Iris ring melanoma: fine needle biopsy.

AIMS: To delineate the diagnostic accuracy of fine needle biopsy in iris ring melanoma and determine the tumour related mortality of this neoplasm. METHODS: A retrospective analysis of 22 patients with iris melanomas that involve the entire 360 degrees of the anterior chamber angle. RESULTS: Iris ring melanomas were correctly diagnosed in all cases. In 11 of 16 cases (69%) a fine needle biopsy performed 180 degrees away from the main mass was positive for an iris ring melanoma. The tumour related mortality in iris ring melanoma cases was four of 22 patients (18%). Actuarial survival analysis showed a 10 year mortality (Kaplan-Meier) of 15%. CONCLUSION: A fine needle aspiration biopsy can be used to diagnose an iris ring melanoma. Iris ring melanomas have significant mortality compared with focal tumours.

Sympathetic ophthalmia complicating helium ion irradiation of a choroidal melanoma.

Sympathetic ophthalmia was diagnosed 49 months after helium ion irradiation of a left choroidal melanoma. The patient maintained good vision until 18 months after therapy, when she developed neovascular glaucoma. This complication required multiple therapeutic procedures, including topical anti-inflammatory and antiglaucomatous drops, 360 degrees peripheral panretinal cryoblation, and a single 180 degrees application of inferior cyclocryotherapy over a 2 1/2-year period. Four weeks after the cyclocryotherapy, inflammation was noted in both eyes, and, one month later, enucleation of the left sympathogenic eye was performed. Serial histopathologic sections showed a full-thickness, fibrovascular, scleral scar and tantalum marker ring suture without uveal incarceration. Penetrating surgical trauma, a uveal melanoma, and multiple nonpenetrating treatments resulted in the development of sympathetic ophthalmia.

Combined nevi of the conjunctiva.

OBJECTIVE: To report the clinical and histologic features of combined nevi of the conjunctiva, a type of nevus that is not uncommon in the skin but rarely has been reported in the conjunctiva. METHODS: Conjunctival nevi and melanomas in the files of the University of California at San Francisco Eye Pathology Laboratory from 1984 to 1999 were reviewed for the presence of features of both standard nevocytic nevi and blue nevi. Clinical histories and, when available, clinical photographs were obtained. RESULTS: Thirty combined nevi were discovered between 1984 and 1999. One case before 1984 had been incorrectly diagnosed as a junctional nevus. The dendritic and spindle-shaped blue nevus cells had been overlooked because they were not recognized as distinct from the standard nevocytic nevus cells. The recognition of blue as well as a brown color, a deep as well as a superficial component in the lesion, or a history of pigmentation since birth may help to establish the correct clinical diagnosis and prevent an unnecessarily deep surgical resection. Although growth of the lesion or "satellites" in some may favor a clinical diagnosis of melanoma, no lesions in this series were malignant. CONCLUSION: Despite a paucity of reports of combined nevi of the conjunctiva in the medical literature, this type of nevus, a combination of a nevocytic and a blue nevus, is common and has been overlooked in the past.

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.

Spontaneous regression of a choroidal melanoma.

Although spontaneous regression of uveal malignant melanoma is rare, its occurrence is not uncommon in cutaneous melanomas. We report a 15-year follow-up of an enlarging posterior pigmented tumor. It initially appeared to spontaneously regress into a flat chorioretinal scar, but, ten years later, it grew markedly and the eye was enucleated. Histopathologic changes similar to those found in regressed cutaneous melanomas were located in the region of the chorioretinal scar.

Failure of choroidal melanoma to respond to helium ion therapy.

Helium ion irradiation is a promising alternative therapy for choroidal melanoma. In short-term follow-up (less than 5 years), more than 90% (18/19) of treated patients demonstrated tumor regression. We had to enucleate five eyes after helium ion therapy either because of continued tumor growth (four patients) or other complications (one patient). Two melanomas continued to grow and seemed to be radioresistant. In two other tumors it retrospectively seemed that the entire lesion was not inside the radiation field. In one patient total retinal detachment and glaucoma developed; enucleation was performed because of a painful eye. Metastatic disease developed in no patients. The treatment failures emphasize that there are a number of unresolved issues regarding the use of charged-particle irradiation in the treatment of melanoma. Further studies must be performed to answer these questions and better delineate the use of these newer forms of therapy.

Metastatic ciliary body carcinoid tumor.

A 50-year-old man with a history of systemic carcinoid had decreased right eye vision and a darkly pigmented cilio-choroidal mass. Results of ultrasonographic and fluorescein angiographic examinations were consistent with a malignant melanoma. Fine-needle aspiration biopsy of the lesion identified it as a carcinoid metastasis. The patient refused both radiation and enucleation; complete excision with a cyclochoroidectomy was performed. Histopathologic examination showed a carcinoid neoplasm covered by marked hyperplasia of the retinal pigment epithelium that produced the pigmented appearance of the lesion.

Iridocyclitis masquerade syndrome.

A 52-year-old man presented with blurred vision and a red, painful eye. There was a marked anterior uveitis associated with a solid white lesion on the iris. The differential diagnosis and appropriate diagnostic workup are discussed. The diagnosis of adenocarcinoma of the lung with metastasis to the iris was made.

A histopathologic study of the position of the shearing intraocular lens in the posterior chamber.

Three human eyes that had successfully undergone Shearing posterior-chamber intraocular lens implants were examined post mortem. Supporting loops of these lenses were located deep within the ciliary body adjacent to a major ciliary artery (the superior loop in two cases), within the ciliary sulcus (the superior loop in one case), within the lens capsule bag (the inferior loop in two cases), and embedded within the iris stroma (the inferior loop in one case).

Ocular vasodilation and angiogenesis in Potter's syndrome.

At autopsy we examined eight eyes of six infants with Potter's syndrome, an idiopathic multisystem condition with a constellation of ocular anomalies, to explore its ocular component more fully and systematically. A striking abnormality in some cases was the presence of dilated intraocular blood vessels, and these were sometimes associated with retinal and preretinal neovascularization reminiscent of the vasoproliferative stage of retrolental fibroplasia. Since oxygen therapy cannot be implicated in these cases, other causes, either genetic or environmental, must be sought. The presence of nonretinal ocular vascular anomalies, such as iris capillary dilation, peripapillary angioma formation, and vascular anomalies in nonocular tissue, point to a generalized derangement in the control of ocular angiogenesis. Our study indicated that an understanding of the pathogenesis of the vascular anomalies in Potter's syndrome is clearly relevant to other more common retinopathies that are associated with neovascularization.

Cytopathologic diagnosis of benign lesions simulating choroidal melanomas.

In three cases of benign pigmented lesions (one melanocytoma and two pigment epithelial adenomas) there was evidence of tumor growth and the lesions were referred to us as uveal melanomas. The fine needle aspiration biopsy specimens were correctly interpreted in the operating room as being benign tumors. The pigment granules in these benign pigmented lesions are much larger than are observed in uveal melanomas. When they were visible through the heavy pigmentation, the cellular detail appeared benign. In two cases the tumors were successfully resected with cyclochroidectomy techniques and the visual outcome was good. The third eye was studied after it had been removed at another institution. Fine needle aspiration biopsy can often differentiate a benign-simulating pigmented lesion from uveal melanoma.

Benign mixed tumor (pleomorphic adenoma) of the lacrimal gland in a 6-year-old boy.

PURPOSE: To report a 6-year-old boy who had benign mixed tumor (pleomorphic adenoma) of the lacrimal gland. METHODS: A 6-year-old boy was referred for evaluation of a lacrimal fossa mass. We performed a lateral orbitotomy and excised the mass. RESULTS: Histopathologic evaluation of the excised orbital mass disclosed a benign mixed tumor (pleomorphic adenoma) of the lacrimal gland. CONCLUSION: Benign mixed tumors (pleomorphic adenomas) of the lacrimal gland are rare in children. However, they must be considered in the differential diagnosis of pediatric orbital masses because of the prognostic importance of removing a pleomorphic adenoma with the capsule intact.

Failure of preenucleation radiation to decrease uveal melanoma mortality.

We analyzed uveal melanoma metastases in a group of 41 patients who received 20 Gy of preenucleation radiation in a Northern California Oncology Group preliminary phase I/II study, and compared their survival rates with a retrospective control group of 31 patients with characteristics matching the entrance criteria but treated with enucleation alone. Using the Cox proportional hazards model, we found that increased tumor diameter, mixed or epithelioid cell type, and radiation adversely affected survival. In vivo studies of cell cycling indicated that 20 Gy of preenucleation radiation appeared to diminish the reproductive integrity of the tumor cells. It is most likely that the failure of preenucleation irradiation to prolong patient survival was because of micrometastases that occurred before treatment.

Foreign-body giant cell reaction to liquid silicone.

Although intravitreal injections of liquid silicone have long been used in complicated retinal detachments and long-term complications such as keratopathy, glaucoma, and cataract are well-known, the effect of liquid silicone on the retina, iris, and angle structures is not as well defined. Histopathologic study of one human eye that was enucleated 20 months after liquid silicone injection showed an intraocular foreign-body giant cell reaction.

Uveal melanoma necrosis after helium ion therapy.

A 71-year-old man underwent enucleation because of intractable pain ten months after receiving helium ion therapy for a uveal melanoma. There was no clinical or ultrasonographic evidence of tumor regression but histologically the tumor appeared almost entirely necrotic.

Orbital melanocytic hamartoma.

A male Chinese neonate had a melanocytic hamartoma that involved the entire orbit and the eye, but did not involve the orbital bones or eyelid skin. Although usually benign, these hamartomas may be locally invasive, and rare cases of malignant transformation have occurred.

Correlation between degree of malignancy and the radioactive phosphorus uptake test in ocular melanomas.

Twenty-nine patients who had undergone enucleation for ocular malignant melanoma were studied. The results of the radioactive phosphorus uptake (32P) tests were correlated with histopathologic observations. There was a positive correlation between the 32P test results and the histologic degree of malignancy. Mixed cell melanomas were more frequent in the patients ranked with higher 32P test results. Spindle cell melanomas occurred more frequently in patients with lower 32P test results. Mitoses per field and degree of malignancy correlated positively with 32P test results when ranked in a masked manner by two ocular pathologists. Tumor volume and clinical factors did not correlate with the 32P test results. While patients with mixed cell melanomas had higher mean 32P test results than those with spindle cell melanomas, a large variance was noted. Because of this the 32P test is unreliable in predicting the histopathology of a tumor in individual cases.

Analysis of radiation failure after uveal melanoma brachytherapy.

We analyzed the number of cycling cells in the DNA synthesis phase and in mitosis in two uveal melanomas unsuccessfully treated with iodine 125 brachytherapy. In one patient, the tumor appeared to have been radioresistant, whereas the other patient had a clinically inapparent area of tumor that was not included in the tumoricidal radiation field. In the first patient, there was uniform tumor cell cycling, whereas the second patient had cycling cells predominantly in the area that did not receive optimal radiation. This gradient of cycling tumor cells in the second patient was observed with both standard histologic examination (mitoses per high-powered field) and bromodeoxyuridine staining (DNA synthesis). The bromodeoxyuridine technique was significantly more sensitive than standard histologic examination in the detection of cycling cells.

Diagnostic modalities in choroidal melanoma.

We prospectively studied 51 patients with choroidal melanomas in a masked manner to determine the accuracy of clinical, ultrasound, and fluorescein diagnosis as well as the accuracy of tumor size measurements. In patients with clear media, clinical diagnosis was the most accurate means of detecting a choroidal melanoma. There was excellent correlation between clinical, ultrasound, and pathology measurements of tumor size. In small melanomas, clinical examination was the most accurate means of measuring tumor diameter and ultrasound the most accurate method of measuring tumor height. Even with a trained ocular oncologist, there was a significant variability in tumor measurements on serial ophthalmoscopic examinations.

Uveal tumour resection.

AIM: To review the ocular retention rates, visual results, and metastases in uveal tumours managed with eye wall resection techniques. METHODS: This was a retrospective analysis of consecutive local uveal tumour resections performed by a single surgeon. All enucleation specimens were reviewed by one author. Both parametric and non-parametric analysis of data were performed. RESULTS: 138 eyes were scheduled for eye wall resection surgery. The mean age was 52 years (range 11-86 years). Tumours involved predominantly the iris in 14 cases, iris-ciliary body in 57, ciliary body alone in 18 patients, and in 49 cases the choroid was involved (ciliochoroidal, iris-ciliary body-choroid, or choroid). 125 eyes harboured melanomas; posterior tumours were more likely to have epithelioid cells (p<0.05). The mean follow up was 6 years. The mean clock hours in iris and iris-ciliary body tumours was 3.5. In tumours that involved the choroid the mean largest diameter was 12.9 mm and the mean thickness 8.5 mm. 105 of 138 (76%) eyes were retained. Histological assessment of surgical margins did not correlate evidence of tumour in enucleated eyes or metastatic disease. Surgical margins of more anterior tumours were more likely to be clear on histological evaluation (p<0.05). Approximately 53% of retained eyes had a final visual acuity of > or =20/40; visual results were significantly better in more anteriorly located tumours (p<0.05). All retained iris tumour cases had > or =20/40 final visual acuity. In tumours that involved the choroid nine of 31 retained eyes kept that level of visual acuity. Eight patients developed metastases; all metastatic events developed in patients with tumours that involved the choroid, and seven of eight were mixed cell melanomas. CONCLUSIONS: 76% of eyes were retained and 53% of these had a final visual acuity of > or =20/40. Only 7% of uveal melanoma patients developed metastatic disease with a mean follow up of 6 years. Survival did not appear to be compromised with eye wall resection and in very thick, more posterior melanomas it appeared that ocular retention and visual results were better than with radiation alone.