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Purpose: The main purpose of treatment of advanced ocular surface and periocular malignant tumors is to eradicate the tumor while trying to preserve visual function and aesthetics. Our purpose is to describe the outcome of a retrospective case series of 10 patients with advanced ocular surface and periocular tumors treated surgically in first instance and then with postoperative interventional radiotherapy (IRT/Brachiterapy)., Materials and Methods: We describe the clinicopathological features, treatments and outcome, in a retrospective case series of 10 patients with advanced tumors involving ocular surface (staging ≥ T2) and eyelids (staging ≥ T3), with involvement of periocular and/or orbit tissues. Patients were first surgically treated, most of them with incomplete excision, and then underwent a post-operative interventional radiotherapy (IRT/Brachytherapy) as an alternative to more invasive and disfiguring surgical retreatment. Tumor location, risk factors, staging, histological features, and follow-up timing were analyzed., Results: Three patients had advanced eyelid basal cell carcinomas, 2 patients were diagnosed with eyelid and conjunctival squamous cell carcinomas, 3 as sebaceous carcinomas, and 2 as primary conjunctival melanomas. The mean follow-up time from IRT to last clinical follow-up was 58.6 weeks, range 28.4-168 (median 43.65, IQR 28.9-72.9). Two patients - one with ocular surface SCC, the other with conjunctival melanoma - had a local recurrence 23.4 and 40,9 weeks after IRT, respectively. An overview of the current knowledge on adjuvant or post-operative IRT is also provided., Conclusions: IRT can be considered an effective therapeutic option to avoid more invasive surgical retreatment in advanced tumors involving eyelids and ocular surface., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Purpose: To evaluate the effect of photodynamic therapy (PDT) as adjuvant treatment, after brachytherapy, in posterior amelanotic choroidal melanomas., Methods: Six patients with posterior amelanotic choroidal melanoma underwent brachytherapy treatment. Tumour response was assessed by fundus examination, fundus photography and A-B scan ultrasonography. The residual tumours were treated with adjuvant PDT performed with infusion of verteporfin intravenously at 6 mg/m 2 body surface area. Five minutes after infusion, a 689 nm laser was applied with a light dose of 100 J/cm 2 over an interval of 166 s., Results: At a median follow-up after brachytherapy of 17.5 months (IQR 16.2-22.5, range 5-42 months), tumours showed a partial reduction of tumour thickness (22.5% as compared to baseline value) and persistent low internal reflectivity at A-B scan ultrasonography. Supplementary photodynamic treatment resulted in complete resolution of the lesion with marked decrease of elevation . Mean decrease in thickness after PDT was 49.9% with respect to previous brachytherapy treatment 22.5% (p = 0.007). The results was achieved within a median period of 4 months (range 2-4 months) after PDT, and there has been no recurrence after a median follow-up of 84.7 months ± 18.7 (range 59 to 107 months)., Conclusion: Combined treatment of brachytherapy and adjuvant PDT in amelanotic uveal melanoma seems to be favourable with regard to complete and rapid tumour regression.

Primary malignant epithelial tumours arising from accessory lacrimal glands (ALGs) are extremely rare, with only few cases reported in literature. They generally appear as gradually increasing masses of the upper or the lower eyelid. Only one case of primary adenocarcinoma or adenocarcinoma not otherwise specified (ACNOS) from ALGs has been reported in literature. Herein, we describe a case of ACNOS arising from ALGs with an atypical clinical presentation and review prior cases of ALGs epithelial malignancies reported in the literature. A 78-year-old man referred to our Ocular Oncology Unit for adjuvant therapy after the excision of a conjunctival tumour of the left eye with a histological diagnosis of squamous cell carcinoma. He underwent topical chemotherapy with MMC and during follow up he presented a multinodular iris mass in his left eye. The MRI of the orbit showed an ocular mass infiltrating orbital soft tissues of the inferior palpebral region with an involvement of the corresponding zygomatic cutis. We performed orbital exenteration and histological studies revealed an epithelial neoplasm with a solido-glandular growth pattern with lumens containing an eosinophilic material positive for PAS and PAS-D. The immunohistochemical findings confirmed the diagnosis of adenocarcinoma NOS from ALGs. Although ALGs epithelial malignancies are extremely uncommon, they should be considered in the differential diagnosis of ocular tumours. A vigilant approach towards these entities is required, since they can be clinically insidious and locally aggressive.

Purpose: To report a case of macular choroidal osteoma treated with photodynamic therapy., Observations: A 34-years old woman with decreased visual acuity in her left eye came to our observation for assessment of an amelanotic choroidal tumor in the left eye. On the basis of ophthalmoscopic and echographic features the tumor was diagnosed as choroidal osteoma. Imaging examination revealed subretinal fluid involving the foveal area associated with alterations of outer neuroepithelial layers and photoreceptors without evidence of choroidal neovascularization. Foveal sparing standard fluence rate photodynamic therapy was performed. After treatment, subretinal fluid reabsorption and visual acuity recovery was noted with progressive restoration of foveal architecture. Due to the relapse of fluid and visual impairment, 1 year after treatment, a second PDT session was made using the same parameters and protocol of treatment. Despite a complete subretinal fluid reabsorption and visual acuity recovery the second treatment was complicated by the development of subretinal fibrosis., Conclusions: PDT is effective to induce subretinal fluid reabsorption and visual recovery in choroidal osteoma located in the macular area. However, the risk of possible complications related to the treatment have to be considered.

Background: Traditionally, surgical excision has been the treatment of choice for ocular surface squamous neoplasia (OSSN). Recurrences after surgery are high. To reduce the risk of recurrence, adjuvant therapies have been increasingly used., Purpose: We compared recurrences and complications of 3 forms of treatment for OSSN: surgical excision (group A), surgical excision plus adjuvant topical mitomycin C (MMC) (group B), and surgical excision plus subconjunctival interferon-α-2b (IFN-α-2b) (group C)., Methods: A retrospective comparative study was conducted between January 2006 and March 2016 at the Ocular Oncology Service of the Catholic University of Rome. Seventy-nine patients with a confirmed histological diagnosis of OSSN were included: 43 were treated with surgical excision (group A), 16 underwent surgical excision plus topical MMC (group B), and 20 underwent surgical excision plus adjuvant subconjunctival IFN-α-2b (group C)., Results: The recurrences were different in the 3 groups. Thirty-one recurrences (72%) were seen in group A, 5 (31%) were found in group B, and 3 (15%) were seen in group C. Eight (50%) patients who received MMC 0.02% complained of ocular discomfort, 10 (62.5%) presented conjunctival hyperemia, while conjunctival chemosis and corneal epitheliopathy were noticed in 2 (13%) and 2 (13%) patients, respectively. All patients treated with subconjunctival IFN-α-2b reported flu-like symptoms. Two patients (10%) complained of ocular discomfort., Conclusions: Our study revealed that OSSN is not always manageable with simple excision. Adjuvant chemotherapy is strongly advisable after surgery to reduce recurrences. Interferon injections and MMC drops are effective in preventing recurrences and should be administered after surgery.

Purpose: To determine genetic features of a pediatric uveal melanoma in a 6-year-old girl by array-based comparative genomic hybridization (a-CGH) and assess prognosis, and to search for constitutional copy number variations (CNVs) encompassing oncosuppressor genes., Methods: High-resolution a-CGH was performed on genomic DNA from cancer cells and from peripheral blood cells. Histopathology and clinical staging of the tumor were simultaneously assessed., Results: Array-based CGH revealed no CNVs on tumor cells associated with poor prognosis; namely, no monosomy 3, losses of 1p, 6q, or 8p, and no gains of 8q. A unique genomic profile was observed, consisting mainly of partial terminal duplications affecting chromosomes 1, 4, 5, 9, 10, 11, 16, and 19, and complete trisomy of chromosomes 6, 7, and 20. The nonmetastatic tumor had predominantly epithelioid histology. No constitutional CNVs encompassing oncosuppressor genes were detected., Conclusions: We report a very rare uveal melanoma characterized by low-risk genomic profile and poor prognostic histologic and clinical features. The child is relapse-free at 1-year follow-up. The unusual CNVs detected by a-CGH suggest specific pathogenic mechanisms.