the eyelid conformer due to inadequate fornices. Amniotic membrane grafting was unsuccessful. Repeat MRI showed tumor recurrence with extension into the nasolacrimal duct. Prompt exenteration and radiotherapy were performed. Three months after completion of radiotherapy, FNA of an enlarged left sided cervical lymph node contained malignant cells indistinguishable from the malignant cells seen in the prior enucleation specimen (AFIP). PET scan was performed for staging purposes which showed numerous lung nodules suspicious for metastatic disease.
The patient has been diagnosed for two years, and to date is doing well with regression of the metastatic neoplasms using the chemotherapeutic agent Gleevec® (imatinib mesylate).
Adenocarcinoma of the CPE is an extremely rare tumor. Extracranial extension is an even more rare behavior for this type of neoplasm. To the authors' knowledge, this is the first case of adenocarcinoma with confirmed metastasis. Further investigation is waranted as to the precise effect of Gleevec®.
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