Nikolaos Trichopoulos

Enucleation With Unwrapped Porous and Nonporous Orbital Implants: A 15-year Experience

Purpose: To compare the rates of implant exposure and implant migration among patients who received an unwrapped nonporous spherical implant versus an unwrapped porous spherical implant immediately after enucleation. Methods: Retrospective analysis of a series of 258 patients who received either an unwrapped nonporous spherical implant (n = 68) or an unwrapped porous spherical implant (n = 190). Actuarial rates of migration of the implant and conjunctival dehiscence leading to implant exposure were computed. Results: Sixty-eight patients received an unwrapped nonporous implant (polymethylacrylate [PMMA]) and 190 patients received an unwrapped porous implant (139 hydroxyapatite [HA] and 51 porous polyethylene [Medpor]). Median follow-up duration in this study was 37.6 months. Implant exposure occurred in 1 of the 68 nonporous implant cases (1.5%) and in 4 of the 190 porous implant cases (2.1%). This difference is not statistically significant (P = 0.85). In contrast, clinically significant implant migration occurred substantially more frequently in the patients who received a nonporous implant. The cumulative actuarial probability of implant migration at 60 months was 15.5% for the nonporous implants versus 0.7% for the porous implants. This difference was statistically significant (P = 0.0003). Conclusions: Orbital implant migration occurred in a significantly greater proportion of patients who received a nonporous implant than in those who received a porous implant. Implant exposure occurred at a low rate that was not significantly different in the two subgroups.

Size Overlap between Benign Melanocytic Choroidal Nevi and Choroidal Malignant Melanomas

PURPOSE To estimate size overlap between large choroidal nevi and small choroidal melanomas by using plotted frequency distributions of tumor size. METHODS Frequency distributions of largest linear basal diameter (LBD) and thickness (TH) of choroidal nevi and melanomas were plotted from published data and cases in the senior authors practice. Relative frequencies of choroidal nevi and melanomas were estimated from published data. Relative frequency distributions of the tumors were plotted to illustrate the extent of overlap between them. RESULTS Comparison of plotted frequency distribution curves for thickness indicated that there were approximately 125 nevi for every melanoma in the TH range 1.5 to 2 mm, approximately 25 nevi for every melanoma in the TH range 2 to 2.5 mm, and approximately 5 nevi for every melanoma in the TH range 2.5 to 3 mm. Similarly, comparison of the plotted frequency distribution curves for LBD of these tumor types indicated that there were approximately 70 nevi for every choroidal melanoma in the LBD range 5 to 6 mm, approximately 10 nevi for every melanoma in the LBD range 6 to 7 mm, and approximately 3 nevi for every melanoma in the LBD range 7 to 8 mm. CONCLUSIONS Because of the markedly greater cumulative lifetime incidence of choroidal nevi, the results of this analysis suggest considerable size overlap between larger nevi and smaller melanomas. Attempts to classify small melanocytic choroidal tumors clinically as benign nevi versus malignant melanomas on the basis of tumor size appear likely to result in multiple misclassifications.

Adenocarcinoma arising from congenital hypertrophy of the retinal pigment epithelium

BackgroundAdenocarcinoma 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).MethodsThe clinical features, surgical management, and histopathological features of a melanotic tumor arising from CHRPE are presented. The tumor was excised by transcleral resection.ResultsHistopathological and immunohistochemical study of the tumor showed it to be an adenocarcinoma of the RPE.ConclusionsAdenocarcinoma 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.

Neuroendocrine tumours metastatic to the uvea: diagnosis by fine needle aspiration biopsy

BackgroundUveal metastasis from a neuroendocrine tumour is rare and can simulate other primary or metastatic uveal tumours, both clinically and cytomorphologically. We describe four cases of uveal metastasis from a neuroendocrine tumour diagnosed by fine needle aspiration biopsy (FNAB).MethodsFour patients were referred for evaluation of a recently detected fundus mass. Two patients had a history of malignant, non-ocular, neuroendocrine neoplasms (Merkel cell carcinoma and lung carcinoid in one patient each). The third patient had a mediastinal mass that had been biopsied inconclusively, while the last patient reported a persistent cough.ResultsOphthalmic examination revealed an amelanotic ciliochoroidal mass in 2 cases and a lightly melanotic and a pale orange choroidal mass in 1 case each. Partial, non-rhegmatogenous, retinal detachment was present in 3 patients. Ocular ultrasonography revealed moderate to high internal reflectivity of the mass in 3 cases and low internal reflectivity in the 4th. Our differential diagnosis in all cases was metastatic carcinoma versus primary uveal melanoma. FNAB of the intraocular mass was performed in all patients to establish a pathologic diagnosis and guide subsequent management. Cytomorphology and immunohistochemical profiles of the aspirates were consistent with metastatic neuroendocrine neoplasms in all patients. Our final diagnosis was metastatic lung carcinoid in 2 patients and metastatic Merkel cell carcinoma and small cell lung carcinoma in 1 patient each. Immediately after FNAB, the intraocular tumour was treated by plaque radiotherapy (3 patients) or fractionated external beam radiotherapy (1 patient). All tumours treated regressed satisfactorily. Two patients expired due to widespread lung carcinoid 11 and 12 months after our initial evaluation respectively. The other two patients are still alive after 38 and 64 months respectively.ConclusionsNeuroendocrine tumours are a heterogeneous group of neoplasms whose diagnosis ultimately depends on the identification of specific cell markers (e.g., neuron-specific enolase, chromogranin, synaptophysin), hormones and neurotransmitters (e.g., gastrin, serotonin, adrenocorticotrophic hormone [ACTH]). FNAB with immunohistochemical stains for neuroendocrine markers can establish a pathologic diagnosis in cases of uveal metastasis from a neuroendocrine tumour. To our knowledge, our patient with Merkel cell carcinoma is the first pathologically proven case of uveal metastasis from this primary malignancy.

Plaque radiotherapy for choroidal and ciliochoroidal melanomas with limited nodular extrascleral extension.

BACKGROUND Currently available clinical information regarding management of posterior uveal melanomas complicated by nodular extrascleral extension is inadequate to determine the role, if any, for plaque radiotherapy in such patients. METHODS The authors performed a retrospective descriptive study of eight patients with a choroidal or ciliochoroidal melanoma complicated by nodular extrascleral extension who were treated by surgical excision of the extrascleral nodule followed immediately by plaque radiotherapy of the intraocular tumour. The calculated volume of the extrascleral nodule was greater than 1 mm3 but less than 1000 mm3 in all cases, and the intraocular tumour was deemed treatable by plaque radiotherapy in all patients. RESULTS Four of the eight patients died during available follow-up, three from metastatic melanoma and one from a second cancer. The median length of follow-up for the four surviving patients was 10.1 years. The actuarial 5-year and 10-year all-cause death rates were 37.5% and 53.1% respectively. One of the eight patients experienced local intraocular tumour relapse following plaque therapy and underwent secondary enucleation. None of the patients experienced orbital tumour recurrence or underwent secondary orbital exenteration. INTERPRETATION Our results coupled with previously published results from another centre suggest that plaque radiotherapy may be an effective local treatment for selected patients with choroidal or ciliochoroidal melanoma complicated by nodular extrascleral extension. The fact that none of the patients in this series or in the previously reported series experienced orbital recurrence following plaque radiotherapy or required secondary orbital exenteration suggests that plaque therapy may be better than enucleation alone in terms of these end points. These results should not be extrapolated, of course, to patients with massive extrascleral tumour extension or a choroidal or ciliochoroidal melanoma too large for plaque radiotherapy.

Prognostic implications of cytopathologic classification of melanocytic uveal tumors evaluated by fine-needle aspiration biopsy

PURPOSE Determine whether cytopathologic classification of melanocytic uveal tumors evaluated by fine-needle aspiration biopsy (FNAB) is a significant prognostic factor for death from metastasis. METHODS Retrospective analysis of cases of clinically diagnosed uveal melanoma evaluated by fine-needle aspiration biopsy from 1980 to 2006. Main outcome evaluated was death from metastasis. Associations between baseline clinical variables and cytopathologic classification were evaluated using cross-tabulation. Prognostic significance of cytopathologic classification was evaluated by Kaplan-Meier and Cox proportional hazards analysis. RESULTS Of 302 studied biopsies, 260 (86.1%) yielded sufficient cells for cytopathologic classification. Eighty of the 260 patients who had a sufficient specimen have already died (P=0.021), 69 from metastatic uveal melanoma. Cell type assigned by cytopathology was strongly associated with metastasis/metastatic death in this series (P=0.0048). Multivariate analysis showed cytopathologic classification to be an independently significant prognostic factor for metastatic death (P=0.0006). None of the 42 patients whose tumor yielded insufficient aspirates (sampled in at least two sites) have developed metastasis or died of metastasis thus far. CONCLUSION In this series, cytopathology of fine-needle aspiration biopsy samples obtained from uveal melanomas was strongly prognostic of death from metastasis. Insufficiently aspirates (2 or more sites sampled) proved to be prognostic of a favorable outcome (i.e., not developing metastasis).

Intraocular lymphoma diagnosed by fine‐needle aspiration biopsy

Acta Ophthalmol. 2010: 88: 705–710

Photodynamic therapy for recurrent hyphema after proton beam radiotherapy of iris melanoma

BackgroundRecurrent hemorrhage with secondary glaucoma is a rare but serious complication following proton beam irradiation (PBI) of iris melanoma. We report a case in which photodynamic therapy (PDT) was successfully used to treat this complication.MethodsThe history, clinical features and management of a patient with iris melanoma who was treated with PBI and later developed recurrent hyphema and increased intraocular pressure are presented. PDT was used to treat the abnormal intralesional blood vessels which caused the recurrent intraocular haemorrhage.ResultsThe intralesional blood vessels regressed following PDT. The patient did not experience any further episodes of hyphema or elevated intraocular pressure.ConclusionsOur case demonstrates that the vascular abnormalities following irradiation of iris melanoma may respond favorably to PDT in selected cases. To our knowledge, this is the first reported case in which PDT was used to treat recurrent hyphema caused by abnormal intralesional blood vessels after proton beam irradiation of iris melanoma.