Histopathological spectrum of ocular surface squamous neoplasia: A retrospective study of 776 lesions.

BACKGROUND: Ocular surface squamous neoplasia (OSSN) comprises neoplasm arising from the ocular surface, which includes conjunctiva, cornea, and limbus and ranges from mild dysplasia to invasive squamous cell carcinoma. PURPOSE: The aim of this work was to study the spectrum of OSSN based on histopathological analysis. Materials and. METHODS: This was a retrospective cross-sectional study comprising 776 histopathologically diagnosed cases of OSSN from January 2004 to December 2014. RESULTS: The mean age of presentation of OSSN was 45 years (median, 45 years; 2 to 87 years) with male preponderance (74%). The most common age group of presentation was 41-60 years (n = 299; 39%). The most common type of OSSN was invasive squamous cell carcinoma seen in 50% (n = 383) eyes followed by severe dysplasia/carcinoma in situ in 31% (n = 250) eyes. Tumor infiltration at base was seen in 16% (n = 124), positive margins in 32% (n = 248), scleral infiltration in 14% (n = 109), intraocular extension in 3% (n = 23), and orbital extension in 4% (n = 26) eyes. OSSN was associated with actinic keratosis in 21% (n = 165) cases. CONCLUSION: Based on histopathology, invasive squamous cell carcinoma is the most common form of OSSN in the Asian Indian population.

Lack of tumour pigmentation in conjunctival melanoma is associated with light iris colour and worse prognosis.

AIM: To investigate whether differences in iris colour, skin colour and tumour pigmentation are related to clinical outcome in conjunctival melanoma. METHODS: Data of 70 patients with conjunctival melanoma from the Leiden University Medical Center (Leiden, The Netherlands) and 374 patients from the Wills Eye Hospital (Philadephia, USA) were reviewed. The relation between iris colour, skin colour and tumour pigmentation versus clinical parameters and outcome was investigated using univariate and multivariate regression analyses. RESULTS: A light iris colour (blue, grey, green) was present in 261 (59%) patients and a dark colour (hazel, brown) in 183 (41%). A low tumour pigmentation was detected in 130 (40%) and a high pigmentation in 197 (60%) patients. Low tumour pigmentation was associated with light iris colour (p=0.021) but not related to skin colour (p=0.92). In univariate analysis, neither iris nor skin colour was related to clinical outcome, while a low tumour pigmentation was related to metastasis formation (HR 2.37, p=0.004) and death (HR 2.42, p=0.020). In multivariate analysis, low tumour pigmentation was related to the development of recurrences (HR 1.63, p=0.043), metastasis formation (HR 2.48, p=0.004) and death (HR 2.60, p=0.014). CONCLUSION: Lightly pigmented tumours occurred especially in individuals with lightly coloured irises. While iris colour or skin colour was not significantly related to clinical outcome, a low tumour pigmentation was related to a worse outcome in patients with conjunctival melanoma. The amount and type of melanin in conjunctival melanocytes may be involved in the pathogenesis and behaviour of selected conjunctival melanoma.

Ocular Human Papillomavirus Infections.

CONTEXT: - Human papillomavirus (HPV) has a well-known role in the pathogenesis of squamous cell carcinoma and precursor lesions of the cervix, anogenital region, and head and neck, but its role in the development of squamous neoplasms of the eye, particularly the conjunctiva, remains unclear. OBJECTIVE: - To review recent evidence implicating HPV in the pathophysiology of ocular lesions. DATA SOURCES: - Published articles obtained from a PubMed search of the English literature were the primary sources for this review. CONCLUSIONS: - The low-risk HPV types 6 and 11 appear to play a role in the development of at least a subset of conjunctival squamous papillomas. The role of HPV in the pathogenesis of pterygium and ocular surface squamous neoplasia is less well defined. There is evidence to suggest that HPV may be a cofactor in the development of these lesions, acting in concert with ultraviolet radiation and/or human immunodeficiency virus infection in a subgroup of cases.

[Ocular surface squamous neoplasia]. / Szemfelszíni laphám-neoplasia.

To summarize actual knowledge on epidemiology, etiology, pathology, clinical apparence and treatment of ocular surface squamous neoplasias. We summarize up-to-date literature on conjunctival intraepithelial neoplasia and invasive squamous cell carcinoma and present some own cases. Ocular surface squamous neoplasia is the most common malignant ocular surface tumor and the third most common ocular malignancy following malignant melanoma and lymphoma. In spite of its low malignant potential, in advanced stages it may reduce visual acuity significantly or even the eye globe has to be removed. In case of metastasis it may also be life-threatening. As local recurrences of ocular surface squamous neoplasias may occur, knowledge of intra- and postoperative adjuvant treatment options are indispensable and regular control examinations are necessary. Identification and adequate treatment of ocular surface squamous neoplasias are necessary in order to avoid its progression and to prevent recurrences. Orv Hetil. 2017; 158(51): 2011-2022.

Meibomian Gland Dysfunction Associated With Periocular Radiotherapy.

PURPOSE: To investigate the influence of periocular radiotherapy on meibomian glands. METHODS: We evaluated 28 patients (40 eyes) who received radiotherapy (RT group) for conjunctival or orbital lymphoma and 30 age-matched control subjects (60 eyes). Subjects underwent slit-lamp examination of the eyelids, Schirmer test, meibography, and evaluation of tear film breakup time (TBUT), Ocular Surface Disease Index (OSDI) scores, meibomian glands evaluation (meiboscore, meibum expressibility, and lid margin abnormality scores), and tear film lipid layer thickness using an ocular surface interferometer. These parameters were compared between subjects in the RT and control groups. RESULTS: Meiboscores as well as meibum expressibility and OSDI scores in the RT group were significantly higher compared with those in the control group (1.6 ± 0.9 vs. 0.4 ± 0.6, 1.6 ± 1.0 vs. 0.2 ± 0.4, and 48.1 ± 21.4 vs. 6.2 ± 4.4, respectively, P < 0.001, all), whereas the Schirmer value (9.2 ± 5.1 vs. 12.3 ± 5.2, P = 0.004), TBUT (4.2 ± 2.5 vs. 6.4 ± 2.6, P = 0.001), and lipid layer thickness (61.0 ± 29.3 vs. 85.2 ± 20.0, P < 0.001) in the RT group were lower compared with those in the control group. The percentage of meibomian gland dropout was significantly correlated with age (P = 0.025) and total radiation dose (P = 0.012), regardless of the target location of irradiation. Even low-dose irradiated eyes (<30 Gy) exhibited significantly higher meiboscores (P < 0.001) and shorter TBUT (P = 0.005) compared with control eyes. CONCLUSIONS: Eyes that received periocular radiotherapy exhibited relatively high tear film instability induced by meibomian gland dysfunction, contributing to the high severity of dry eye symptoms.

Spontaneous regression of a conjunctival malignant melanoma.

INTRODUCTION: Conjunctival malignant melanoma (MM) is a rare malignancy and accounts for 2% of all ocular malignancies. We describe what we believe to be the first reported case of complete spontaneous regression of a conjunctival malignant melanoma. METHODS: A 75-year-old female presented with a large, pigmented lesion in her left lower fornix. Histopathology revealed a mixed cell type MM. The tumour regressed spontaneously. Now, 6 years after she first presented, she remains well and there is no sign of recurrence or metastasis. CONCLUSION: Spontaneous regression is not always synonymous with complete cure from the underlying disease but may represent a temporary regression due to host protective mechanisms. In our patient regression and cure were complete.

Quiste conjuntival epitelial gigante primario orbitario / Orbital giant conjunctival epithelial primary cyst

Caso clínico: Varón de 15 años de edad, sin antecedente traumático o quirúrgico, que presenta proptosis con desplazamiento inferior del globo ocular derecho de cuatro meses de evolución. La ecografia y la TAC muestran una lesión quística de 3,5 × 2cm. Se reseca mediante orbitotomía transconjuntival. La histopatología muestra un quiste con un epitelio no queratinizado sin células caliciformes. Después de 10 años de evolución no ha recidivado . Discusión: Los quistes epiteliales conjuntivales primarios son muy infrecuentes. Suelen ser de pequeño-moderado tamaño; los gigantes son excepcionales. El diagnóstico por imagen es fundamental para el diagnóstico diferencial y el tratamiento quirúrgico(AU)

Case report: A 15-year-old male, with no previous traumatic o surgical ocular injury, presented with a right eye proptosis and inferior displacement which began 4 months earlier. Ultrasound and CT showed a cystic lesion of 3.5 × 2cm in the superior orbit. Surgical resection was performed by transconjunctival orbitotomy. The histopathology examination showed a cyst with nonkeratinized epithelium, and without goblet cells. After 10 years of follow-up, there have been no new lesions. Discussion: Conjunctival primary orbital cysts are very uncommon. They are usually of small-moderate size; giant cysts are exceptional. Diagnosis by imaging is essential to establish the differential diagnosis and surgical treatment(AU)

Regression of conjunctival tumor during dietary treatment of celiac disease.

A 3-year-old girl presented with a hemorrhagic conjunctival lesion in the right eye. The medical history revealed premature cessation of breast feeding, intolerance to the ingestion of baby foods, anorexia, and abdominal distention. Prior to her referral, endoscopic small intestinal biopsy had been carried out under general anesthesia with a possible diagnosis of Celiac Disease (CD). Her parents did not want their child to undergo general anesthesia for the second time for the excisional biopsy. We decided to follow the patient until all systemic investigations were concluded. In evaluation, the case was diagnosed with CD and the conjunctival tumor showed complete regression during gluten-free dietary treatment. The clinical fleshy appearance of the lesion with spider-like vascular extensions and subconjunctival hemorrhagic spots, possible association with an acquired immune system dysfunction due to CD, and spontaneous regression by a gluten-free diet led us to make a presumed diagnosis of conjunctival Kaposi sarcoma.

Solid corneal dermoids and subconjunctival lipodermoids: impact of differentiated surgical therapy on the functional long-term outcome.

PURPOSE: The functional long-term outcome after differentiated surgical therapy of solid corneal dermoids and subconjunctival lipodermoids with special regard to determinants for amblyopia will be assessed. METHODS: Forty-six consecutive patients undergoing surgery for solid epibulbar dermoids, subconjunctival lipodermoids, or both were included. Visual acuity, refraction, keratometry, and degree of amblyopia were determined. Surgical therapy for corneal dermoids consisted of lamellar sclerokeratectomy, lamellar keratoplasty, corneoscleroplasty, and lamellar removal with autologous episcleral transplant. Surgical therapy for lipodermoids consisted of excision and reduction of the volume of the tumor. Follow-up examination was performed on average 4.5 years after surgical intervention. RESULTS: A significant correlation between tumor volume and preoperative visual acuity could be observed in patients with solid corneal dermoids not occluding the optical axis. Visual acuity improved significantly from 0.21 +/- 0.4 to 0.35 +/- 0.4 after surgery of corneal dermoids. Most eyes had concomitant hyperopia. Postoperative visual acuity correlated positively with preoperative visual acuity (P = 0.0001). After tumor excision, hyperopia and astigmatism were not reduced significantly on average. Nineteen of 47 patients suffered from amblyopia. Amblyopia was more often observed in patients with preoperative hyperopia > or =2 diopters and astigmatism >2 diopters. Visual acuity, refraction, and astigmatism were not changed significantly by surgery in patients with subconjunctival lipodermoids. CONCLUSIONS: Epibulbar dermoids require differentiated surgical therapy. Amblyopia is a major threat of solid corneal dermoids. The incidence of amblyopia seems to depend on preoperative occlusion of the optical axis and preoperative degree of hyperopia and astigmatism.

Intraepithelial and invasive squamous neoplasms of the conjunctiva in Ibadan, Nigeria: a clinicopathological study of 46 cases.

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.

Clinical follow-up of conjunctival malignant melanoma.

PURPOSE: To investigate the clinical features and course of conjunctival malignant melanoma in Korea. METHODS: The medical records of 15 patients, 5 males and 10 females, diagnosed with conjunctival malignant melanoma who had been treated at Severance Hospital from May 1991 to March 2004 were reviewed retrospectively. The clinical parameters of the patients, tumors, and treatment were analyzed for their relation to outcome measures. RESULTS: The mean age at the time of diagnosis was 53.4 years (+/- 11.7 years). In all cases, the disease was unilateral and all patients had experienced at least one recurrence. Local lymph node metastasis was found in 3 patients (20%) and the mean time to metastasis was 3.5 years. Systemic metastasis was found in 6 patients (40%) and the mean time to metastasis was 9.3 years. There were 5 cases of tumor-related death (33.3%), 4 of which were attributed to systemic metastasis. The Kaplan-Meier estimates of cumulative survival rate were 90% at 30 months and 56.6% at 70 months. CONCLUSIONS: Although conjunctival malignant melanoma is a rare disease, it is life-threatening and complete tumor excision at an early stage is mandatory, as is additional therapy to prevent local recurrence and systemic metastasis.

Low-grade, malt-type, primary B-cell lymphoma of the conjunctiva.

Although primary ocular lymphomas may be found in the conjunctiva, eye lids and lacrimal glands, the majority nevertheless occur in the orbit. Only a few cases of primary conjunctival lymphoma have been described in the literature. A 68-year-old man presented with a painless swelling of the epibulbar conjunctiva of the right eye. A diffuse lymphoid infiltrate consisting of small-sized lymphoid cells with the morphology and distribution characteristics of mucosa-associated lymphoid tissue was observed. Immunohistological study demonstrated the B lymphocyte lineage of tumor cells and Bcl-1 and bcl-2 rearrangements were negative. After clinical staging including thoracic, abdominal, brain and orbital CT scans, fiberoptic gastroscopy and bone marrow biopsy, no other foci of this lymphoma were found. Radiation therapy was given and the patient currently remains free of lymphoma 30 months after diagnosis.

Carcinoma epidermóide da conjuntiva: relato de um caso atípico / Squamous cell carcinoma of the conjunctiva: an atipic case report

É apresentado um caso de carcinoma epidermóide de conjuntiva, com invasäo para a intimidade do globo ocular e que posteriormente evoluiu com metástase para linfonodos loco-regionais. O tratamento realizado foi exanteraçäo, e posteriormente, na ocorrência de metástase, parotidectomia e quimioterapia. Apesar de ser um caso recente, o paciente apresenta-se bem

Carcinoma epidermóide conjuntival associado a melanoma cutâneo / Epidermoid carcinoma of the conjunctiva associated to cutaneous melanoma

Os autores relatam um caso de carcinoma epidermóide de conjuntiva associado a melanoma cutâneo, comentam acerca da etiologia e seus fatores comuns, fazem comentários acerca da correta denominaçäo das lesöes neoplásicas de conjuntiva

[Static and dynamic infrared thermometry and thermography in malignant melanoma of the uvea and conjunctiva]. / Statische und dynamische Infrarotthermometrie und -thermographie beim malignen Melanom der Uvea und Konjunktiva.

BACKGROUND: Thermometry and -graphy prove asymmetries of the circulation. Contact-free thermometry of the cornea is applied to find out whether this method can contribute to differential diagnosis of ocular melanomas. MATERIALS AND METHODS: Under standardized conditions the temperature of the cornea was measured in 30 patients with malignant melanoma of the choroid and the conjunctiva and 35 healthy subjects. The instruments were a handpyrometer (HPM, Messgerätewerk, Magdeburg) and the thermovision camera of AGA Infrared System, Sweden, with dynamic recording in colour. RESULTS: In healthy subjects there are no significant asymmetries in temperature under standardized conditions. The temperature of the cornea is elevated in both malignant melanomas of the choroid and the conjunctiva. CONCLUSIONS: Thermometry and -graphy of the cornea can contribute to the differentiation of malignant ocular melanomas from other ophthalmological diseases. They are also suited in long term observation of the course of the melanomas.

[Sturge-Weber syndrome: glaucoma with elevated episcleral venous pressure]. / Sturge-Weber-Syndrom: Glaukom mit erhöhtem episkleralen Venendruck.

The present report describes clinical findings in 13 patients with Sturge-Weber syndrome, 11 of whom had glaucoma (22-52 mm Hg). The episcleral venous pressure (EVP) was high in all glaucomatous eyes (14-23 mm Hg). Tonography showed a marked decrease in outflow facility. The observations suggest that glaucoma in Sturge-Weber syndrome is caused by a) elevated episcleral venous pressure and b) secondary damage of the trabecular meshwork with reduction of outflow facility. The cause and pathogenesis of these lesions are discussed. In nine patients a diffuse angiomatosis involving most of the choroid (1.5-4.0 mm thick), as well as the episcleral perilimbal plexus, was demonstrated by ultrasonography.

Cystic conjunctival masses as the presenting sign of disseminated lymphoma.

Systemic malignant lymphoma rarely presents as conjunctival masses. Biopsy of unusual cystic conjunctival masses from a 49-year-old woman showed malignant lymphoma. On subsequent examination, the patient was found to have disseminated systemic malignant lymphoma. Examination of possible predictive clinical features of the conjunctival lesions showed that these were of little value in diagnosing the systemic malignancy.