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5-Fluorouracil for the treatment of intraepithelial neoplasia of the conjunctiva and cornea.

OBJECTIVE: To evaluate the efficacy of pulse dosing of topical 5-fluorouracil (5-FU) in the treatment of conjunctival and corneal intraepithelial neoplasia. DESIGN: Prospective, noncomparative case series. PARTICIPANTS: Seven patients with histologic evidence of intraepithelial neoplasia were identified by conjunctival biopsy or tumor excision. METHODS: Seven patients with a minimum of 7 months of follow-up were treated with pulsed dosing of 1% 5-FU. Topical 1% 5-FU was administered four times daily for 2 to 4 days for each cycle. The number of initial treatment cycles was two to six, with the time between cycles being 30 to 45 days. MAIN OUTCOME MEASURES: The presence or absence of clinically evident intraepithelial neoplasia was evaluated after each treatment interval. Patients were also monitored for adverse reactions to the use of topical 5-FU. RESULTS: Four patients remain disease free with a mean follow-up of 18.5 months (range, 7-36 months) with no additional treatment after the initial treatment cycles (mean, 3.75 cycles; range, 2-5 cycles). Three patients had recurrence of disease after the initial treatment cycles. Two patients were treated with additional cycles for recurrent disease (six cycles in one patient and five cycles in the other patient) and are free of disease at 20 and 21 months after treatment, respectively. One patient had persistent disease despite treatment with topical 5-FU and was treated with topical mitomycin C with resolution of the disease without recurrence for 16.5 months. No adverse reactions to pulse dose treatment with topical 5-FU were noted. CONCLUSIONS: Pulsed dosing with 1% topical 5-FU for the treatment of conjunctival and corneal intraepithelial neoplasia, alone or as an adjunct to excision of bulky disease, is a well-tolerated and effective method of treatment.

Administration, Topical↗

Sclerokeratitis: an unusual presentation of squamous cell carcinoma of the conjunctiva.

OBJECTIVE: To describe three cases of squamous cell carcinoma of the conjunctiva presenting with corneal and scleral inflammation, thinning, and perforation without any tumor formation. DESIGN: Three case reports. PARTICIPANTS: Three male subjects aged 76, 66, and 61 years. INTERVENTION: The subjects had symptoms of external ocular inflammation with focal corneal or scleral thinning and inflammation, as well as interstitial keratitis in two cases. Initial diagnosis of sclerokeratitis with limbal thinning was made in all three in addition to a pterygium in case 2. MAIN OUTCOME MEASURES: Diagnosis of the carcinoma was delayed for 2 to 24 months in two cases, whereas case 2 was diagnosed on excision of a pterygium from the involved area. RESULTS: All subjects developed intraocular extension after further scleral thinning and perforation without tumefaction. CONCLUSIONS: Squamous cell carcinoma of the conjunctiva may be seen without a distinct mass and can masquerade as sclerokeratitis, scleromalacia, or interstitial keratitis. It seems that diffuse growth with inflammation leads to thinning, necrosis, and perforation of the ocular wall with resultant intraocular spread. Squamous cell carcinoma should be considered in the differential diagnosis of corneal and scleral thinning, perforation, and inflammation of unknown cause, especially in the older male subject.

Aged↗

Local chemotherapy with interferon-alpha for conjunctival mucosa-associated lymphoid tissue lymphoma: a preliminary report.

OBJECTIVE: To evaluate the efficacy of subconjunctival interferon-alpha for the treatment of conjunctival mucosa-associated lymphoid tissue (MALT) lymphoma. DESIGN: Small, noncomparative, interventional case series. PARTICIPANTS: Five patients with histologically proven conjunctival MALT lymphoma were studied prospectively. METHODS: Patients were given 1,500,000 international units (IU) of interferon-alpha (IFN-alpha) (Roferon-A) subconjunctivally inside the lesion, three times a week for four weeks. If there was even a minimal response, a further cycle of 1,000,000 IU three times a week for four weeks was administered. Patients received a maintenance dose of 1 million IU, every 15 days for 4 times, after clinical resolution of the lesion. MAIN OUTCOME MEASURES: Patients were followed clinically, with slit lamp examination, for evidence of tumor disappearance or recurrence. RESULTS: Complete response was obtained in all patients. The lesion resolved completely by the eighth week. Four patients did not show any local recurrence with a median follow-up of 21 months (12-36 months). One patient presented with a recurrence after 11 months, in association with systemic lymphoma progression. CONCLUSIONS: Local chemotherapy with IFN-alpha seems to be an effective treatment modality, alternative to radiotherapy, for conjunctival MALT lymphomas.

Adult↗

Conjunctival lymphoid tumors: clinical analysis of 117 cases and relationship to systemic lymphoma.

OBJECTIVE: To investigate the clinical features of conjunctival lymphoid tumors and factors predictive of systemic lymphoma. DESIGN: Retrospective, observational case series. SETTING: Clinical practice of ocular oncology. PARTICIPANTS: The participants included 117 consecutive patients with lymphoid tumors of the conjunctiva treated at the Oncology Service of Wills Eye Hospital between 1974 and 1999. MAIN OUTCOME MEASURE: The main outcome measure was the development of systemic lymphoma. Cox proportional regression models were used to calculate the risk of eventual systemic lymphoma. Kaplan-Meier survival estimates were used to analyze the development of systemic lymphoma as a function of time. RESULTS: There were 55 males (47%) and 62 females (53%); 110 patients (94%) were white and 7 patients (6%) were African AMERICAN: The mean age at ocular presentation was 61 years, and the conjunctival lymphoid infiltrate was unilateral in 72 patients (62%) and bilateral in 45 patients (38%). In 8 patients (7%), initial unilateral conjunctival disease evolved into bilateral involvement over a mean of 32 months. Additional sites of ocular involvement were found in 27 patients (23%) and included lymphoid tumor in the eyelid in 3 cases, orbit in 18, choroid in 5, and vitreous in 1. Systemic lymphoma was known to exist before ocular diagnosis in 16 patients; (14%; for a mean of 51 months) and was found subsequent to ocular diagnosis in 20 patients (17; at a mean of 21 months). Therefore, of 117 patients with conjunctival lymphoid infiltration, 36 (31%) had or eventually developed systemic lymphoma, and 81 (69%) did not manifest systemic lymphoma during the mean follow-up of 38 months. Of the 64 patients with unilateral conjunctival involvement, 11 (17%) manifested systemic lymphoma, and of the 53 patients with bilateral involvement, 25 (47%) manifested systemic lymphoma. By univariate analysis, the clinical factors at date first seen predictive of the presence or development of systemic lymphoma included location of the tumor at an extralimbal site (fornix or midbulbar conjunctiva; P = 0.02) and increasing number of conjunctival tumors (P = 0.02). Using Kaplan-Meier life table analysis of those 101 patients who had conjunctival lymphoid tumor(s) and no evident systemic lymphoma at presentation, systemic lymphoma was eventually discovered in 7% of patients at 1 year, 12% at 2 years, 15% at 5 years, and 28% at 10 years. Overall, only one patient (<1%) died of systemic lymphoma, at 28 months after the diagnosis of the ocular disease. CONCLUSIONS: Lymphoid tumors of the conjunctiva are associated with systemic lymphoma in 31% of patients. Systemic lymphoma is found more often in those patients with forniceal or midbulbar conjunctival involvement and in those with multiple conjunctival tumors. Long-term systemic follow-up is advised, because related systemic lymphoma can manifest many years later.

Adult↗

Treatment of conjunctival and corneal intraepithelial neoplasia with topical interferon alpha-2b.

OBJECTIVE: To evaluate the role of topical interferon alfa-2b (IFNalpha2b) in the treatment of conjunctival and corneal intraepithelial neoplasia (CIN). DESIGN: Noncomparative case series. PARTICIPANTS: Five patients with histologically proven CIN or recurrences of proven CIN were studied prospectively. INTERVENTION: After histologic confirmation, patients were given topical recombinant IFNalpha2b (INTRON A, Schering Plough, Kenilworth, NJ) 1 million IU/ml four times a day. Patients were continued on interferon until complete resolution of the tumor had occurred. MAIN OUTCOME MEASURES: Patients were followed clinically and photographically for evidence of tumor resolution. RESULTS: All patients had complete resolution of the CIN lesion on IFNalpha2b. The mean time to clinical resolution was 11.6 weeks (range, 4-22 weeks). The mean follow-up was 17.6 months (range, 7-28 months). One patient had a clinical recurrence of his corneal CIN 1 year after tumor resolution. This patient was retreated, resulting in clinical resolution within 6 weeks, and has been tumor free for 8 months of follow-up.

Administration, Topical↗

Outcome of patients with periocular sebaceous gland carcinoma with and without conjunctival intraepithelial invasion.

PURPOSE: To evaluate the clinical findings, histopathologic features, methods of management, and outcomes in 25 patients with eyelid sebaceous gland carcinoma with and without conjunctival intraepithelial (pagetoid) invasion. PARTICIPANTS: Twenty-five patients with sebaceous gland carcinoma of the periocular region. DESIGN: Retrospective, observational case series. METHODS: The patients were divided into two groups: group A included those with sebaceous gland carcinoma with conjunctival intraepithelial (pagetoid) invasion, and group B included those with sebaceous gland carcinoma without conjunctival intraepithelial invasion. The demographic, clinical, and histopathologic features of sebaceous gland carcinoma were reviewed separately for each group. Ocular management results and systemic prognosis were reviewed. RESULTS: Of the 25 patients, 18 (72%) were females and 7 (28%) were males. The mean age at diagnosis was 65 years (range, 17-90 years). There were 11 patients (44%) in group A, and 14 (56%) in group B. The most common symptom in group A was ocular irritation (45%), and in group B the most common symptom was eyelid lump (86%). The most common sign in group A was diffuse eyelid thickening (72%), and in group B the most common sign was a distinct eyelid mass (43%). Six patients (55%) in group A, compared with one patient (7%) in group B, had both upper and lower eyelid involvement. The most frequent location of tumor epicenter was the outer third of the eyelid in both groups: 37% in group A and 57% in group B. Histopathologic origin of tumor was multicentric in four patients (36%) of group A and six patients (50%) of group B. Before referral, local recurrence had been found in 4 of 12 patients (33%) who underwent surgical treatment elsewhere. After referral, local recurrence was detected in 1 patient (9%) in group A and in 2 patients (14%) in group B. Orbital exenteration was necessary in 4 patients (36%) in group A and in one patient (7%) in group B. Tumor-related metastases occurred in two patients (18%) in group A and in two patients (14%) in group B. CONCLUSIONS: Sebaceous gland carcinoma with conjunctival intraepithelial (pagetoid) invasion has different clinical features compared with that without pagetoid invasion. Conjunctival intraepithelial (pagetoid) invasion carries a higher risk for orbital exenteration, but the risk of tumor-related metastases is similar in both groups.

Adolescent↗

Patterns of regional and distant metastasis in patients with conjunctival melanoma: experience at a cancer center over four decades.

OBJECTIVE: The regional lymph nodes generally are believed to be the most common first site of metastasis for conjunctival malignant melanoma, but the pattern of nodal metastasis in this disease has not been well established. The goal of this study was to determine the frequency, location, and timing of regional lymph node metastasis in patients with conjunctival melanoma treated at one cancer center over four decades. DESIGN: Retrospective case series. PARTICIPANTS: Twenty-seven patients. METHODS: The clinical records of 27 patients with conjunctival malignant melanoma were reviewed retrospectively. MAIN OUTCOME MEASURES: The rates of local conjunctival recurrence, regional nodal metastasis, and distant metastasis were analyzed along with overall survival. The follow-up time ranged from 2.5 to 17 years (median, 6 years). RESULTS: Eleven patients (41%) experienced clinical regional lymph node metastasis 1.5 to 6.0 years (mean, 3.2 years) after the initial diagnosis. The involved lymphatics were the preauricular (parotid) nodes in 8 patients (73%), the submandibular nodes in 1 patient (9%), and the deeper cervical nodes in 2 patients (18%). In seven patients (26%), distant metastasis developed without evidence of prior or concurrent regional nodal disease. Patients in whom distant metastasis developed without clinical evidence of regional nodal involvement were more likely to have had local conjunctival recurrence (P = 0.03) and a higher number of local recurrences (P = 0.05) compared with patients with regional lymph nodes as the site of first metastasis. The 5- and 10-year overall survival rates were 74% and 41%, respectively. CONCLUSIONS: Regional lymph node metastasis occurred in a higher percentage of patients with conjunctival malignant melanoma than has been reported previously. Preauricular lymph nodes were most commonly involved. Distant metastasis without prior or concurrent lymph node involvement was not a rare event.

Adult↗

Clinical spectrum of primary ophthalmic rhabdomyosarcoma.

PURPOSE: To review the clinical presentation, histopathologic analysis, management, and ocular and systemic outcome of rhabdomyosarcoma affecting the ocular region. DESIGN: Retrospective, noncomparative, consecutive, interventional case series. PARTICIPANTS: Thirty-three consecutive patients with primary ophthalmic involvement of rhabdomyosarcoma from a single tertiary care center specializing in ocular oncology. MAIN OUTCOME MEASURES: Final visual acuity, treatment complications, local recurrence, and distant metastasis. RESULTS: The mean age at presentation was 10 years (median, 7 years; range, 1 month-68 years). At presentation, 8 patients (24%) were older than age 10 years and 4 patients (12%) were older than 20 years. The rhabdomyosarcoma was primarily located in the orbit in 25 cases (76%), conjunctiva in 4 cases (12%), eyelid in 1 case (3%), and uveal tract in 3 cases (9%). Symptoms or signs related to the tumor were present for a mean of 5 weeks and included proptosis in 10 patients (30%), eyelid swelling in 7 patients (21%), and blepharoptosis in 6 patients (18%). The initial diagnosis before referral to us included rhabdomyosarcoma in 8 cases (24%), conjunctivitis in 5 cases (15%), orbital or preseptal cellulitis in 5 cases (15%), idiopathic orbital inflammatory pseudotumor in 4 cases (12%), and others. Using the Intergroup Rhabdomyosarcoma Study Group staging and treatment protocols, the tumor was classified as group I in 4 cases (12%), group II in 12 cases (36%), group III in 16 cases (48%), and group IV in 1 case (3%). Treatment included surgical debulking and various regimens of chemotherapy and radiotherapy for the periocular tumors and enucleation for the three intraocular tumors. Local tumor recurrence was detected in 6 patients (18%). Orbital exenteration was necessary for tumor recurrence in 2 cases (6%). Long-term visual outcome of the 28 patients who maintained their globe was 20/20 to 20/40 in 11 patients (39%), 20/50 to 20/100 in 5 patients (18%), and 20/200 to no light perception in 12 patients (43%). Regional lymph node metastasis was detected in 2 patients (6%), one at initial visit and one after therapy. Distant metastasis occurred in 2 patients (6%), one detected at initial visit and one after therapy. With mean follow-up of 8.3 years, tumor-related death occurred in 1 patient (3%). CONCLUSIONS: Rhabdomyosarcoma can present in the orbit, eyelid, conjunctiva, and uveal tract. After treatment, local tumor recurrence occurs in 18%, metastasis in 6%, and death in 3%.

Adolescent↗

Successful treatment with 5-fluorouracil of conjunctival intraepithelial neoplasia refractive to mitomycin-C.

PURPOSE: To describe the histopathological findings and successful treatment with 5-fluorouracil (5-FU) of a conjunctival intraepithelial neoplasia with limbal stem cell deficiency that was refractive to topical mitomycin-C (MMC). DESIGN: Interventional case report. INTERVENTION: A 64-year-old male patient presented with a diffuse conjunctival intraepithelial neoplasia (CIN) that was excised with concurrent keratoepithelioplasty. Because of a recurrence and the presence of limbal stem cell deficiency, he was placed on topical MMC. Despite two courses of MMC, the tumor size did not decrease, and topical 5-FU was started 1 year after MMC therapy began. Limbal autograft transplantation was performed thereafter. MAIN OUTCOME MEASURES: The clinical and histopathologic findings including impression cytology and biomicroscopic observations. RESULTS: After 5-FU treatment, the patient was free of the tumor clinically and cytologically, and the corneal surface had cleared. No recurrence was observed during the 30 months after the 5-FU therapy. Serious complications have not been observed. CONCLUSIONS: Topical 5-FU may be a therapeutic option for the treatment of patients with MMC-resistant CINs. The success of 5-FU is believed to be the result of a difference in the mechanism of cytotoxicity of MMC and 5-FU or the additive effects of the two agents.

Antibiotics, Antineoplastic↗

Topical mitomycin-C for partially excised conjunctival squamous cell carcinoma.

PURPOSE: To evaluate the efficacy of topical mitomycin-C (MMC) for treatment of postoperative residual conjunctival squamous cell carcinoma (SCC). DESIGN: Retrospective noncomparative case series. PARTICIPANTS: Five patients, two males and three females, with conjunctival and histologically proven incompletely excised conjunctival SCC. METHODS: Patients were treated with topical MMC. Two to three courses of topical MMC, 0.02% or 0.04%, were applied four times daily for 14 days per course. One month after the final treatment, the scar area with surrounding normal conjunctiva was excised, and histologic evaluation was done. MAIN OUTCOME MEASURES: No evidence of malignant cells in excised tissues. RESULTS: Histologic evaluation of the five specimens showed no malignant cells. Conjunctival scarring with inflammatory response was observed. No regrowth was reported during the follow-up period of 18 to 37 months. The complications of MMC use included mild to moderate conjunctival hyperemia in three patients. All signs and symptoms were resolved after discontinuation of the treatment. CONCLUSIONS: Application of topical MMC is an efficient treatment for residual conjunctival SCC. Longer follow-up is required to confirm these findings.

Administration, Topical↗

Human papillomavirus 16 and 18 expression in conjunctival intraepithelial neoplasia.

OBJECTIVE: To evaluate conjunctival intraepithelial neoplasia (CIN) and normal conjunctiva for the presence of human papillomavirus (HPV) DNA and for expression (as detected by the presence of mRNA) of the HPV E6 region. DESIGN: Prospective, case-controlled study. PARTICIPANTS: Ten consecutive patients who underwent CIN excision by one surgeon (CLK) and five age-matched control subjects who underwent retinal detachment repair at the Bascom Palmer Eye Institute. METHODS: A reverse transcriptase in situ polymerase chain reaction (PCR) technique was used to search for the presence of HPV mRNA in CIN specimens from 10 consecutive patients who underwent CIN excision by one surgeon (CLK) at Bascom Palmer Eye Institute, as well as in clinically uninvolved conjunctival specimens from the same eyes of these patients. In addition, conjunctival specimens from five control subjects (age-matched to five of the cases), who had no clinically identifiable conjunctival disease and who underwent retinal detachment repair at the Bascom Palmer Eye Institute, were analyzed in a similar manner. The clinical diagnoses of CIN and normal conjunctiva were confirmed histopathologically in all cases by an ocular pathologist, who was masked as to the patients' clinical diagnoses, and the PCR testing was performed by an investigator (GJN) who was masked as to the clinical diagnoses. RESULTS: HPV 16 DNA and mRNA were present in five CIN specimens, and HPV 18 DNA and mRNA were present in the other five CIN specimens; neither HPV 16 or 18 DNA nor mRNA were detected in any of the control specimens or in any of the clinically uninvolved conjunctival specimens (P < 0.001). In each of the CIN specimens, 20% to 40% of the dysplastic cells expressed the HPV E6 region. CONCLUSIONS: HPV 16 or 18 DNA and mRNA corresponding to the E6 region were detected in all CIN specimens examined. HPV 16 or 18 DNA or mRNA was not present in any of the control or uninvolved conjunctival specimens. The consistency of the current findings with those reported for human cervical malignant lesions, and the fact that the protein encoded by the E6 region of HPV 16 and 18 has been shown to form a complex with the protein encoded by the host tumor suppressor gene p53, provide strong evidence for an etiologic role of HPV in the development of CIN.

Carcinoma in Situ↗

Corneally displaced malignant conjunctival melanomas.

PURPOSE: To characterize and classify malignant conjunctival melanomas with exclusively corneal invasive growth. DESIGN: Population-based, nationwide retrospective cross-sectional study. PARTICIPANTS: Patients with primary malignant conjunctival melanoma diagnosed between 1967 and 2000 in Finland. METHODS: On the basis of all available clinical and histopathologic data of tumors diagnosed during the study period, malignant conjunctival melanomas that first demonstrated invasive growth on the cornea without evidence of conjunctival tumors other than primary acquired melanosis were identified, their prevalence calculated, and their characteristics reviewed. On the basis of these cases and literature data, a classification for "corneal melanoma" was developed. MAIN OUTCOME MEASURES: Frequency and type of corneal involvement, recurrence, and survival. RESULTS: Patients with exclusively corneal invasive tumor accounted for 5% (95% confidence interval, 1-12) of 85 consecutive primary conjunctival melanomas. Two were separated from the limbus by clear cornea (type I), and two paralleled but did not invade the limbal conjunctiva (type II). Two were associated with clear evidence of primary acquired melanosis. None of the tumors recurred after local excision, and no metastases were observed during a median follow-up of 2 years 5 months (range, 1 year 8 months-7 years 10 months). CONCLUSIONS: Primary malignant conjunctival melanomas can grow on the cornea without conjunctival involvement other than acquired melanosis. They are easily removed and do not cause lymphatic metastases. The term "corneally displaced malignant conjunctival melanoma" would best describe their supposed conjunctival origin and actual corneal location.

Aged↗

Topical mitomycin-C for pagetoid invasion of the conjunctiva by eyelid sebaceous gland carcinoma.

PURPOSE: To evaluate the efficacy of topical mitomycin-C for pagetoid invasion of the conjunctiva by sebaceous gland carcinoma. DESIGN: Prospective noncomparative consecutive interventional case series. INTERVENTION: All patients received topical 0.04% mitomycin-C four times daily for 1 week followed by 1 week off medication. The treatment cycles were repeated until resolution of the conjunctival malignancy was clinically evident. PARTICIPANTS: Four patients with histopathologically proven intraepithelial (pagetoid) invasion of the conjunctiva by sebaceous gland carcinoma were managed with this regimen. RESULTS: The mean patient age was 78 years. Before treatment, the main tumor site included the upper eyelid in two cases and the lower eyelid in two cases. Previous resection of the tumor had been performed elsewhere in three cases over the prior 6 years. At the time of our examination, map biopsies confirmed pagetoid invasion involving 25% to 90% of the conjunctival surface, with bulbar, forniceal, and tarsal conjunctival involvement in all four cases and corneal extension in one case. There was no evidence of deep tumor within the conjunctival stroma or tarsus in any case. After treatment, medication intolerance and early discontinuation occurred in one patient, and continued tumor progression was documented. Of the remaining three patients, chemotherapy was used for a mean of four cycles with complete resolution of the pagetoid invasion, confirmed histopathologically in two cases, and without recurrence in all three cases over 12 months (mean) follow-up. The medication caused moderate temporary local irritation but no serious intraocular or extraocular complications. CONCLUSIONS: Preliminary evidence suggests that topical mitomycin-C is effective treatment for pagetoid invasion of the conjunctiva by sebaceous gland carcinoma. Longer follow-up is necessary to assess the duration of tumor control.

Adenocarcinoma, Sebaceous↗

Ocular adnexal lymphoid proliferations: clinical, histologic, flow cytometric, and molecular analysis of forty-three cases.

PURPOSE: To describe the clinical features, histologic findings, flow cytometric immunophenotypes, and molecular profiles of ocular adnexal lymphoid proliferations. STUDY DESIGN: Prospective noncomparative case series. PARTICIPANTS: Forty-three patients suspected of having ocular adnexal lymphoid proliferations were biopsied and prospectively evaluated. METHODS: Provisional diagnoses were made on the basis of routine histology and immunohistochemistry for B and T cells. Results of flow cytometric immunophenotyping (FCI) and molecular assessment using polymerase chain reaction for immunoglobulin heavy chain (IgH) and TCR gamma chain gene rearrangement and bcl-2/IgH translocation were then incorporated into a final diagnosis. Demographic and clinical outcome data were collected. MAIN OUTCOME MEASURES: Final diagnosis based on histology, flow cytometry, and polymerase chain reaction. RESULTS: Forty-three cases were studied. Final diagnoses included 17 lymphomas, 18 chronic inflammations, 4 reactive lymphoid hyperplasias, and 4 atypical lymphoid infiltrates. Preliminary evaluation accurately categorized all 43 cases as either lymphoma or nonlymphoma. FCI permitted more precise subclassification of the lymphomas according to the Revised European American Lymphoma (REAL) system of nomenclature as follows: eight marginal zone B cell (mucosa-associated lymphoid tissue type), three mantle cell, two follicular, three large cell, and one lymphoplasmacytoid lymphoma. FCI showed a clonal B cell proliferation in 94% (16 of 17) of the lymphomas; FCI identified a clonal B cell population in 4% (1 of 25) of cases of nonlymphomas. Molecular evidence of clonality was identified in 88% (15 of 17) of lymphomas, 39% (7 of 18) of chronic inflammations, and 50% (4 of 8) of reactive lymphoid hyperplasias and atypical lymphoid infiltrates. CONCLUSIONS: The histologic diagnosis of ocular adnexal lymphoid lesions is highly accurate when determined by an experienced pathologist. FCI refines the histologic diagnosis and classification. Results of molecular studies should be interpreted in conjunction with clinical, histologic, and immunophenotyping findings.

Adolescent↗

Sebaceous gland adenoma of the tarsal conjunctiva in a patient with Muir-Torre syndrome.

PURPOSE: To highlight the recognition and diagnosis of Muir-Torre syndrome (MTS) in patients with sebaceous tumors of the eyelid/conjunctiva and to determine the role of immunohistochemical markers as a screening test in patients with Muir-Torre syndrome. DESIGN: Single interventional case report. METHODS: The clinical and family history was reviewed from the records of a 41-year-old man who had a sebaceous gland adenoma of the tarsal conjunctiva of the left upper eyelid. The lesion was completely excised and submitted for histopathologic examination. Immunohistochemical stains using an antibody to MSH2 were performed on the paraffin-embedded sections of the tumor. RESULTS: Histopathologic examination of the tumor showed a lobular pattern with basaloid cells at the periphery of the lobules with central areas of sebaceous differentiation. Immunohistochemical stains showed a lack of MSH2 expression in the tumor, which is highly consistent with MTS. CONCLUSIONS: Muir-Torre syndrome should be considered in patients initially seen with sebaceous tumors of the eyelid. Immunohistochemistry for MSH2 is a practical initial approach to screen for MTS in patients with sebaceous tumors.

Adenoma↗