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Purpose: To describe the cases of conjunctival melanoma (CM) and report the disease-free interval (DFI) and overall survival (OS)., Methods: The charts of 22 patients who were admitted to two hospitals between 1985 and 2006 were reviewed for pertinent data, including demographics, site of involvement in the conjunctiva and sub-sites, surgical treatment, and adjuvant treatment., Results: There were 10 (45.45%) males and 12 (54.55%) females. Mean age was 52.3 years. In this group, 15 patients (68.1%) involved the bulbar conjunctiva, and 7 (31.9%) involved the palpebral conjunctiva. Of the 22 patients, 72.72% had a history of conjunctival melanosis. The average tumor size was 20.4 mm. Eight (36.36%) patients underwent orbital exenteration, 2 (9.06%) had enucleation, 5 (22.72%) had wide excision of the lesion followed by radiotherapy, 2 (9.06%) had orbital exenteration with neck dissection, and the remaining 5 patients (22.72%) were considered adequately treated only with wide excision. Eight (36.36%) patients received adjuvant treatment. Disease-free survival at 5 years was 51% and the overall survival at 5 and 10 years was 50% and 37%, respectively., Conclusion: Conjunctival melanoma is a rare entity. Tumor behavior is aggressive, and the optimal treatment is surgery with adjuvant therapy.

Conjunctival melanoma is a rare tumor, while malignant melanoma of the uveal tract is the most common primary intraocular tumor in adults. The authors highlighted the case of a 68-year-old male patient presented in June 2011 in the Ophthalmology Clinic of the Emergency County Hospital of Craiova, Romania, accusing foreign body sensation and the appearance of a tumor in the lower fornix of his right eye. The patient was clinically and paraclinically investigated, and clinically diagnosed with de novo malignant melanoma. The tumor was surgically excised and the lower fornix reconstructed with oral mucosa self-graft. Histopathological diagnosis was de novo, invasive, ulcerated malignant melanoma of the conjunctiva. Ki-67 immunohistochemical staining was also performed. After surgery, the patient was sent to the Oncology Department for specific treatment. Despite the histological poor prognosis factors present in our patient, two years after surgery and chemotherapy, the patient had no clinical sign of local or distance recurrent disease. Continuous ophthalmologic and oncological surveillance is necessary.

The development of optical coherence tomography (OCT) technology has helped to usher in a new era of in vivo diagnostic imaging of the eye. The utilization of OCT for imaging of the anterior segment and ocular surface has evolved from time-domain devices to spectral-domain devices with greater penetrance and resolution, providing novel images of anterior segment pathology to assist in diagnosis and management of disease. Ocular surface squamous neoplasia (OSSN) is one such pathology that has proven demonstrable by certain anterior segment OCT machines, specifically the newer devices capable of performing ultra high-resolution OCT (UHR-OCT). Distinctive features of OSSN on high resolution OCT allow for diagnosis and differentiation from other ocular surface pathologies. Subtle findings on these images help to characterize the OSSN lesions beyond what is apparent with the clinical examination, providing guidance for clinical management. The purpose of this review is to examine the published literature on the utilization of UHR-OCT for the diagnosis and management of OSSN, as well as to report novel uses of this technology and potential directions for its future development., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Purpose: To evaluate the current standard of care of ophthalmologists who are likely to encounter ocular surface squamous neoplasia (OSSN) in their practices and to compare this with data gathered in 2003., Methods: Invitations to a web-based survey were sent to members of the Ocular Microbiology and Immunology Group. In addition, the survey was posted to the Cornea Society Listserv, Keranet. The survey contained questions regarding the surgical and medical management of OSSN and postcare follow-up. The results of this survey were compared with the results of a 2003 survey, where possible, to identify the areas of change in the standard of care., Results: Eighty-one ophthalmologists participated in the survey. Seventy-nine percent of the responders thought that there was enough evidence for topical monotherapy in OSSN, but only 58% reported ever using topical agents as monotherapy. First-line topical therapy was interferon α2b (56%) followed by mitomycin C (MMC) (37%). A shift from surgical excision alone to excision followed by topical therapy was seen when comparing the 2012 survey to the 2003 survey. The preferred postexcision topical agent was MMC. Seventy-five percent of responders evaluate their patients every 2 to 4 months during the first 2 years., Conclusions: Topical agents were being used more in 2012, either in combination with surgical excision or as monotherapy, compared with those used in 2003. Interferon has replaced MMC as the agent most used for topical monotherapy, possibly because of a favorable safety and tolerance profile. Prospective randomized trials are needed to better define the ideal practice patterns.

Background: In order to evaluate clinical and pathological characteristics of ocular surface squamous neoplasia (OSSN) in Asian population., Methods: Medical records were reviewed of 30 eyes of 30 Korean patients who underwent en-bloc excision and biopsy for tumors in the corneal and conjunctival epithelium in the suspicion of OSSN., Results: Tumors developed predominantly in males (21/30 patients, 70%) at the mean age of 64.8 years (range: 39-82 years). Histologic examination revealed that 11 cases were squamous cell carcinoma (SCC), eight were conjunctival or corneal intraepithelial neoplasm (CIN) with dysplastic cells in the epithelium, eight simple hyperplasia, and three inflammatory lesions. The majority of SCC (9/11 eyes, 81.8%) involved the nasal conjunctiva and cornea, while one of eight eyes (12.5%) with CIN was located at the nasal side. Vascularization was combined in all eyes with SCC, in two of eight (25%) with CIN, in two of three with inflammation, and in none with hyperplasia. Both cornea and conjunctiva were involved in ten of 11 eyes (90.9%) with SCC, in six of eight (75%) with CIN, in two of eight (25%) with hyperplasia, and in two of three (66.7%) with inflammation. When classified based on clinical appearance, seven of 11 eyes (63.6%) with SCC were papilliform sessile masses, and four of eight (50 %) with CIN showed gelatinous growth onto the surface. All lesions were treated with en-bloc excision and cryotherapy. Recurrence occurred in seven out of 19 patients (36.8%) with SCC or CIN during the mean follow-up of 30.0 months-four patients with SCC and three with CIN. Hyperplasia or lesions with inflammation did not recur. The recurrence rate was not associated with histological diagnosis of a lesion. However, the use of the postoperative chemotherapy including topical mitomycin C or interferon α 2b had a significant negative correlation with the recurrence., Conclusions: In total, 36.7% of tumors in the corneal and conjunctival epithelium were SCC, and 26.7% were CIN. Recurrence occurred in 36.8% of patients with SCC or CIN after primary excision and cryotherapy. The use of postoperative chemotherapy was a significant factor negatively affecting the recurrence.

Background: To report the results of patients undergoing combined excision, cryotherapy, and intraoperative mitomycin-C (EXCRIM) for primary ocular surface squamous neoplasia (OSSN) METHODS: A retrospective review of a non-comparative interventional case series. Histopathologically confirmed primary localized (less than four clock hours) OSSN treated with EXCRIM using adjuvant 0.02 % mitomycin-C (MMC) were included in the study. The main outcome measures were recurrence and complications related to MMC., Results: The study enrolled 28 eyes of 28 patients with OSSN with a median age of 64.5 (range 43 to 84) years. The mean tumor size was 6.9 × 4.35 mm. There was corneal involvement in 23 of 28 (82 %). Seven patients (21 %) had delayed epithelial healing. Two of eight patients (25 %) with squamous cell carcinoma (SCC) had positive lateral margins. There were no recurrences over a mean follow-up of 49 months (range 24 to 96)., Conclusion: The excision of OSSN combined with cryotherapy and intraoperative MMC is effective with a low recurrence rate. Long-term follow-up yielded favorable results.

Ocular melanoma is the second most common type of melanoma after cutaneous and the most common primary intraocular malignant tumor in adults. Large majority of ocular melanomas originate from uvea, while conjunctival melanomas are far less frequent. Incidence of uveal melanoma has remained stable over last three decades. Diagnosis is in most cases established by clinical examination with great accuracy. Local treatment of uveal melanoma has improved, with increased use of conservative methods and preservation of the eye, but survival rates have remained unchanged. Recent advances in cytogenetics and genetics enhanced prognostication and enabled to determine tumors with high metastatic potential. However, due to lack of effective systemic therapy, prognosis of patients with metastasis remains poor and metastatic disease remains the leading cause of death among patients with uveal melanoma. Conjunctival melanoma is rare, but its incidence is increasing. It mostly occurs among white adults. In majority of cases it originates from preceding primary acquired melanosis. Current standard treatment for conjunctival melanoma is wide local excision with adjuvant therapy, including brachytherapy, cryotherapy and topical application of chemotherapeutic agent. Rarity of this tumor limits conduction of controlled trials to define the best treatment modality. As well as for uveal melanoma, prognosis of patients with metastasis is poor because there is no effective systemic therapy. Better understanding of underlying genetic and molecular abnormalities implicated in development and progression of ocular melanomas provides a great opportunity for development of targeted therapy, which will hopefully improve prognosis of patients with metastatic disease.

Purpose: To evaluate the prognostic utility of the American Joint Committee on Cancer (AJCC) staging system for ocular adnexal lymphoma (OAL).
, Methods: A multicenter, consecutive case series of patients with biopsy-proven conjunctival, orbit, eyelid, or lacrimal gland/sac lymphoma was performed. The electronic pathology and clinical records were reviewed for new or recurrent cases of ocular adnexal lymphoma. The main outcome measures included pathology and clinical staging (AJCC and Ann Arbor systems), treatment, and recurrence (local and systemic). Statistical analysis included demographic evaluations and the Kaplan-Meier survival probability method.
, Results: Extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue were the most common (n=60/83, 72%). The most common Ann Arbor clinical stages were IE (76%) followed by IIE (17%) and IIIE (7%). Pathology identified 13 cases (15%) that were upstaged to group IV (p=0.017). Similarly, AJCC clinical stages were cT1NOMO (21.7%), cT2NOMO (44.6%), cT3N0M0 (5%), and cT4NOMO (2.4%). Local control was achieved in 75% of treated patients. There were 19 local recurrences from which 14 (74%) belonged to the non-radiation treatment groups. Lower-risk groups (T1 and T2 without lymph node involvement or metastatic disease of AJCC and IE of Ann Arbor) had longer disease-free survival than the higher-risk groups (AJCC T1, T2 with nodal involvement or metastatic disease, T3, and T4 as well as Ann Arbor II, III, and IV). The overall mean follow-up was 43.3 months (range 6-274).
, Conclusions: Regardless of stage, recurrence and disease-free survival were more closely related to treatment and histopathology rather than tumor size or site-specific location.

Importance: Conjunctival papilloma is a benign epithelial tumor occurring in both children and adults with varying clinical features and outcomes. In this article, we describe our experience regarding the difference in the clinical features and outcomes of conjunctival papilloma based on age at initial examination., Objective: To evaluate the clinical features, treatment, and outcomes in patients with conjunctival papilloma based on age at initial examination., Design: Retrospective study., Setting: Ocular Oncology Service, Wills Eye Hospital, Philadelphia, Pennsylvania., Participants: Ten children and adolescents (aged ≤20 years) and 63 adults (aged >20 years) with conjunctival papilloma., Interventions: Excisional biopsy, cryotherapy, oral cimetidine, topical or injection interferon alfa-2b, and photodynamic therapy., Main Outcome Measure: Tumor response., Results: A comparison of conjunctival papillomas between age groups revealed significant differences in the mean number of tumors per eye (children and adolescents vs adults, 2 vs 1; P = .05), tumor basal dimension (8 vs 6 mm; P = .05), and associated feeder vessels (20% vs 47%; P = .05). Primary treatment included sole treatment with oral cimetidine (15% vs 5%), topical interferon alfa-2b (0% vs 1%), cryotherapy (0% vs 3%), photodynamic therapy (0% vs 1%), excisional biopsy and cryotherapy (38% vs 65%), excisional biopsy and cryotherapy with adjuvant oral cimetidine (8% vs 9%), and excisional biopsy and cryotherapy with adjuvant topical or injection interferon alfa-2b (38% vs 15%). Significant differences in age groups in treatment outcome during the follow-up period (mean, 24 vs 38 months) included complete regression with single treatment (38% vs 95%; P < .01) and tumor recurrence (15% vs 1%; P = .05)., Conclusions and Relevance: Conjunctival papillomas are larger and more likely to be multiple in children and adolescents than in adults. Excisional biopsy and cryotherapy with or without adjuvant oral cimetidine and/or topical interferon alfa-2b provide satisfactory tumor control. Papilloma recurrence is more common in children and adolescents than in adults.

Purpose: To assess the efficacy of interferon alpha-2b (IFNα2b) in the management of ocular surface squamous neoplasia (OSSN)., Methods: This is a retrospective, nonrandomized interventional case series study of 80 patients with 81 tumors treated with IFNα2b eye drops and/or injection combined with surgical excision when necessary. The main outcome measure was complete response or partial response based on the American Joint Committee on Cancer classification., Results: The OSSN was classified as Tis (n = 10, 12%), T1 (n = 13, 16%), T2 (n = 6, 7%), T3 (n = 51, 63%), and T4 (n = 1, 1%). IFNα2b was used as immunotherapy alone (n = 22, 27%) or combined with surgery (n = 59, 73%). Overall (n = 81), complete response was achieved in 90% Tis, in 100% T1, in 100% T2, in 94% T3, and in 100% T4. Specifically for immunotherapy (n = 22), IFNα2b alone achieved complete response in 75% (3/4) Tis, in 100% (8/8) T1, and in 70% (7/10) T3. Planned IFNα2b plus surgery (n = 59) achieved control in 100% (6/6) Tis, in 100% (5/5) T1, in 100% (6/6) T2, in 100% (41/41) T3, and in 100% (1/1) T4. Tumor recurrence was noted in 5% (4/81) of cases over a median follow-up of 1 year. Ocular side effects included conjunctival hyperemia (n = 4, 5%), ocular irritation (n = 3, 4%), superficial punctate keratitis (n = 3, 4%), and conjunctival follicles (n = 1, 1%). Systemic side effects included postinjection flu-like syndrome for 1 day (n = 7, 9%)., Conclusions: IFNα2b, when appropriately combined with surgical excision for OSSN, provides complete control in 95% of cases overall, specifically in 90% Tis, in 100% T1, in 100% T2, in 94% T3, and in 100% T4.

The case of a 57-year-old woman with a rapidly growing unpigmented conjunctival tumor is presented. Despite the clinical appearance the histology surprisingly confirmed a malignant melanoma of the conjunctiva. Various phenotypes and the rareness of this disease complicate a clinical diagnosis. Therapeutically, a complete excision of the tumor including a safety margin, adjuvant topical chemotherapy and closely monitored follow-up are crucial.

Purpose: To characterize the clinicopathological features of diffuse large B-cell lymphoma (DLBCL) of the ocular adnexal region., Methods: The present series of orbital and adnexal DLBCLs were found by searching the Danish Registry of Pathology between 1980 and 2009. Histological specimens were re-evaluated using a panel of monoclonal antibodies. Clinical files from all patients with confirmed DLBCL were collected., Results: A total of 34 patients with DLBCL of the ocular adnexal region were identified. Eighteen of the patients were men. The patients had a median age of 78 years (range 35-97 years). Ninety-seven per cent of the patients had unilateral ocular adnexal region involvement, and the orbit (76%) was the most frequently affected site. Nineteen patients (56%) presented with Stage I lymphoma. Of these, 18 were diagnosed with primary lymphoma. Four patients (12%) had Stage II, one patient (3%) had Stage III and ten patients (29%) presented with Stage IV lymphoma. The 5-year overall survival (OS) rate for the whole study group was 20%. The patients with Stage I lymphoma had a significantly better 5-year OS rate (28%) than patients in Stage II-IV (5-year OS rate, 9%). In Cox regression analysis, concordant bone marrow involvement and the International Prognostic Index (IPI) score were prognostic factors for OS., Conclusions: Diffuse large B-cell lymphoma of the ocular adnexal region is mainly prevalent in elderly patients. Most patients had unilateral orbital involvement. The overall prognosis is poor. Concordant bone marrow involvement and the IPI score were independent prognostic factors for mortality., (© 2012 The Authors. Acta Ophthalmologica © 2012 Acta Ophthalmologica Scandinavica Foundation.)

Background: Squamous cell carcinoma of the conjunctiva is described in the ophthalmic literature as a rare, slow-growing tumour of the eye, normally affecting elderly men around 70 years of age. In Africa, however, the disease is different. The incidence is rising rapidly, affecting young persons (around 35 years of age), and usually affecting women. It is more aggressive, with a mean history of three months at presentation. This pattern is related to the co-existence of the HIV/AIDS pandemic, high HPV exposure, and solar radiation in the region. Various interventions exist, but despite therapy, there is a high recurrence rate (up to 43%) and poor cosmetic results in late disease. This review was conducted to evaluate the interventions for treatment of conjunctival squamous cell carcinoma in HIV-infected individuals., Objectives: To evaluate the effect of interventions for treating squamous cell carcinoma of the conjunctiva in HIV-infected individuals on local control, recurrence, death, time to recurrence, and adverse events., Search Methods: Using a sensitive search strategy, we attempted to identify all relevant trials, regardless of language or publication status, from the following electronic databases; PubMedPubMed, EMBASE and The Cochrane Library. We also searched clinical trial registries; WHO International Clinical Trials Registry Platform (ICTRP) and the US National Institutes of Health Clinicaltrials.gov. We searched the international conference proceedings of HIV/AIDS and AIDS-related cancers from the AIDS Education Global Education System (AEGIS). Searches were conducted between January and February 2012., Selection Criteria: Randomised controlled trials (RCTs) involving HIV-infected individuals with ocular surface squamous neoplasia., Data Collection and Analysis: We independently screened the results of the search to select potentially relevant studies and to retrieve the full articles. We independently applied the inclusion criteria to the potentially relevant studies. No studies were identified that fulfilled the selection criteria., Main Results: No RCTs of interventions currently used against conjunctival squamous cell carcinoma in HIV-infected individuals were identified.There is one ongoing RCT in Kenya that was registered in July 2012., Implications for Practice: Current clinical practice in treatment of squamous cell carcinoma of the conjunctiva rests on a weak evidence base of case series and case reports., Implications for Research: Randomised controlled trials for treatment of this disease are needed in settings where it occurs most frequently. Preventive interventions also need to be identified. HIV/AIDS research has not focused on treatment of this tumour.

The rarity of conjunctival melanoma has impeded progress in the management of patients with this cancer; however, much progress has occurred in recent years. Primary acquired melanosis is now differentiated histologically into hypermelanosis and conjunctival melanocytic intra-epithelial neoplasia, for which an objective reproducible scoring system has been developed. Mapping and clinical staging of conjunctival disease has improved. Adjunctive radiotherapy and topical chemotherapy have made tumour control more successful, with reduced morbidity. Genetic analyses have identified BRAF and other mutations, which may predict responsiveness to new chemotherapeutic agents, for example Vemurafenib, should metastatic disease develop. Multicentre studies are under way to enhance survival prediction by integrating clinical stage of disease with histological grade of malignancy and genetic abnormalities. Such improved prognostication would not only be more relevant to individual patients, but would also provide greater opportunities for basic science research.

Purpose of Review: Ocular surface malignancy is a serious complication in HIV infection, but can often result in successful treatment if diagnosed appropriately. In the literature, most reviews focus on information for the ophthalmic community. Here, we provide a review of the literature with the pertinent information for the nonophthalmologist, as they are the first point of contact for most HIV patients., Recent Findings: Ocular surface squamous neoplasia (OSSN) is the most common nonpigmented ocular surface malignancy. It can be treated well with surgery or topical chemotherapy, the newest method of treatment. When presenting in young patients, a high percentage have been found to be HIV positive. Kaposi's sarcoma is an AIDS-defining malignancy and critical to diagnose. It cannot be cured, but treatment is effective for keeping it controlled. Conjunctival lymphoma can be recognized with the salmon patch appearance. External beam radiation, systemic chemotherapy, and intralesional injections are the mainstays of treatment., Summary: Ocular surface malignancy manifests significantly in the HIV population. OSSN, Kaposi's sarcoma and conjuctival lymphoma all have different clinical presentations. The capacity of the managing physician to recognize these tumours and refer to an ophthalmologist is essential for appropriate treatment.

Background: Clinical experience of our single center in dealing with pigmented epibulbar lesions - melanocytic conjunctival tumors is presented. Since 2008 we use the topical treatment with mitomycin C (MMC) as an alternative or adjunctive method for excision in the treatment of melanocytic neoplasia of the conjunctiva., Methods: The retrospective case series of 85 patients with pigmented lesions of the conjunctiva - melanocytic conjunctival tumors, histopathologically examined in the period 2001-2010 is presented. Since 2008 we started to apply MMC in the treatment of primary acquired melanosis (PAM) and dysplastic nevi. We apply MMC topically directly after an excision as 2-times five minutes application., Results: In 85 patients with pigmented lesions of the conjunctiva histopathological findings after excision of the lesion showed in 68 (80 %) cases melanocytic nevocelullar nevus, out of which 55 cases were combined and 13 cases were junctional nevi. In 60 (80 %) cases of melanocytic nevi atypia was found in 25 patients (42 %), nevus without atypia was present in 35 cases (58 %). PAM with atypia was found in 16 patients (classified since 2000). During the period of application of MMC we diagnosed only one patient with primary conjunctival melanoma. There was no presence of relapse of the pigmented lesion either after primary excision or after excision with MMC., Conclusion: Resection of more than one quadrant of bulbar conjunctiva in patients with pigmented lesions of the conjunctiva in cases of conjunctival nevus with atypia and PAM with atypia combined with topical MMC chemotherapy is an alternative therapy for residual pigmented lesions. There was no presence of relapse of pigmentation in area of excision with or without using MMC during the surgery in patients with PAM. The number of our patients is not sufficient yet to draw a conclusion (Fig. 6, Ref. 21).

Pigmented conjunctival lesions primarily include melanoma, nevus, primary acquired melanosis (PAM), and complexion-associated melanosis. The incidence of conjunctival malignant melanoma (CMM) has significantly increased in the USA and Europe over the years, and it carries a very serious risk for metastasis and melanoma-related death. Conjunctival melanoma can arise de novo or from precursor lesions, nevus, and PAM. PAM is histologically separated into two distinct forms: PAM with atypia and without atypia. PAM with severe atypia progresses to malignant melanoma in up to 50% of cases and PAM without atypia virtually never progresses. Nevus is a benign melanocytic tumor that only rarely undergoes malignant transformation. Complexion-associated melanosis is frequently seen in more darkly pigmented individuals and does not progress to melanoma, although it can become very large. A detailed literature review on the various pigmented conjunctival lesions together with a schematic approach to diagnosis and management is presented., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Ocular surface squamous neoplasia is a rare complication after a kidney transplant, related with increased risk and poor prognosis. Generally, ocular surface squamous neoplasia in kidney transplant patients is associated with skin lesions. We report a case of ocular surface squamous neoplasia without skin lesions in a kidney transplant recipient. Systematic periodic ophthalmic evaluation of recipients may help ensure the early diagnosis of subtle ocular surface squamous neoplasia.

Introduction: Melanocytic tumors comprise a wide range of lesions. These lesions can be nevi, primary acquired melanosis with or without atypia or malignant as conjunctival melanoma. Early recognition of the clinical features allows precise diagnosis and adequate intervention, reducing the rates of metastatic disease and mortality associated with conjunctival melanoma., Purpose: To review clinical features, diagnosis, clinical and surgical treatment modalities of the different precursor pigmented lesions (Nevi and primary acquired melanosis) and malignant melanoma., Methods: Literature review was performed accessing the database of MEDLINE, PUBMED, LILACS e SciELO between 1980 and 2011. The keywords used, individually or combined, were: conjunctival melanoma, primary acquired melanosis, nevi, treatment, chemotherapy, recurrence, metastasis e mortality., Results: Relevant clinical information for the diagnosis of malignant melanoma and differentiation from other pigmented conjunctival lesions were accessed. Clinical and pathological features and different modalities of treatment for malignant melanoma have been reviewed., Conclusions: Patients presenting with a pigmented conjunctival lesion should be evaluated by an experienced ophthalmologist. Ocular history, family history of melanoma, and clinical features of the lesion need careful assessment and malignancy risk evaluation. Digital photo documentation should be performed. Clinical or surgical planning should be done based on the clinical suspicion. Careful histopathology analysis is required for treatment orientation and prognostic factors identification, mainly in cases of malignancy.

Purpose: To describe the clinical findings and prognosis of conjunctival malignant melanomas., Subjects: We investigated the clinical findings and prognosis of 11 cases of conjunctival malignant melanomas diagnosed and treated at Tokyo Medical University Hospital., Results: The mean age at presentation was 62.3 years, male/female ratio was 6/5. Cases arose from primary acquired melanosis (PAM) in 45% of the patients, from conjunctival nevus in 9%, from both conjunctival nevus and PAM in 27% and de novo in 18%. In all cases local resection was performed with either adjuvant cryotherapy, or similar methods for diagnosis and treatment. Postoperative local recurrence was detected in 45% of the patients. According to the Kaplan-Meier survival curve, local recurrence at 2 years is 38%. Total surgical excision of the tumor was performed for recurrent lesions. Three cases were treated with systemic chemotherapy following surgical excision for metastasis., Conclusion: A combination of surgical excision with adjuvant therapy has been used for the treatment of conjunctival melanoma. A more effective therapeutic strategy is required because of the high rate of recurrence and metastasis seen in this study.

Objective: To evaluate the efficacy of interferon alpha-2b (IFNα2b) for extensive ocular surface squamous neoplasia (OSSN)., Design: Retrospective, interventional case series., Participants: Eighteen eyes in 18 patients., Methods: Each patient with giant OSSN (a single tumor ≥15 mm basal diameter or ≥6 limbal clock-hours) was managed with topical IFNα2b (1 million IU/ml) 4 times daily or with injection IFNα2b (a portion of 10 million IU/ml vial) with follow-up every 1 to 3 months., Main Outcome Measures: Tumor response, recurrence, and treatment complications were evaluated., Results: Eighteen patients with giant OSSN (median diameter [MD], 20 mm; median clock-hours [MCH], 6) were treated with topical IFNα2b (n = 12), injection IFNα2b (n = 3), or both (n = 3). The IFNα2b achieved complete tumor control (immunotherapy) in 13 eyes and partial tumor control with reduction in size (immunoreduction) in 5 eyes. Topical IFNα2b alone (n = 12) provided complete immunotherapy in 7 eyes (MD, 12 mm; MCH, 9) over a median period of 5 months and immunoreduction by 74% in 5 eyes (MD, 20 mm; MCH, 3), allowing for subsequent surgical excision (n = 3), photodynamic therapy (n = 1), or cryotherapy (n = 1) for tumor control. Injection IFNα2b alone (n = 3; median, 1 injection) provided complete control of giant tarsal conjunctival OSSN (MD, 20 mm) over a 1-month period. A combination of topical and injection IFNα2b (n = 3; median, 3 injections) completely resolved larger tumors (MD, 30 mm; MCH, 6) over a 6-month period. Complications of IFNα2b included transient flu-like symptoms (n = 3), corneal epithelial defect (n = 2), and conjunctival hyperemia (n = 1). During a median follow-up of 11 months, there were no tumor recurrences, but 2 new tumors appeared at a remote site from the original tumor, requiring operative intervention., Conclusions: In 72% of giant OSSNs IFNα2b achieved complete control (immunotherapy); there was a reduction in size (immunoreduction) in 28% of giant OSSNs., (Copyright © 2012 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

Objective: To evaluate clinical and histopathologic factors of squamous conjunctival neoplasia associated with recurrence., Design: Retrospective, clinical case series., Participants: One hundred one eyes of 99 patients with squamous conjunctival dysplasia, carcinoma in situ, or squamous cell carcinoma., Methods: Review of the medical records, pathology reports, and color photographs., Main Outcome Measures: Demographic information, laterality, tumor size, extension, pathologic diagnosis, seventh edition of the American Joint Committee on Cancer Classification (AJCC) staging system stage, treatment methods, recurrence, and duration of follow-up., Results: Malignant squamous conjunctival neoplasia was seen most commonly in males at a median age of 71 years. Recurrences were seen in 12.9% (n = 13/101), with 92.3% occurring 6 to 12 months after primary treatment. Recurrence was not correlated significantly to age, gender, laterality, clinical appearance or focality of the tumor at presentation. However, tumors larger than 5 mm in diameter, tumors extending more than 2 mm onto the cornea, and tumors with local invasion (corneal, scleral, intraocular or orbital invasion) were associated with a higher risk of recurrence. Increasing AJCC T-stage was correlated strongly to the incidence of recurrence (P = 0.0006). Rates were 1.7% for Tis-staged tumors, 0% for T1- and T2-staged tumors, 34.3% for T3-staged tumors, and 50% for T4-staged tumors. Histopathologic diagnosis was correlated to recurrence (P = 0.037). None of the tumors defined histologically as dysplasia showed recurrence, whereas 12.8% of carcinoma in situ tumors and 22.2% of squamous cell carcinoma tumors recurred. Although the overall recurrence rate was 12.9%, the rate for tumors treated primarily at the authors' center was 4%, significantly less than the recurrence rate in previously operated tumors (P = 0.0003). Lymph nodes demonstrated positive results in 1%, and in no patient did distant metastasis develop., Conclusions: Advanced AJCC T-stage, locally invasive tumors, and more pathologically aggressive tumors were at higher risk for recurrence. Inadequate initial therapy also was an important risk factor for recurrence. Treatment strategies should be affected by tumor staging at presentation., (Copyright © 2012 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

A case of a squamous cell carcinoma of the conjunctiva is presented. The lesion was removed with recurrence in another conjunctival site. The patient received cycles of 5-Fluorouracil as adjuvant treatment to the surgical removal and presented unfavorable evolution, requiring orbital exenteration. Comments are made on the use of antimitotic drugs in the management of these lesions.

Ocular surface squamous cell neoplasia in an anophthalmic socket is an exceptionally rare occurrence. We report a 62-year-old white man who had his left eye enucleated at age 2 and developed an invasive squamous cell carcinoma 60 years later. He received multiple treatments, including excisional biopsy, topical mitomycin C chemotherapy, cryotherapy, and finally exenteration. We review the literature to evaluate the clinical characteristics, time of onset following enucleation, treatment, and outcome of previously reported cases. Our case emphasizes the importance of a thorough examination of the anophthalmic socket, including upper and lower lid eversion., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Objective: To report clinical and histopathologic features of vascular tumors of the conjunctiva., Design: Retrospective, noninterventional case series., Participants: A total of 140 patients., Intervention: None., Main Outcome Measures: Tumor diagnosis, anatomic location, clinical features, management, and histopathology., Results: There were 140 vascular tumors of the conjunctiva with 93% benign and 7% malignant. The specific diagnoses included lymphangioma/lymphangiectasis (n = 54, 36%), pyogenic granuloma (n = 31, 22%), capillary hemangioma (n = 20, 14%), Kaposi's sarcoma (n = 10, 7%), acquired sessile hemangioma (n = 10, 7%), racemose hemangioma (n = 7, 5%), varix (n = 4, 3%), cavernous hemangioma (n = 3, 2%), and glomangioma (n = 1, <1%). The lesions were unilateral in 89% and discovered at median age of 41 years. Bilateral lesions included lymphangiectasia, Kaposi's sarcoma, and racemose hemangioma. All tumors were typically found in adults with the exception of capillary hemangioma (<1 year) and glomangioma (16 years). The median tumor diameter was 6 mm, with the largest median diameter (15 mm) with Kaposi's sarcoma and (12 mm) glomangioma and capillary hemangioma and the smallest median diameter (3 mm) with cavernous hemangioma. The most common anatomic sites included bulbar conjunctiva, which was extralimbal in 80 patients (57%), limbal in 19 patients (14%), tarsal conjunctiva in 27 patients (19%), and forniceal conjunctival in 24 patients (17%). Clinical features included cystic component in 24% of patients (seen with lymphangioma, glomangioma, and varix), well-defined margins in 64% of patients, and feeder vessels in 39% of patients., Conclusions: A variety of vascular tumors can occur in both children and adults at various anatomic locations in the conjunctiva. They tend to occur unilaterally on the bulbar conjunctiva, and the majority of tumors are benign., (Copyright © 2011 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

Purpose: To describe a rare case of conjunctival leiomyosarcoma initially diagnosed as a poorly differentiated squamous cell carcinoma., Methods: Clinical, light microscopic, immunohistochemical, and ultrastructural findings are reported., Results: A 56-year-old Caucasian woman was referred with a history of a progressive, rapidly growing mass in her left eye. Biopsy of the mass and histology yielded a first diagnosis of a poorly differentiated conjunctival squamous cell carcinoma. Orbital exenteration was performed 2 weeks later. Macroscopically, the exenteration specimen showed a soft mass completely involving the conjunctiva and extending to the eyelids and orbital structures. Histological examination revealed a malignant tumour composed of atypical, predominantly epithelioid large cells. Immunohistochemical and ultrastructural studies combined with the light microscopic findings contributed to clarify the diagnosis of epithelioid leiomyosarcoma. The patient was started on chemotherapy and radiotherapy, but died a few months later from widespread metastases., Conclusions: primary involvement of the orbit by a leiomyosarcoma is rare, but this eventuality should be considered in the differential diagnosis of rapidly growing orbital and conjunctival masses.

Background: To describe the outcome of patients treated by conservative surgical excision followed by adjuvant plaque brachytherapy for early-stage primary or recurrent conjunctival melanoma., Design: Retrospective, non-comparative, interventional case series., Participants: We reviewed 19 eyes in 19 consecutive patients presenting with biopsy proven conjunctival melanoma with pathologic stage pT1c or less., Methods: Patients with primary or recurrent early-stage conjunctival melanoma were identified and treated using a conservative resection technique (tractional micro-dissection) involving avoidance of deep dissection and modest lateral clearance followed by adjuvant plaque brachytherapy., Main Outcome Measure: Local recurrence and ocular complications., Results: Mean age was 55.2 years and male : female (8:11). Mean follow up was 43.1 months (range 30.1-54.3 months). All patients were treated by conservative resection followed by adjuvant Iodine-125 plaque brachytherapy. The treatment dose was 100 Gy to a depth of 1.5-3.0 mm., Outcomes: median visual acuity and intraocular pressure were unchanged after surgery. Six patients experienced corneal ulceration in the immediate postoperative period. No patients experienced recurrence at the treatment site or metastases. Three patients experienced new lesions distant from the treatment site., Conclusions: Conservative resection and adjuvant plaque brachytherapy is an effective and well-tolerated modality for the management of patients with early-stage conjunctival melanoma., (© 2011 The Authors. Clinical and Experimental Ophthalmology © 2011 Royal Australian and New Zealand College of Ophthalmologists.)

Conjunctival melanocytic tumors represent a spectrum of pigmented tumors that include benign, premalignant and malignant tumors. Conjunctival nevi are the most common pigmented tumors and are typically found in the interpalpebral bulbar conjunctiva. These lesions usually contain fine clear cysts on slit lamp biomicroscopy. Primary acquired melanosis includes lesions from increased melanin pigmentation without proliferation of melanocytes to melanoma in situ. In the new classification system, the idea is to use the term 'primary acquired melanosis' only as a clinical description, highlighting the fact that the biologic behavior of acquired melanotic lesions cannot be predicted solely based upon clinical grounds without histopathologic examination. Conjunctival melanoma represents only 5% of all melanomas arising in the ocular region and is associated with a high mortality rate. The management of primary acquired melanosis, nevi and conjunctival melanomas involves various modalities used either alone or concomitantly depending on the size and extent of the lesion.

Purpose: To analyze the outcome of excisional biopsy of ocular surface (conjunctival and corneal) squamous cell intraepithelial (in situ) neoplasia with and without the adjunctive use of intraoperative and postoperative mitomycin C., Methods: A retrospective case review was conducted on 34 consecutive patients with histopathologically proven ocular surface squamous cell intraepithelial neoplasia who were treated from January 1, 1980, to December 31, 2008, at University of Iowa Hospitals and Clinics. Eyes in which a minimum follow-up period of 3 months was available were included in the statistical analysis., Results: Of 34 eyes, 32 met the inclusion criteria. Adjunctive therapy was provided with mitomycin C in 17 eyes, including 13 that had only postoperative treatment and 4 that had only intraoperative treatment. The adjunctive use of mitomycin C was associated with a significantly reduced prevalence of recurrence (5.9% vs. 66.7%; P = 0.0005). When the surgical margins were positive, the use of adjunctive mitomycin C was associated with a reduced prevalence of tumor recurrence (12.5% vs. 55.6%), but this difference was not statistically significant (P = 0.13). When surgical margins were negative, the adjunctive use of mitomycin C was significantly associated with a reduced prevalence of recurrence (0% vs. 83.3%; P = 0.002)., Conclusions: The adjunctive use of mitomycin C is significantly associated with a reduction in the prevalence of postoperative recurrences of ocular surface squamous cell intraepithelial neoplasia and should be considered as an adjunctive therapy even when surgical margins are negative.

Background: Conjunctival intraepithelial neoplasia (CIN) is the most common tumor of the ocular surface and is a precursor to invasive squamous cell carcinoma. CIN comprises a spectrum of premalignant epithelial neoplasia that usually begins near the limbus and spreads to the cornea., Methods: This case report reviews the clinical findings and management of CIN, with a discussion of the histopathology and etiology of this tumor., Conclusion: CIN has a variety of clinical presentations, including gelatinous, leukoplakic, or papilliform. Because of their malignant potential, these lesions must be differentiated carefully and treated promptly. Traditional management of CIN is surgical excision with cryotherapy, but other topical treatments are being explored with promising results., (Copyright © 2010 American Optometric Association. Published by Elsevier Inc. All rights reserved.)

Background: Conjunctival melanoma is an uncommon malignancy with the potential for significant morbidity. Ulceration is known to be an indicator of more aggressive cutaneous melanoma. This factor has not been previously evaluated in conjunctival melanoma., Methods: Twenty-six patients with conjunctival melanoma who underwent excision by a single surgeon at a cancer referral center treated between June 2003 and July 2008 were included. The medical records were reviewed for clinical and pathological data. Outcomes assessed included local recurrence, regional lymph node metastasis, distant metastasis, use of adjuvant topical chemotherapy or adjuvant radiation therapy, final visual acuity and death., Results: Local recurrence was seen in three patients (12%). Regional lymph node metastasis and distant metastasis were each seen in four patients (15%). Tumors >2.0 mm thick were more likely to develop distant metastasis than those with a thickness of <2.0 mm (p = 0.0331). Ulceration was noted in 10 of 26 patients. Regional lymph node metastasis and distant metastasis were each seen in 4 of 10 patients with ulceration, and none of the patients without ulceration., Conclusions: Ulceration in conjunctival melanoma may be an important histopathologic predictor of a more aggressive tumor. Globe-sparing multi-modality management of conjunctival melanoma can yield acceptable rates of local-regional recurrence., (Copyright © 2010 John Wiley & Sons A/S.)

Objective: To assess the influence of hepatitis C virus (HCV) infection on disease appearance and outcome of ocular adnexal non-Hodgkin lymphoma (ONHL)., Design: Retrospective comparative study (from January 1, 1992, through December 31, 2006)., Methods: The medical records of 129 patients with ONHL were retrospectively reviewed. All the patients were tested serologically for the presence of HCV infection. Patients were divided into 2 groups according to the presence or absence of HCV infection., Main Outcome Measures: Prevalence of HCV infection, staging to evaluate the extent of disease at the onset, and clinical outcome data on overall and disease-free survival., Results: The prevalence of HCV infection among the patients with ONHL was 17.8%. Seropositivity for HCV infection was significantly associated with extraorbital lymphoma at the onset (P = .006). High prevalence of mucosa-associated lymphoid tissue disease (79.8%) was registered. Protocol therapy included radiotherapy and chemotherapy, depending on the stage of the disease. Complete remission was achieved in 99 patients (76.7%). A total of 23.6% of patients with HCV-seronegative status and 21.7% of those with HCV-seropositive status experienced relapse of the lymphomatous disease. No significant differences in the 5-year overall survival and disease-free survival between the 2 groups were observed., Conclusions: Prevalence of HCV infection in patients with ONHL is a relevant issue, accounting for 17.8% of the examined patients. Infection with HCV may influence the initial appearance of ONHL because it is associated with more widespread disease at the onset. However, the overall and disease-free survival of the infected patients are not statistically different than that of patients who are not infected.

Purpose of Review: The current dilemmas in the diagnosis and management of conjunctival melanoma are reviewed in this paper. Available evidence for the role of sentinel lymph node biopsy as it applies to conjunctival melanoma is examined., Recent Findings: The incidence of conjunctival melanoma appears to be on the rise. Analyses of 770 patients with conjunctival melanoma reveals a nearly 50% rate of local recurrence, and up to a 38% 10-year mortality. Adverse outcomes can be minimized by adjuvant therapies and 'no touch' techniques at the time of initial surgery. The metastatic spread occurs via both lymphatic channels and hematogenously. Sentinel lymph node biopsy does not appear to have a significant impact on survival or subsequent treatment., Summary: The management of conjunctival surface melanomas is complicated by local recurrence and metastatic disease, particularly for lesions greater than 2 mm in thickness and arising from nonbulbar conjunctiva. Early complete excision with appropriate adjuvant cryotherapy and topical chemotherapy may be curative in some patients. Conjunctival anatomy and high recurrence rate with resultant metastatic spread undermines the utility of sentinel lymph node biopsy in this condition. Further research and innovation for detection of systemic micrometastases and treatment of metastatic disease are needed.

The main conjunctival tumors are represented by (i) conjunctival melanoma, ocular surface squamous neoplasia, and conjunctival lymphoma, which are malignant tumors, and (ii) conjunctival nevus, papilloma, and choristoma, which are benign. In usual practice, ophthalmologists need to know how to differentiate conjunctival melanomas and carcinomas from the other conjunctival tumors by clinical examination. Indeed, the therapeutic approach to these two malignant diseases presents a number of particularities such as the need for surgery under general anesthesia, if possible, to minimize the risk of local dissemination and further local recurrence, and detailed information on the tumor location and measurements before surgery (Figures, diagram, sizes) to precisely define the irradiation field during complementary radiotherapy. The clinical signs and main therapeutic principles of conjunctival tumors are reviewed in this article., (Copyright (c) 2010 Elsevier Masson SAS. All rights reserved.)

Background: Because of high local recurrence rates after excision of conjunctival melanoma adjuvant local chemotherapy employing mitomycin C (MMC) or irradiation is recommended. Brachytherapy is possible with ruthenium-106-plaques ((106)Ru) or with the strontium-90-plaques ((90)Sr)., Patients and Methods: Fifty-six patients received an excision and adjuvant radiotherapy of conjunctival melanoma between 1992 and 2007. The mean follow-up was 42 months (12 - 151 months). Mean age was 62 (28- 86) years. As an adjuvant radiotherapy 15 patients received X-ray irradiation, 12 patients received (106)Ru-brachytherapy, 4 patients received proton beam therapy and 16 patients with conjunctival melanoma were treated with adjuvant strontium-90 brachytherapy after tumour excision. Four patients received proton beam irradiation and in 13 patients an exenteratio was performed., Results: Twelve patients (21 %) developed tumour recurrences in or adjacent to the irradiated area. Thirteen patients (22 %) showed a recurrence distant from the primary site. Ten patients (18 %) developed systemic metastasis during follow-up. Seven patients (46 %) had no recurrence during the follow-up. Three patients (20 %) had a recurrence in the treated or adjacent areas. Eight patients (53 %) developed new tumours in non-treated areas., Conclusions: Adjuvant radiotherapy allows an acceptable local tumour control rate after excision of conjunctival melanoma. No obvious differences regarding tumour control or systemic metastasis could be seen between the different modes of radiotherapy used., (Copyright Georg Thieme Verlag KG Stuttgart . New York.)

Background/aims: To retrospectively evaluate the clinicopathological features, treatment modalities and factors affecting prognosis in patients with both conjunctival intraepithelial and invasive squamous neoplasms., Method: Review and analysis of clinical and pathological records of all cases histologically diagnosed in the Department of Pathology, University College Hospital, Ibadan, Nigeria between January 1990 and December 2004., Results: There were a total of 46 cases in 45 patients (eight intraepithelial carcinomas, 37 invasive squamous cell carcinomas (SCC) and a single case of mucoepidermoid carcinoma in a 71-year-old man). The intraepithelial neoplasms occurred in patients aged between 20 and 90 years. Seven of these patients had actinic changes on histology. For invasive SCC, patients' ages ranged from 18 to 84 years with a mean age of 53 years. Male to female ratio was 1:1. The majority presented with an orbital mass and loss of vision. Twenty-two (60%) of the patients with invasive SCC had enucleation or exenteration as the primary modality of treatment with or without radiotherapy or chemotherapy. Altogether for intraepithelial and invasive squamous neoplasms, the duration of presenting complaints ranged from 1 month to 5 years with an average of 2 years. Human immunodeficiency virus (HIV) infection and smoking were not significantly associated with these cases but a significant number had outdoor occupation. Low socioeconomic status and inability to afford treatment was common among our patients., Conclusion: Patients with invasive SCC in Nigeria present late and have significant delay before having any form of treatment. This contributes to the poor outcome. Human papilloma virus (HPV) and actinic aetiology are possible strong causative agents in Nigerians.

Purpose: The aim of this work is to report the long-term results of using immunotherapy for the management of cornea and conjunctiva intraepithelial neoplasia and squamous cell carcinoma after surgical excision of the neoplasm., Methods: Ten eyes of 10 patients with cornea and conjunctiva intraepithelial neoplasia or squamous cell carcinoma had wide surgical excisions of the neoplasm after evaluation of the level of corneal involvement using ultrasound biomicroscopy. All eyes received topical cyclosporine A (0.05%) and topical mitomycin C (0.01%) 4 times daily for 12 weeks after surgery. All eyes were followed up every month for 6 months and then every 6 months for another 18 months., Results: All eyes showed total cure with no recurrence during the 2-year follow-up period. Epithelial toxicity (punctate keratopathy) occurred in 3 eyes, ocular irritation and mild conjunctival hyperemia in 5 eyes, and lid toxicity in 2 cases during the treatment with mitomycin C. There were no serious complications that necessitated stopping the treatment., Conclusion: During a 2-year follow-up period, the use of topical cyclosporine A (0.05%) combined with mitomycin C (0.01%) as an adjunctive treatment after surgical excision in cornea and conjunctiva intraepithelial neoplasia and squamous cell carcinoma was found to prevent tumor recurrence, especially in extensive lesions, when surgical excision cannot ensure a tumor-free margin.

A 44-year-old male patient first noticed a pain-free light red swelling of the right eye in 1983. Based on the results of the early indocyanine green angiography, MRI and sonography, a racemose hemangioma of the conjunctiva was diagnosed. Although a racemose hemangioma already exists at birth, it remains clinically silent for a long time and symptoms often first appear in early adulthood. In this patient external surgical excision was terminated due to substantial bleeding. A symptomatic treatment in the sense of surface care is considered to be the best option.