Iwona Rospond-Kubiak

Management of patients with uveal metastases at the Liverpool Ocular Oncology Centre

Background Uveal metastasis is the most common intraocular malignancy. Methods This was a retrospective study of all patients with uveal metastases referred to the Liverpool Ocular Oncology Centre between January 2007 and December 2012. Biopsy was performed as a primary investigation if the clinical examination suggested metastasis with no evidence of any extraocular metastases. Results Ninety-six patients (109 eyes) were included. Breast and lung carcinomas were the most common primary malignancies, affecting 41 and 27 patients, respectively. The median time interval between detection of primary cancer and uveal metastasis was 24 months (range 1–288 months). Thirty-nine patients underwent ocular biopsy, confirming the diagnosis in all patients. The biopsy indicated the site of origin in 24 out of the 27 without a known primary tumour. In 7 of these 27 cases, previous systemic investigations had failed to identify the primary tumour. Seventy-three patients received external beam irradiation; two patients received photodynamic therapy; and two patients had Ru-106 plaque radiotherapy. The visual acuity was stable or improved in 75.5% of the cases. Conclusions Immediate biopsy provides a quick diagnosis that may expedite treatment and improve any opportunities for conserving vision while facilitating the general oncologic management on these patients.

The Pediatric Choroidal and Ciliary Body Melanoma Study: A Survey by the European Ophthalmic Oncology Group

PURPOSE To collect comprehensive data on choroidal and ciliary body melanoma (CCBM) in children and to validate hypotheses regarding pediatric CCBM: children younger than 18 years, males, and those without ciliary body involvement (CBI) have more favorable survival prognosis than young adults 18 to 24 years of age, females, and those with CBI. DESIGN Retrospective, multicenter observational study. PARTICIPANTS Two hundred ninety-nine patients from 24 ocular oncology centers, of whom 114 were children (median age, 15.1 years; range, 2.7-17.9 years) and 185 were young adults. METHODS Data were entered through a secure website and were reviewed centrally. Survival was analyzed using Kaplan-Meier analysis and Cox proportional hazards regression. MAIN OUTCOME MEASURES Proportion of females, tumor-node-metastasis (TNM) stage, cell type, and melanoma-related mortality. RESULTS Cumulative frequency of having CCBM diagnosed increased steadily by 0.8% per year of age between 5 and 10 years of age and, after a 6-year transition period, by 8.8% per year from age 17 years onward. Of children and young adults, 57% and 63% were female, respectively, which exceeded the expected 51% among young adults. Cell type, known for 35% of tumors, and TNM stage (I in 22% and 21%, II in 49% and 52%, III in 30% and 28%, respectively) were comparable for children and young adults. Melanoma-related survival was 97% and 90% at 5 years and 92% and 80% at 10 years for children compared with young adults, respectively (P = 0.013). Males tended to have a more favorable survival than females among children (100% vs. 85% at 10 years; P = 0.058). Increasing TNM stage was associated with poorer survival (stages I, II, and III: 100% vs. 86% vs. 76%, respectively; P = 0.0011). By multivariate analysis, being a young adult (adjusted hazard rate [HR], 2.57), a higher TNM stage (HR, 2.88 and 8.38 for stages II and III, respectively), and female gender (HR, 2.38) independently predicted less favorable survival. Ciliary body involvement and cell type were not associated with survival. CONCLUSIONS This study confirms that children with CCBM have a more favorable survival than young adults 18 to 25 years of age, adjusting for TNM stage and gender. The association between gender and survival varies between age groups.

Primary uveal lymphoma effectively managed with oral chlorambucil: a case report

IntroductionOcular lymphomas account for five to 10 percent of all extra-nodal lymphomas. Primary uveal lymphoma is quite a rare entity and usually unilateral. We present a case of a primary uveal lymphoma with conjunctival and orbital extension, successfully managed with oral chlorambucil.Case presentationA 71-year-old Caucasian man presented to our facility with visual loss in his only functioning eye (left). On clinical examination, we found a conjunctival lesion with a choroidal infiltration and a secondary retinal detachment. Ultrasound and magnetic resonance imaging studies revealed a choroidal tumour mass and two other lesions around the optic nerve. Results from an incisional biopsy revealed a low-grade B-cell lymphoma (CD20+, CD43+, bcl2+, CD3-). A diagnosis of primary uveal lymphoma was made. Our patient was started on a chemotherapy regime with no effect, and then oral chlorambucil was administered, with a relatively good result. At 10 months after the start of chlorambucil treatment, a best-corrected visual acuity of 0.4 was recorded, the choroidal mass had practically disappeared and the extra-ocular lesions had shrunk.ConclusionsIn all, 61 to 80 cases of primary uveal lymphoma have already been described in the literature. Generally, it is an indolent tumor with a good prognosis. However, there are some reports of aggressive tumor behavior a few years after initial diagnosis (about eight percent of cases). Other treatment options are orbital irradiation at low doses (20 to 40 Gy) or steroid administration. This is the first documented report of the efficacy of oral chlorambucil in the treatment of primary uveal lymphoma.

Small-cell lung carcinoma metastasis to the iris – case presentation

Introduction Metastatic tumors are the most common intraocular malignancies in adults. Anterior segment metastases account for about 7.2–14.1% of all intraocular metastatic tumors with those to the iris representing 3.6–7.8% of the cases. Breast and lung are the most frequent primary sites of metastasizing cancer. The aim of this study is to present the case of a 43-year-old patient with a rare small-cell lung carcinoma metastasis to the iris. Case report A patient with small-cell lung carcinoma and metastases in the brain who had been treated with chemo- and radiotherapy attended an outpatient clinic because of redness of her left eye. Based on the ophthalmological examination, a diagnosis of numerous metastatic tumors to the iris was made and the patient was referred for re-chemotherapy. Complete regression of the iris lesions was observed after 5 months but unfortunately two months later the patient died, due to progression of her systemic disease. Discussion Among lung cancers, adenocarcinomas are much more likely to produce distant metastases to the iris than small-cell carcinomas [4]. More than 50% of patients with small-cell lung carcinoma have metastases in the brain. The survival rate in cases of lung cancer with eye involvement is poor and approximates to 7 months. However, since there is a strong probability of angle involvement and subsequent secondary glaucoma, the early onset of treatment can improve the patients quality of life during the terminal phase of the disease.

Poststreptococcal syndrome mimicking conjunctival lymphoma

BackgroundPoststreptococcal syndrome (PSS) can be a consequence of nonpurulent primary infection with group A streptococci (GAS). Postreptococcal uveitis is a well recognized entity with quite a few descriptions in the literature, but so far no conjunctival involvement has been reported.The aim of the study is to present a rare case of postreptococcal conjunctival lesions mimicking a lymphoma.Case presentation19-years-old Caucasian female presented with pink, nodular infiltrates in the right conjunctiva that occurred a few months after upper respiratory tract infection and tonsillectomy. Histopathological examination of collected lesion samples revealed inflammatory reaction with lymphocytes proliferation and failed to rule out a myeloma. Complementary flow-cytometry did not show monoclonal proliferation of lymphocytes B. During follow-up we observed the complete regression of conjunctival lesions after the benzyl penicillin treatment prescribed by ENT specialist due to elevated plasma ASO levels. Therefore, we suppose that those lesions must have represented a part of poststreptococcal syndrome.ConclusionsTo conclude, this is, to the best of our knowledge, the first report of conjunctival involvement in the course of PSS related to group A streptococci.

Ruthenium brachytherapy for intraocular growth of a conjunctival squamous cell carcinoma: a case report

The aim of this study is to report the use of ruthenium brachytherapy as an adjunctive treatment in the management of an intraocular extension of the conjunctival squamous cell carcinoma (SCC). In 2013, a 73-year-old man presented with a tumor blocking the angle and infiltrating corneal endothelium. The patient was treated with iridectomy, and after one month, ruthenium brachytherapy was applied. 4.5 years post-irradiation, the treated eye is painless, and no recurrence was noted. We advocate the use of ruthenium brachytherapy as an eye salvage treatment with an intraocular extension of conjunctival SCC.

Demodex spp. within the limbal dermoid – causal pathogen or an irrelevant finding? Case report

The aim of the study is to report a case of limbal dermoid associated with infestation of Demodex species (Demodex spp.). A 27-year-old man presented with ocular discomfort and a pink conjunctival lesion in his left eye. The lesion had been present since birth and it occassionally grew eyelashes. The tumour was located in an inferior temporal quadrant, involving approximately 1/6 of the limbal surface area. High frequency ultrasound revealed deep corneoscleral inifiltration, so a subtotal resection was performed. Histological evaluation revealed the presence of Demodex spp. in an abnormal sebaceous gland located within the tumour. Limbal dermoid might be associated with Demodex spp. infestation, if abnormal sebaceous glands are formed inside the lesion.

Ruthenium brachytherapy for uveal melanoma – single institution experience

Purpose The aim of this study was to report on results of uveal melanoma treatment with ruthenium-106 (106Ru) brachytherapy with long-term follow-up, in terms of local tumor control, eye retention rate, radiation retinopathy, and patients’ survival. Material and methods Medical records of patients treated with ruthenium plaque due to uveal melanoma at the Department of Ophthalmology, Poznan University of Medical Sciences, Poland, between 1994 and 2014 were retrospectively reviewed. Results We identified 126 patients: 53 men, 73 women, mean age 60.04 years (range, 21-89). The largest basal diameter ranged from 4.04 mm to 18.9 mm (median, 10.67 mm), tumor height was 1.9 mm to 7.42 mm (median, 4.8 mm). Median scleral radiation dose was 570 Gy (range, 235-1,500 Gy), median apical dose 100 Gy (range, 60-129 Gy). Median follow-up was 66.5 months (range, 2-261 months). We noted a total of 19 (15%) recurrences. The actuarial rate of recurrence was 9.5% at 3 years, and 13.5% at 5 years postoperatively. Nine (7%) eye globes were lost, median time to enucleation was 5 years. The eye retention rate at 5 years was 92.7% and 81% at 10 years. Forty-three (34%) patients died before the end of the study, 24 (19%) of them due to metastatic disease. Metastatic death was related to: tumor size and TNM stage at presentation (p = 0.002 vs. p = 0.0006, respectively) but not to age, gender, and plaque dosimetry. Conclusions 106Ru brachytherapy is an effective, globe sparing treatment that provides good tumor control and a high rate of survival. However, some ocular complications tend to appear late post-treatment, and therefore long-term follow-up is advised.

Core Endoresection as Neoadjuvant Treatment of Uveal Melanoma

Aim: To present a case of neoadjuvant treatment of uveal melanoma with a limited core endoresection, which enabled ruthenium plaque radiotherapy to be administered. Material and Methods: A 77-year-old woman presented in 2005 with a ciliochoroidal uveal melanoma measuring 7.2 by 6.5 mm basally with a thickness of 6.2 mm. The vision in the affected eye was 6/9. Because of diagnostic uncertainty, we performed a biopsy, which produced a copious sample. The biopsy serendipitously reduced the tumour thickness to 4.4 mm, making ruthenium plaque brachytherapy possible. Results: Histopathological examination showed the tumour to be a melanoma predominantly of spindle cell type. Postoperative complications included a dense vitreous haemorrhage, which cleared spontaneously, and raised intraocular pressure, controlled with latanoprost. Seven years after brachytherapy, the vision was 6/5 and the thickness of the residual tumour was 2.5 mm. Nine years after treatment, there was no evidence of any intra- or extraocular seeding or distant metastases. Conclusions: Core endoresection of a ciliochoroidal melanoma may reduce tumour thickness enough to allow subsequent ruthenium plaque radiotherapy.

Conjunctival metastasis of a renal cell carcinoma in a 59-year-old man – clinicopathological case report

The aim of the study was to present a rare case of conjunctival metastasis of a renal cell carcinoma. A 59-year-old man presented in 2012 with rapidly growing, painless, and occasionally bleeding right eye conjunctival tumour. The patient had a history of advanced renal cell carcinoma diagnosed 3 weeks previously. The conjunctival tumour was resected, and histopathological report confirmed the working diagnosis of a metastatic tumour. The patient received palliative radiation to the central nervous system but died from disseminated disease 8 weeks later. Conjunctival metastasis of a renal cell carcinoma is exceedingly rare. It appears in a stage of disseminated disease.