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Clinical features predictive of orbital exenteration for conjunctival melanoma.

PURPOSE: Conjunctival malignant melanoma can sometimes be difficult to control locally, and orbital exenteration may be necessary. To our knowledge, the risk factors that portend exenteration have not been previously identified. The purpose of this study was to review patients who underwent orbital exenteration for advanced disease and to identify retrospectively the clinical factors predictive of ultimate exenteration. METHODS: The records of patients with conjunctival melanoma who were managed on the Oncology Service at Wills Eye Hospital from 1974 to 1997 were reviewed. Factors predictive of orbital exenteration were analyzed using Cox proportional hazards regression models. RESULTS: Of 151 consecutive patients with conjunctival melanoma, 20 (13%) required orbital exenteration for advanced disease. The number of melanoma excisions performed before referral ranged from 0 to 11, with a mean of 2. The clinical factors predictive of orbital exenteration on multivariable analysis were visual acuity of 20/200 or worse, lack of tumor pigmentation, and extralimbal tumor location. All exenterations were performed using an eyelid-sparing technique. At mean follow-up of 51 months after exenteration, 4 patients (20%) had died of metastasis, 3 (15%) were alive with metastasis, and 13 (65%) had not developed metastasis. CONCLUSIONS: Conjunctival melanoma can show aggressive local behavior, and orbital exenteration is necessary in up to 13% of cases at a tertiary referral center. Patients with risk factors such as poor visual acuity, amelanotic tumor, and extralimbal tumor location may require wider excision and radiation therapy to control the tumor and avoid orbital exenteration. It is hoped that modern surgical approaches will decrease the need for orbital exenteration.

Adult↗

Sentinel node biopsy for orbital and ocular adnexal tumors.

PURPOSE: To describe a technique for sentinel node mapping and biopsy in patients with orbital or adnexal tumors. METHODS: Five patients with orbital and adnexal tumors were studied. Two patients had malignant eyelid melanomas (one of the skin and one of the conjunctiva), one with orbital invasion. Two patients had sebaceous gland carcinoma, and one patient had a mucoepidermoid carcinoma of the conjunctiva; 500 microCi of Technetium-99m sulfur nanocolloid (Nycomed Amersham, Princeton, NJ) diluted to 1.0 mL was injected intradermally at the lateral canthus. The patients were positioned as they would be during surgery. Lymphoscintigraphy was performed by means of anterior, lateral, and oblique views. The tracer was followed to the first lymphatic basin, and the sentinel node was identified. Cutaneous markers were placed to denote the site. During surgery, lymphoscintigraphy scans and a hand-held gamma probe were used to locate the sentinel node. Once excised, the sentinel node was sent for histopathology. Frozen sectioning confirmed the presence of lymphoid tissue. Permanent sections with immunohistochemical markers were performed to examine for metastatic disease. RESULTS: The sentinel node biopsy technique was applied to 5 patients with orbital and adnexal tumors. All lymph nodes were free of tumor on histopathologic examination. CONCLUSIONS: Sentinel node mapping and biopsy are possible for orbital and adnexal tumors. The morbidity of elective lymph node dissection and adjuvant radiotherapy can be avoided. Our results are preliminary, and further work must be done to identify the lymphatic basins of the orbit and ocular adnexa.

Adenocarcinoma, Sebaceous↗

Multiple endocrine neoplasia type III.

The multiple endocrine neoplasia (MEN) syndromes are autosomally dominant inherited disorders in which hyperplastic or neoplastic changes occur in a wide variety of tissues. The specific syndromes are classified according to the endocrine glands affected. MEN type I consists of an aggregation of tumors of parathyroid, pancreatic, and pituitary glands. The association of medullary carcinoma of the thyroid (MCT) and pheochromocytoma is called MEN type II or type IIA, and if combined with mucosal neuromas, intestinal ganglioneuromatosis, and prominent corneal nerves, is named MEN type III or type IIB. Individuals afflicted with MEN type III are characterized by a marfanoid habitus, mucosal neuromas involving oral and ocular tissues, and a number of ophthalmologic findings including prominent corneal nerves, thickened eyelids, and subconjunctival neuromas. These features are easily recognized during the ocular exam, allowing the ophthalmologist to make an early diagnosis of this syndrome prior to the onset of life-threatening manifestations like medullary thyroid carcinoma and pheochromocytoma.

Adult↗

Massive precorneal extension of squamous cell carcinoma of the conjunctiva.

A 73-year-old man had only light perception vision from a slowly enlarging fleshy papillomatous mass covering the entire cornea. The lesion was excised completely, leaving a clear corneal bed and 20/25 vision. Histopathologically, the lesion was found to be squamous cell carcinoma in situ with early microinvasion of the basement membrane. Viral studies for human papilloma virus were negative. This case represents a massive superficial involvement of the cornea by squamous cell carcinoma and shows the resistance of Bowman's membrane to tumor penetration.

Aged↗

Ocular surface neoplasia masquerading as chronic blepharoconjunctivitis.

PURPOSE: To present the clinical characteristics and difficulties in the diagnosis of various ocular surface malignancies mimicking features of chronic blepharoconjunctivitis and to summarize the current therapeutic approach and prognosis of patients. METHODS: Six patients with slowly evolving signs of persistent inflammation underwent a conjunctival biopsy after a prolonged course of medical treatment. The medical records of the patients were reviewed. RESULTS: Histopathologic examination of the biopsy specimens revealed intraepithelial squamous neoplasia (one patient), invasive squamous cell carcinoma (one patient), sebaceous carcinoma (two patients), and conjunctival lymphoma (two patients). CONCLUSION: Although uncommon, ocular surface malignancies may involve the conjunctiva diffusely and present as chronic conjunctivitis. A high index of suspicion and an early histopathologic examination are essential to not delay diagnosis.

Adenocarcinoma, Sebaceous↗

Conjunctival epithelial neoplasias in organ transplant patients receiving cyclosporine therapy.

PURPOSE: To report two cases of conjunctival epithelial neoplasias occurring after long-term systemic cyclosporine therapy in organ transplant recipients. METHODS: Case report. Each patient underwent an excision of an extensive limbal conjunctival lesion, followed by a histopathologic analysis. RESULTS: Histopathologic examination results revealed a carcinoma in situ and a squamous cell carcinoma in patients 1 and 2, respectively. Recurrence occurred in patient 1, who needed topical mitomycin C 0.02% treatment. No recurrence was observed in patient 2 after a follow-up of 24 months. CONCLUSION: To our knowledge, these are the first reported cases of its kind after longterm systemic cyclosporine therapy. Further studies will be needed to determine the exact role of cyclosporine in the induction of conjunctival epithelial neoplasias and the need for regular ophthalmologic examination of transplant recipients.

Carcinoma in Situ↗

Treatment of conjunctival intraepithelial neoplasia with topical drops of mitomycin C.

PURPOSE: To evaluate the efficacy and risks of complications of topical mitomycin C (MMC) for small-size conjunctival intraepithelial neoplasia (CIN). METHODS: Eight patients with clinically diagnosed CIN <8 mm were included in the study. Patients received topical drops of MMC, 0.02-0.04%, 4 times daily for 14 days. Retreatment was done when lesions were not eliminated or recurred after the first treatment. RESULTS: Three patients remained disease free after one course of MMC application. Retreatment was done in four patients because of lesions that decreased in size but were not eliminated, and for regrowth in one case. After retreatment, the lesions were eradicated in four patients, whereas in one patient, the treatment failed, and the lesion was surgically excised. The complications of MMC use included mild conjunctival hyperemia in two patients and mild allergy in one patient, which resolved after discontinuation of the treatment. CONCLUSION: Application of topical MMC is an effective treatment for most but not all cases of small-size CIN.

Adult↗

Bilateral conjunctival mucosa-associated lymphoid tissue lymphoma misdiagnosed as allergic conjunctivitis.

PURPOSE: This report aimed to discuss two nearly identical cases of primary conjunctival mucosa-associated lymphoid tissue (MALT) lymphoma mimicking allergic conjunctivitis. METHODS: Two patients were referred for intractability to conventional treatment of initial diagnoses of allergic conjunctivitis. On ocular examination, normally pigmented, giant papilla-like lesions were found bilaterally in the upper conjunctiva. Excisional biopsies were performed. RESULTS: Histopathologic and immunohistochemical examination of the conjunctival biopsies indicated MALT lymphoma in both patients. The patients subsequently received radiation therapy and achieved complete remission with no evidence of recurrence during follow-up periods of 13 and 11 months. CONCLUSION: Conjunctival low-grade lymphoma may share similar clinical features with allergic conjunctivitis. Ophthalmologists should be concerned that primary conjunctival low-grade malignant lymphoma can be misdiagnosed as allergic conjunctivitis

Adult↗

Regression of presumed primary conjunctival and corneal intraepithelial neoplasia with topical interferon alpha-2b.

PURPOSE: To evaluate topical interferon alpha-2b (IFNalpha2b) as a lone therapy in the treatment of primary conjunctival and corneal intraepithelial neoplasia (CIN). METHODS: Noncomparative, prospective, interventional case series. Seven patients from three institutions, treated between February and October 1999, with presumed primary CIN lesions (clinically diagnosed by corneal specialists) were given topical IFNalpha2b drops (1 million units/mL) four to six times daily. Follow-up was performed biweekly until there was complete clinical resolution of the presumed CIN lesions. Patients were to continue topical IFNalpha2b drops for 1 month after clinical resolution. Patient charts and clinical photographs were reviewed, and data were analyzed. RESULTS: All seven eyes had complete resolution of the presumed CIN lesions after an average of 77.0 +/- 59.2 days (range, 28-188 days). Average posttreatment follow up was 12.4 +/- 2.5 months (range, 9-16 months). No patients were lost to follow-up. No recurrences have yet been seen. Side effects of treatment were limited to mild conjunctival hyperemia and follicular conjunctivitis in four (57.1%) eyes. In all cases, there was total resolution of conjunctival hyperemia and follicular changes within 1 month after cessation of the medication, without additional treatment. CONCLUSIONS: Topical IFNalpha2b alone may be an effective treatment of primary CIN. It appears to be a safe alternative to radiation, intralesional IFNalpha2b injection, and surgical excision with cryotherapy. Larger population studies with longer follow-up are recommended to better assess the risk of recurrence and other possible adverse effects.

Administration, Topical↗

The intraoperative use of mitomycin-C in excision of ocular surface neoplasia with or without limbal autograft transplantation.

PURPOSE: To evaluate the efficacy of intraoperative mitomycin-C in excision of ocular surface neoplasia for prevention of recurrence. METHODS: Seven patients (eight eyes), three men and four women, aged 56 to 87 years (mean, 73.8 years), with lesions suspicious for corneal or conjunctival neoplasia, were operated on between October 1998 and March 2000. During excision of the lesion, mitomycin-C 0.02% was applied intraoperatively for 5 minutes. In two cases, excision was combined with conjunctival limbal autograft transplantation. All excised lesions were sent for histopathologic evaluation. RESULTS: During the follow-up period ranging from 6 to 28 months (mean, 16 months) one patient (one eye) died of an unrelated cause. Histopathologic study showed four cases of squamous cell carcinoma, one case of carcinoma in situ, two cases of dysplasia, and one case of actinic keratosis. Of the eight eyes, no clinical recurrence of the lesion occurred in seven eyes, whereas one eye with squamous cell carcinoma showed mild recurrence 5 months after surgery and was successfully treated with topical mitomycin-C. Up to the last follow-up of this case 10 months later, the lesion did not recur. CONCLUSION: The excision of conjunctival and corneal epithelial neoplasia combined with the intraoperative use of mitomycin-C seems to reduce the recurrence rate. The combined use of mitomycin-C and conjunctival limbal autograft transplantation in two cases did not alter the surgical outcome. More cases and a longer follow-up are needed to establish the efficacy of such an approach.

Aged↗

Combined surgery, cryotherapy, and mitomycin-C for recurrent ocular surface squamous neoplasia.

PURPOSE: To report the outcome of combined excision, cryotherapy, and antimetabolite treatment of recurrent ocular surface squamous neoplasia. METHODS: The patients with recurrent ocular surface squamous neoplasia were treated by excision of lesion, cryotherapy of limbus, and conjunctival margin followed by 0.02% Mitomycin C application at the time of surgery. Patients have been followed up for at least 1 year. RESULTS: A total of five eyes of five patients with recurrent ocular surface squamous neoplasia were treated by combined excision, cryotherapy, and Mitomycin C. Histopathologic diagnosis included invasive squamous cell carcinoma in four cases and squamous dysplasia in one case. No recurrences have been noted for a follow-up period of more than 1 year now. CONCLUSION: Combining excision with cryotherapy and Mitomycin C application at the time of surgery is a very effective therapy for recurrent ocular surface squamous neoplasia. It is relevant for cases in large, poor countries where patients present late and are less likely to come for follow-up care.

Adult↗

Mitomycin C therapy for conjunctival-corneal intraepithelial neoplasia.

PURPOSE: To evaluate the efficacy and safety of topical mitomycin C (MMC) for conjunctival-corneal intraepithelial neoplasia (CCIN). METHODS: One patient with primary CCIN received seven applications for 3 minutes of mitomycin C 0.02%, for 2 weeks, on alternative days. The size of the CCIN before and after the treatment and ophthalmic mitomycin C related complications were evaluated. RESULTS: The lesion started to regress during the second month after the last application of mitomycin C, and by the third month it disappeared completely. The patient remains disease free after 36 months follow up. The complications of mitomycin C included a mild tearing and a slight conjunctival hyperemia that resolved 7 days after the end of the therapy. CONCLUSION: Multiple applications of mitomycin C could be an effective treatment for selected cases of CCIN.

Aged↗

The use of postoperative topical mitomycin C in the treatment of recurrent conjunctival papilloma.

PURPOSE: To describe the use of postoperative topical mitomycin C (MMC) in the treatment of recurrent conjunctival papilloma. METHODS: Case report. RESULTS: We report a 26-year-old man with recurrent conjunctival papilloma despite repeated surgical excision, cauterization, and cryotherapy. He was then treated with excision by cryotherapy, followed by a 2-week course of topical MMC eyedrops prescribed at postoperative day 7 (0.02 mg/mL, four times daily). No recurrence was observed 24 months postoperatively, and no complication was observed during the follow-up period. CONCLUSION: Postoperative topical MMC may be a useful adjunct in the management of recurrent conjunctival papilloma.

Adult↗

Prevalence of HIV with conjunctival squamous cell neoplasia in an African provincial hospital.

PURPOSE: To evaluate the prevalence of HIV seropositivity among patients with malignant conjunctival squamous cell carcinoma (SCC) and carcinoma in situ (CIS) and to reassess the potential linkage, albeit well documented, between ocular surface epithelial dysplasia (OSED) and HIV infection. PATIENTS AND METHODS: A case-control design study was conducted in an African provincial hospital. Twenty-three African black patients underwent excisional biopsy of conjunctival malignant lesions. In 18 of these patients, ELISA for HIV antibodies was performed prior to the excisional biopsy. RESULTS: Pathological evaluation revealed SCC in 12 (52%) patients, CIS in six (26%) patients, and Kaposi sarcoma (KS) in five (22%) patients. Eighteen patients (78.3%) agreed to take a serological HIV test, and among these, seropositivity for HIV was significantly (p < 0.01) higher (92.3%, 12 of 13) in the SCC/CIS subgroup than in a control group with benign conjunctival lesions (28.5%, two of seven). The most common (91.7%) clinical finding in the SCC/CIS/HIV group (12 patients) was corneal overriding. Conjunctival malignancy was the first presenting sign for AIDS in 50% of our patients. CONCLUSIONS: A significantly high rate of HIV seropositivity was found in a group of African black patients with conjunctival SCC/CIS compared with a control group with benign conjunctival lesions. The direct correlation between HIV infection and SCC/CIS was reconfirmed in a case-control study. Therefore, an HIV test should probably be performed in cases of conjunctival SCC/CIS or dysplasia, especially among patients in high-risk populations.

Adult↗

Squamous cell carcinoma of the conjunctiva in patients with atopic eczema.

PURPOSE: To report squamous cell carcinoma (SCC) of the conjunctiva in patients with atopic eczema. METHODS: A retrospective study of six patients with conjunctival SCC associated with atopic eczema. Clinical and pathologic records were reviewed. RESULTS: Ten patients with conjunctival SCC were seen from October 1999 to May 2002 at the Department of Ophthalmology at the University of Essen. Six of 10 patients had also atopic eczema. These six patients were treated either with local excision and ocular surface reconstruction or proton beam radiotherapy. None of the patients had local recurrences or metastases. CONCLUSION: Atopic eczema is a common dermatological disease. An association with conjunctival SCC has not been reported so far. Squamous cell carcinoma has also been found in patients with AIDS and other immunosuppressed individuals. The dysfunction of T lymphocytes found in these disorders might play a crucial role in the malignant transformation of conjunctival tissue. Because of the reported frequency of SCC, patients with atopic eczema should be monitored for abnormalities.

Adult↗

Combination of adult inclusion conjunctivitis and mucosa-associated lymphoid tissue (MALT) lymphoma in a young adult.

PURPOSE: To report a patient who was diagnosed with combined adult inclusion conjunctivitis (AIC) and mucosa-associated lymphoid tissue (MALT) lymphoma. METHODS: This is a case report. RESULTS: An 18-year-old male patient presented with chronic conjunctivitis and giant follicles. Evaluation by chlamydial antigen assay was positive. Conjunctival biopsy for the immunohistochemical stain and polymerase chain reaction of the left eye showed MALT lymphoma. CONCLUSIONS: MALT lymphoma can masquerade as other ocular surface diseases. Chlamydial infection causes chronic inflammation of the conjunctiva. Both of these diseases should be considered as a differential diagnosis of refractory follicular conjunctivitis. It is worthy of further study to determine whether chronic inflammation resulting from chlamydial infection increases the risk of MALT lymphoma or it is coincidental.

Adolescent↗

A case of conjunctiva-cornea intraepithelial neoplasia successfully treated with topical mitomycin C and interferon alfa-2b in cycles.

PURPOSE: To report a case of conjunctiva-cornea intraepithelial neoplasia (CCIN) treated with topical mitomycin C (MMC) and interferon alfa-2b in cycles. METHODS: A 52-year-old woman referred for limbal stem cell deficiency and epithelial defect was found instead to have CCIN. The diagnosis was confirmed by impression cytology and treated in cycles with topical 0.02% MMC for 14 days in the first cycle, 12 days in the second cycle, and 3 days in the third cycle followed by topical interferon alfa-2b 1 x 106 U/mL for 11 days. RESULT: Topical MMC for 2.5 cycles resulted in 95% reduction of CCIN and improvement of 2 lines of vision. Because of intolerance to MMC, she was switched to topical interferon alfa-2b for 11 days, resulting in total resolution of CCIN, which lasted for at least 10 months. CONCLUSIONS: After differentiation from a persistent corneal epithelial defect and limbal stem cell deficiency by dye staining and impression cytology, the patient in this case of CCIN was successfully treated with topical MMC and interferon alfa-2b in cycles.

Administration, Topical↗