Cases reported "Uveal Neoplasms"

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1/17. ciliary body melanocytoma with anterior segment pigment dispersion and elevated intraocular pressure.

    PURPOSE: To discuss a case in which melanocytoma of the ciliary body presented with widespread pigment dispersion and elevated intraocular pressure (IOP). methods: A 64-year old woman presented with increased IOP and persistent anterior segment inflammation in her right eye. She had undergone a trabeculectomy for uncontrolled IOP before presentation. Slit-lamp examination, gonioscopy, and ultrasound biomicroscopy revealed a ciliary body mass with extension into the subconjunctiva in addition to widespread pigmentary dispersion in the anterior segment. Enucleation of the right eye was performed for histopathologic evaluation, as a diagnosis of ciliary body melanoma was made on clinical examination. RESULTS: Histopathologic findings were diagnostic of a melanocytoma of the ciliary body with necrosis and focal malignant transformation with extension of melanocytoma cells and melanophages into the subconjunctival space, trabecular meshwork, and anterior chamber angle. CONCLUSION: Melanocytoma of the ciliary body is a rare intraocular tumor that may present with pigment dispersion and secondary elevated IOP. Careful examination of the anterior segment is imperative in such cases.
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2/17. Ring melanoma--a rare cause of refractory glaucoma.

    BACKGROUND: Ring melanoma of the ciliary body and iris is extremely rare and often has adverse histology. This tumour may cause raised intraocular pressure. methods: A review of four cases of ring melanomas with insidious presentations seen in the ocular oncology service over a 12 month period. RESULTS: All four patients presented with unilateral anterior segment abnormalities and refractory glaucoma. The misdiagnoses of the causes of the glaucoma included angle recession from previous blunt trauma (patient 1); iridocorneal endothelial (ice) syndrome supported by endothelial specular microscopy (patients 2 and 3); and melanocytoma on ciliary body biopsy (patient 4). Two patients were treated by several cyclodiode ciliary body ablation treatments and the other two underwent trabeculectomies and Molteno tubes. Two of the four patients have since died from their disease. CONCLUSION: The ophthalmologist should re-evaluate the diagnosis in patients with anterior segment abnormalities and refractory ipsilateral glaucoma. Endothelial specular microscopy and biopsy of the suspicious lesion may give misleading reassurance. The potential presence of an anterior uveal melanoma must always be considered.
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3/17. Anterior uveal and episcleral metastases from carcinoma of the breast.

    breast carcinoma is the most common primary tumor producing intraocular metastasis. Metastases to the iris and ciliary body are relatively rare. The authors report a case of a 61-year-old lady, operated for carcinoma of the left breast 3 years back, who presented with symptoms and signs of acute narrow-angle glaucoma in the right eye. A diffuse whitish plaque-like mass in the upper nasal quadrant of the iris with an episcleral nodule on the limbus in the corresponding area and all the signs of acute narrow-angle glaucoma were present in the right eye. intraocular pressure was controlled medically. Fine-needle aspiration cytology from the episcleral nodule showed malignant cells. Histopathology of the excised nodule showed metastatic poorly differentiated carcinoma, and the cellular pattern was similar to the carcinoma of the breast. There was no other metastasis anywhere in the body. Fine-needle aspiration cytology from an external lesion of the eye is a less invasive and easier procedure than paracentesis to diagnose the metastatic nature of the lesions. The rare features in our case are the clinical presentation as acute glaucoma and the ocular structures being the first and only site of metastasis.
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4/17. Differential diagnosis of anterior chamber cysts with ultrasound biomicroscopy: ciliary body medulloepithelioma.

    PURPOSE: To describe a case with motile cyst in the anterior chamber in the right eye of a 7-year-old boy. methods: The right eye's visual acuity was 20/50. intraocular pressure was 59 mmHg. Slit-lamp examination showed prominent rubeosis iridis and a grey-white mass floating freely in the anterior chamber. Ultrasound biomicroscopy revealed a cystic mass in the anterior chamber. A diagnostic cyclectomy with removal of the anterior chamber cyst was performed. Histopathology of the anterior chamber lesion showed an intact cyst composed of medullary epithelial cells. Medulloepithelioma with malignant criteria was diagnosed and the eye was enucleated. RESULTS: pathology demonstrated an medulloepithelioma with a few mitotic figures and nuclear pleomorphisms within the ciliary body. The patient was followed for 8 months without any metastasis in the orbit or elsewhere. CONCLUSION: Intraocular medulloepithelioma is a rare embryonic benign or malignant neoplasm typically diagnosed in the first decade of life as a ciliary body mass. A dislodged, free-floating anterior chamber cyst associated with neovascular glaucoma is typical of medulloepithelioma in children. This unique presentation should be differentiated from congenital iris epithelial, post-traumatic, epithelial, parasitic and neoplastic cysts. Ultrasound biomicroscopy is useful for analysing the structure of the anterior segment mass. ciliary body medulloepithelioma is characterized by echogenic mass heterogeneity and an irregular surface containing multiple cystic cavities. Lack of glial differentiation may predict a better clinical outcome in primary neuroectodermal brain tumours.
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5/17. Mucinous adenocarcinoma metastatic to the iris, ciliary body, and choroid.

    A 67-year-old woman presented with signs of severe intraocular inflammation and secondary glaucoma. The initial diagnosis was uveitis, and an anterior chamber paracentesis with cytological study of the aspirate failed to establish an aetiological diagnosis. After three trabeculectomies had failed to control the intraocular pressure, the blind eye was enucleated. On histopathological examination a mucinous adenocarcinoma was found to cover diffusely the iris surface and to involve the ciliary body and peripheral choroid. The patient subsequently developed evidence of widespread metastatic disease and died shortly thereafter. Although a primary tumour was never found, histochemical and immunohistochemical studies of the enucleated eye suggested that the lesion originated in the gastrointestinal tract. In cases of intractable glaucoma and anterior chamber inflammation, metastatic carcinoma should be included in the differential diagnosis, and efforts should be made to substantiate the diagnosis by a systemic examination or a biopsy.
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6/17. iris melanoma with increased intraocular pressure. Differentiation of focal solitary tumors from diffuse or multiple tumors.

    A 70-year-old man presented with an apparent focal iris ciliary body mass and increased intraocular pressure. Fine needle aspiration biopsy 180 degrees from the main lesion demonstrated uveal melanoma cells in the angle. The eye was enucleated, and multiple separate iris and ciliary body melanomas were noted. This case demonstrates the usefulness of fine needle aspiration biopsy for both diagnosis and optimum therapy in selected cases.
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7/17. Malignant transformation of an iris melanocytoma. A case report.

    A 34-year-old Caucasian woman was diagnosed as having a pigmented iris tumor showing recent growth and satellite lesions. The tumor was associated with pigmentation of the anterior chamber angle and secondary unilateral glaucoma. After local excision, histopathologic studies revealed the plump polyhedral cells typical of melanocytoma. However, the examination of additional sections showed evidence of malignancy. The diagnosis of a melanocytoma that transformed into malignant melanoma was made and later confirmed by electron microscopic studies. Following surgical excision of the tumor, the eye maintained normal intraocular pressure. There was no evidence of recurrence 4 years after surgery.
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8/17. Simultaneous bilateral primary diffuse malignant uveal melanoma: case report with pathological examination.

    A 50-year-old Japanese housewife became blind owing to bilateral diffuse malignant melanoma of the choroid, ciliary body, and iris. These were histologically proved when enucleation was required because of blind painful eyes with raised intraocular pressures. She presented with reduced visual acuity and night blindness. Small scattered atrophic areas were observed between disc and macula on both sides, which fluorescein angiography showed were due to patchy atrophy of the retinal pigment epithelium. There was reduced amplitude in the electroretinogram. Inferior retinal detachments soon appeared and spread to become total. Pathological examination showed diffusely thickened choroid on both sides, due to infiltration by epithelioid and spindle-shaped malignant melanoma cells, which also affected the thickened ciliary body and iris diffusely. A carcinoma of the uterus successfully treated three years previously and the probably multicentric origin of the malignancy in each eye, and its bilateral occurrence, suggest a tumour-producing tendency in this patient, an interpretation which also applies to one of the other eight reported cases.
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9/17. Results of penetrating keratoplasty in the iridocorneal endothelial syndrome.

    Five patients (four men and one woman ranging in age from 30 to 41 years) with the iridocorneal endothelial syndrome (four with Chandler's syndrome and one with essential iris atrophy) underwent penetrating keratoplasty for complaints relating to diminished visual acuity or pain. After follow-up periods ranging from one year to four years and seven months (average, 2.7 years), postoperative visual acuities ranged from 20/15 to 20/30. No evidence of recurrence of the corneal abnormalities observed before grafting was apparent in the donor corneas at the last examinations. Two patients who required medical treatment for intraocular pressure control preoperatively also required adjuvant therapy postoperatively. These results suggested that penetrating keratoplasty is a relatively safe and effective procedure for patients with diminished vision or other complaints related to corneal abnormalities in the iridocorneal endothelial syndrome. It does not, however, restore to normal the iris and angle structures affected adversely by the progression of the corneal endotheliopathy and therefore other measures may be required to control intraocular pressure.
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10/17. iris hamartomas (Lisch nodules) in a case of segmental neurofibromatosis.

    iris hamartomas (Lisch nodules), previously reported in cases of peripheral neurofibromatosis, were found in a 14-year-old girl with segmental neurofibromatosis. The girl was in the 75th percentile for height (168 cm), the 90th percentile for weight (63 kg), and the 50th percentile for head circumference (55 cm). Her blood pressure was 105/70 mm Hg. Her visual acuity (20/20 and J 1), ocular motility, pupillary responses, visual fields, color vision, stereopsis, and intraocular pressure were within normal limits. Biomicroscopy showed several tan elevated nodular hamartomas on the anterior surface of the right peripheral iris but none elsewhere in the right eye and none in the left eye. cafe-au-lait spots and freckling were also limited to the right side of the body. The child had no palpable neurofibromas and was in good health and of normal intelligence. There was no family history of neurofibromatosis, multiple cafe-au-lait spots, axillary freckling, macrocephaly, or learning disabilities. The absence of iris hamartomas has been used to distinguish segmental neurofibromatosis from the peripheral and central or acoustic forms. Thus, their presence in this case is clinically significant. The segmental form not only produces fewer complications but carries less genetic risk. In segmental neurofibromatosis, the Lisch nodules would be expected to be unilateral rather than bilateral, ipsilateral to the side of the cutaneous involvement, and more frequently associated with contiguous cutaneous lesions.
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