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PubMed1991

HMB-45 immunohistochemical staining of conjunctival melanocytic lesions.

HMB-45 is a monoclonal antibody recently described as being highly specific for melanocytic proliferations of the skin and in metastases of melanotic lesions. To determine a possible role for HMB-45 in ophthalmic pathology, 45 conjunctival lesions, including 23 melanomas, were analyzed using immunohistochemical techniques with anti-S-100 and HMB-45 as primary antibodies. Nineteen (82.6%) of the melanomas exhibited HMB-45 positivity, and 19 (82.6%) contained S-100 protein, with concordance of all but two cases. Seven cases of primary acquired melanosis were studied; one (33%) of three with atypia was HMB-45 positive, as were two (50%) of four without atypia. Among nevi, 1 (9.1%) of 11 showed faint staining with HMB-45. Fifteen conjunctival epithelial dysplasias were negative with HMB-45. At present, HMB-45 appears to offer no great advantage over S-100 protein in the analysis of conjunctival melanomas. Its role in the distinction of benign from atypical or malignant junctional melanocytic proliferations remains unclear.

Antibodies, Monoclonal↗
PubMed1992

Cellular blue nevus of the conjunctiva.

BACKGROUND: Cellular blue nevi of the conjunctiva are extremely rare, and their natural history and malignant potential have not been fully ascertained. METHODS: A report of an unusually well-documented case of growth of a cellular blue nevus is presented, along with a review of current knowledge of this lesion. RESULTS: A 71-year-old woman presented with a darkly pigmented raised lesion of the conjunctiva, which had slowly enlarged over 47 years. There were nonconfluent areas of involvement of the upper and lower lids. Results of biopsy showed the lesion to be a cellular blue nevus, with no evidence of malignancy. CONCLUSION: This well-documented case of slow growth and spread without malignant transformation adds to the knowledge of this rare lesion.

Aged↗
PubMed1992

Malignant lymphoma of the ocular adnexa associated with the benign lymphoepithelial lesion of the parotid glands. Report of two cases.

BACKGROUND: The benign lymphoepithelial lesion of the salivary/lacrimal glands is generally regarded as a lymphoproliferative disorder that may be associated with Sjögren syndrome. Although lymphomatous transformation in patients with Sjögren syndrome is well documented, few reports have appeared describing similar findings in patients with a benign lymphoepithelial lesion. METHODS: The authors report the unusual occurrence of malignant lymphoma involving the ocular adnexa in two patients with a histopathologically documented benign lymphoepithelial lesion of the parotid glands. RESULTS: Both patients developed B-cell lymphomas. The eyelid tumor in case 1 expressed IgM/kappa cell surface markers by flow cytometry, and the orbital/conjunctival masses in case 2 showed neoplastic nodules of B cells that were only immunoreactive to lambda light chains (avidin-biotin complex method). CONCLUSION: To the authors' knowledge, this is the first report of ocular adnexal lymphoma arising in patients with a benign lymphoepithelial lesion of the parotid glands. This report establishes that ocular adnexal lymphoma may arise in a patient with a preexisting benign lymphoepithelial lesion.

Aged↗
PubMed1992

Treatment of ocular adnexal Kaposi's sarcoma in acquired immune deficiency syndrome.

PURPOSE: To determine the most effective, safe, and cost-efficient treatment for ocular adnexal Kaposi's sarcoma in the acquired immune deficiency syndrome. METHODS: Eighty-two patients with ocular adnexal Kaposi's sarcoma related to the acquired immune deficiency syndrome were examined, and 25 were selected to participate in a 3-year study that treated the ocular lesions based on stage of development and location. RESULTS: Of 14 patients with bulbar conjunctival Kaposi's sarcoma treated with surgical excision, 2 stage III lesions recurred during a follow-up that ranged from 8 to 31 months; no stage I or stage II lesion recurred. Of 7 patients with eyelid Kaposi's sarcoma treated with cryotherapy, 2 stage III lesions recurred during a follow-up that ranged from 9 to 24 months; no stage I or stage II lesion recurred. Four patients with stage III Kaposi's sarcoma of the bulbar conjunctiva were treated with fluorescein angiography-based surgical excision. None of these lesions recurred during a follow-up that ranged from 4 to 8 months. CONCLUSION: A treatment regimen for ocular adnexal Kaposi's sarcoma related to the acquired immune deficiency syndrome based on tumor stage and location, using cryotherapy and surgical excision with or without fluorescein angiography, is effective, safe, and cost-efficient.

Acquired Immunodeficiency Syndrome↗
PubMed1992

Intraepithelial sebaceous carcinoma of the conjunctiva and skin of the eyelid.

The authors report a 65-year-old woman with a 10-year history of conjunctivitis and progressive loss of lashes due to widespread intraepithelial sebaceous carcinoma of the conjunctiva and skin of the eyelid. After surgery, serial sections of the entire conjunctiva and eyelids showed a single focus of invasive carcinoma in the bulbar conjunctiva, which probably arose from the epithelium. The absence of cilia on the affected lower lid was associated with neoplastic obliteration of the cilial orifices and low-grade, smoldering lipogranulomatous inflammation. This type of tumor-related alopecia is likely to masquerade as blepharitis because there is no identifiable tumefaction or clinical evidence of recurrent chalazia to suggest an underlying sebaceous carcinoma. The 10-year history of "conjunctivitis" before diagnosis indicates that intraepithelial sebaceous carcinoma may have a prolonged clinical course.

Aged↗
PubMed1993

Primary basal cell carcinoma of the limbal conjunctiva.

BACKGROUND: The authors describe a 66-year-old man with primary basal cell carcinoma of the conjunctiva. He presented with a fleshy nodular conjunctival lesion of 4 months' duration, located at the nasal limbus of the left eye. No associated cutaneous lesions were present. RESULTS: The lesion was completely excised, and results of histopathologic examination showed a primary basal cell carcinoma of the conjunctiva. CONCLUSION: This report provides clinicopathologic evidence that primary basal cell carcinoma can rarely occur in the conjunctiva. The authors summarize two well-documented previously reported cases of this unusual lesion.

Aged↗
PubMed1995

Prevalence of primary acquired melanosis and nevi of the conjunctiva and uvea in the dysplastic nevus syndrome. A case-control study.

PURPOSE: To investigate whether conjunctival and uveal nevi and primary acquired melanosis are more common in individuals with the dysplastic nevus syndrome than in control subjects derived from the general population. METHODS: Power calculations were used to determine the sample size. After invitation, 162 individuals with the dysplastic nevus syndrome and 119 control subjects, matched for sex and age but otherwise randomized from the Stockholm county census file, were entered into the study. All individuals were examined in a masked fashion by the same ophthalmologist, and the presence of conjunctival and uveal melanocytic lesions and the iris color, skin type, and hair color of each individual were recorded. Contingency tables and odds ratios were used for statistical evaluation. RESULTS: The proportions of individuals with the dysplastic nevus syndrome featuring primary acquired melanosis of the conjunctiva, or nevi of the iris and choroid were not significantly different from those of control subjects. However, individuals with the dysplastic nevus syndrome appeared to have a more sun-sensitive skin type and a reddish or blond hair color more often than control subjects. CONCLUSION: In contrast to previous reports, this study suggests that ocular melanocytic lesions are no more common in individuals with the dysplastic nevus syndrome than in the general population. Therefore, this work does not provide support that periodic ophthalmic surveillance of individuals with the dysplastic nevus syndrome for the purpose of detecting conjunctival or uveal melanomas, or their precursors, is meaningful.

Adult↗
PubMed1995

Topical 5-fluorouracil in treating epithelial neoplasia of the conjunctiva and cornea.

PURPOSES: To evaluate the efficacy of topical 5-fluorouracil (5-FU) in treating conjunctival and corneal epithelial neoplasia. METHODS: Three patients underwent surgical excision of bulky disease followed by topical 1% 5-FU in artificial tear base for 2 to 3 weeks or until epithelial separation occurred. An additional three patients underwent treatment with topical 1% 5-FU alone. RESULTS: Minimum follow-up was 6 months. In one patient with conjunctival in situ carcinoma and no detectable normal conjunctiva, who had normal findings on conjunctival histologic examination after application of topical 5-FU, a focus of invasive disease requiring orbital exenteration. One patient had a favorable response to 5-FU therapy but required a repeat excision for control of bulky disease. Four patients have remained disease-free for 10, 13, 18, and 30 months after topical 5-FU therapy. CONCLUSION: With its potential selective toxicity on dysplastic epithelium, topical 5-FU shows promise as an adjunctive treatment for managing conjunctival and corneal epithelial neoplasia.

Administration, Topical↗
PubMed1995

Burkitt lymphoma presenting as a conjunctival mass.

PURPOSE: A sporadic Burkitt lymphoma, which presented as a primary conjunctival mass, demonstrates the approach to clinical diagnosis, staging, and current management of this rapidly growing malignant tumor, as well as newer concepts of its pathogenesis. PATIENT AND METHODS: A 16-year-old girl had rapid development of a conjunctival mass 6 weeks after an infectious mononucleosis-like illness, associated with a positive monospot test. A biopsy of the tumor was immunostained for lymphocyte markers. Additional clinical workup included computed tomographic scanning, nasopharyngeal biopsy, and studies of blood, bone marrow, and spinal fluid. In situ hybridization for Epstein-Barr virus (EBV)-encoded nuclear RNA was performed on the tumor and adenoidal tissue. RESULTS: The conjunctival biopsy showed a typical Burkitt lymphoma with markers positive for predominantly B lymphocytes. Computed tomographic scans indicated residual tumor in the anterior orbit. Biopsy of thickened adenoidal tissue showed only benign lymphoid hyperplasia. Evidence of EBV infection was found in the adenoidal tissue but not in the tumor cells. The patient was treated with combined chemotherapy on a Pediatric Oncology Group Study protocol for localized non-Hodgkin lymphoma, and is disease-free 4 1/2 years after diagnosis. CONCLUSIONS: A sporadic Burkitt lymphoma arising in the conjunctiva appears to be the first reported in this site. The lymphoma responded well to chemotherapy, which is now the usual course in stage 1 disease. A direct role for EBV in the oncogenesis of this sporadic tumor, similar to that in the endemic form, could not be supported because EBV-encoded nuclear RNA was absent in the tumor cells.

Adolescent↗
PubMed1996

Scleritis and mucosal-associated lymphoid tissue lymphoma: a new masquerade syndrome.

PURPOSE: To present a new masquerade syndrome showing features of mucosal-associated lymphoid tissue (MALT) lymphoma associated with choroidal white dots and scleritis. Differentials including systemic lymphoma, central nervous system lymphoma, and etiologies of white-dot syndromes and scleritis are discussed. PATIENTS AND METHODS: A 42-year-old man who had decreased vision and ocular redness of his right eye for 4 years had a biopsy-proven diffuse anterior and posterior scleritis associated with intense circumferential perilimbal chemosis and ipsilateral yellow-white choroidal dots. A new conjunctival biopsy was performed because of unresponsiveness to high-dose systemic steroid and cyclophosphamide therapy. Immunostains for lymphocyte markers were preformed. RESULTS: A morphologically and immunohistochemically typical, monotypical mu-kappa immunoglobulin light chain secreting B-cell MALT-lymphoma was diagnosed. Eighteen months after completion of radiotherapy, the patient recovered completely, except for the choroidal dots, which remained unchanged. CONCLUSION: When scleritis, even histologically proven, fails to respond to immunosuppressive therapy, a new biopsy is mandatory to rule out a misdiagnosed MALT lymphoma.

Adult↗
PubMed1997

Mitomycin C treatment for conjunctival-corneal intraepithelial neoplasia: a multicenter experience.

OBJECTIVE: The purpose of the study is to evaluate the efficacy and risks of topical mitomycin C (MMC) for conjunctival-corneal intraepithelial neoplasia (CCIN). DESIGN: The study design was a clinical case series of CCIN. PARTICIPANTS: Seventeen patients, 16 with biopsy-confirmed CCIN and 1 with invasive squamous cell carcinoma (SCC), were included in the study. INTERVENTION: Patients received topical drops of MMC 0.02% to 0.04% four times daily from 7 to 28 days. Retreatment was done in cases of lesion recurrence. MAIN OUTCOME MEASURES: The size of the CCIN before and after the treatment and ocular complications post-MMC application were evaluated. RESULTS: Ten patients remained disease-free after one course of MMC application. In one case, residual CCIN remained very small without regrowth. In the one patient with invasive SCC and in five patients with CCIN, regrowth occurred within 6 months of the first treatment. After retreatment, invasive SCC and CCIN in an additional two patients were eradicated. In two cases, although the size of the lesions decreased after two and three applications of MMC, regrowth occurred, and the CCIN returned to its original size. In the final case, limited recurrence has occurred and no retreatment has been done. The complications of MMC use included mild-to-moderate conjunctival hyperemia and mild allergy, which resolved after discontinuation of the treatment. Severe pain manifested when treatment was longer than 14 days. CONCLUSIONS: Application of topical MMC is an efficient treatment for most but not all cases of CCIN.

Administration, Topical↗
PubMed1997

Oculocerebral dysgenesis in the linear nevus sebaceous syndrome.

PURPOSE: The authors document the association of peripapillary staphyloma and an atypical variant of hemimegalencephaly with the linear nevus sebaceous syndrome. BACKGROUND: Linear nevus sebaceous syndrome is an uncommon neurocutaneous disorder that has a propensity to involve the eyes. METHODS: Clinical, histopathologic, and neuroimaging findings are examined in a child with linear nevus sebaceous syndrome. RESULTS: In addition to bilateral peripapillary staphylomas, ophthalmologic abnormalities included a corneal dermoid, a complex conjunctival choristoma, macular hypoplasia, and optic nerve hypoplasia with contralateral optic atrophy. Magnetic resonance imaging disclosed a rare form of hemimegalencephaly characterized by hypoplasia of an optic radiation within the enlarged, dysgenetic cerebral hemisphere. CONCLUSION: The spectrum of oculocerebral dysgenesis in the linear nevus sebaceous syndrome can be expanded to include peripapillary staphyloma and atypical hemimegalencephaly with hypoplasia of an optic radiation.

Brain↗
PubMed1997

Late recurrences and the necessity for long-term follow-up in corneal and conjunctival intraepithelial neoplasia.

PURPOSE: The purpose of the study was to elucidate the natural history of corneal-conjunctival intraepithelial neoplasia (CIN) and suggest treatment and follow-up guidelines. METHODS: The records of all histologically proven cases of CIN at the Royal Victorian Eye and Ear Hospital between 1979 and 1994 were reviewed. RESULTS: Seventy-nine eyes of 76 patients had a pathologic diagnosis of CIN and were observed for up to 15 years. The lesion recurred in 31 eyes (39%) overall. There was no statistical difference shown in the likelihood of recurrence based on histologic classification. Complete excision was attempted in each case. In 18 eyes, dysplastic cells were evident at the excision margin. Ten (56%) of these tumors recurred, compared to a 33% recurrence rate in completely excised lesions. The time to the first recurrence ranged from 33.0 days to 11.5 years between the first and second surgeries, with 11 lesions recurring after more than 4 years. Incompletely excised lesions reappeared more rapidly (average, 2.5 years) than did those with clear surgical margins (average, 3.8 years). Seven cases progressed to invasive squamous cell carcinoma and four cases led to blindness or removal of the eye. CONCLUSIONS: This study suggests that excision margin at the time of surgery is the most important factor in predicting recurrence. The slow growth of the recurrent lesions combined with the ever-present malignant potential leads the authors to suggest that all patients with a history of CIN warrant annual follow-up for the remainder of their lives.

Adult↗
PubMed1998

Lymphoproliferative lesions of the ocular adnexa. Analysis of 112 cases.

OBJECTIVE: Lymphoproliferative lesions of the ocular adnexa were analyzed to examine (1) the suitability of the Revised European-American Lymphoma (REAL) classification for the subtyping of the lymphomas in these sites; (2) the predictive value of the REAL classification for the evolution of these tumors; and (3) the frequency and prognostic impact of tumor type, location, proliferation rate (Ki-67 index), p53, CD5 positivity and the presence of monoclonality within these tumors. DESIGN: Retrospective review. METHODS: The clinical, histomorphologic, immunohistochemical, and molecular biologic (polymerase chain reaction [PCR]) features of lymphoid proliferations of the ocular adnexa were studied. STUDY MATERIALS: The ocular adnexal lymphoproliferative lesions were located as follows: orbit in 52 patients (46%), conjunctiva in 32 patients (29%), eyelid in 23 patients (21%), and caruncle in 5 patients (4%). RESULTS: Reactive lymphoid hyperplasia was diagnosed in 12 cases and lymphoma in 99 cases; 1 case remained indeterminate. The five main subtypes of lymphoma according to the REAL classification were extranodal marginal-zone B-cell lymphoma (64%), follicle center lymphoma (10%), diffuse large cell B-cell lymphoma (9%), plasmacytoma (6%), and lymphoplasmocytic lymphoma (5%). Age, gender, and anatomic localization of the lymphomas did not have prognostic significance during a follow-up period of 6 months to 16.5 years (mean, 3.3 years). Extent of disease at time of presentation was the most important clinical prognostic factor: advanced disease correlated with increased risk ratios of having persistent disease at the final follow-up and with lymphoma-related death (P < 0.001). Histomorphologic features and immunohistochemical markers positively correlating with disseminated disease at presentation, stage at final follow-up, and occurrence of lymphoma-related death included cytologic atypia (P < 0.001), MIB-1 proliferation rate (P < 0.001), and tumor cell p53 positivity (P < 0.001). The MIB-1 proliferation rates greater than 20% in extranodal marginal-zone B-cell lymphoma corresponded to at least stage II lymphoma (P < 0.05). CONCLUSION: The REAL classification is suitable for the subdivision of the ocular adnexal lymphomas. The MIB-1 proliferation rate and p53 positivity may aid the prediction of disease stage and disease progression, whereas PCR can support the diagnosis and reduce the number of histologically indeterminate lesions.

Adolescent↗
PubMed1999

Perilesional and topical interferon alfa-2b for conjunctival and corneal neoplasia.

OBJECTIVE: To evaluate the role of combined subconjunctival and topical interferon alfa-2b (IFN alpha 2b) in the treatment of conjunctival and corneal intraepithelial neoplasia (CIN). DESIGN: Noncomparative case series. PARTICIPANTS: Six patients with histologically proven CIN or recurrences of histologically proven CIN were studied prospectively. INTERVENTION: Patients were given a single subconjunctival/perilesional injection of recombinant IFN alpha 2b (Schering Plough, Kenilworth, NJ) 3 million international units (IU) in 0.5 ml and then started receiving topical interferon drops (1 million U/ml) four times a day. Patients were followed weekly until complete resolution of the tumor. After 1 week, patients with minimal response while receiving topical therapy were retreated with perilesional injections three times a week until resolution. Patients received topical interferon drops for a month after clinical resolution of the lesion. MAIN OUTCOME MEASURES: Patients were followed clinically and photographically for evidence of tumor resolution. RESULTS: The authors present a series of six patients who were treated successfully with a combination of subconjunctival/perilesional and topical IFN alpha 2b. All six patients had complete clinical resolution of the CIN lesion within 6 weeks of initiation of treatment. In the time of follow-up (average, 7.2 months; range, 2-11 months), there have been no treatment failures or recurrences. CONCLUSION: IFN alpha 2b may be a viable medical alternative to surgical excision for primary or recurrent CIN.

Administration, Topical↗
PubMed1999

Conjunctival lymphoma masquerading as chronic conjunctivitis.

OBJECTIVE: Malignant lesions of the conjunctiva may present with slowly evolving signs resembling inflammation. The authors describe the clinical and histopathologic findings of two patients with bilateral conjunctival lymphoma who presented with a history of chronic conjunctivitis without clinically noticeable subconjunctival nodules. DESIGN: Case report. PARTICIPANTS: Two patients. INTERVENTION: Both patients underwent conjunctival biopsy for evaluation of persistent conjunctival inflammation that did not respond to various medical treatment methods. RESULTS: Histopathologic examination revealed extranodal marginal zone B-cell lymphoma (mucosa-associated lymphoid tissue type) in one patient and follicle center lymphoma in the other patient. Both patients subsequently received radiation therapy and achieved a complete remission with no evidence of recurrence in the follow-up period of 20 and 16 months, respectively. CONCLUSIONS: Conjunctival lymphoma should be included in the differential diagnoses of chronic conjunctivitis. Persisting signs and symptoms of conjunctivitis not responding to standard treatment should prompt biopsy.

Adult↗