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      A Rare Case of Conjunctival Myxoma and a Review of the Literature

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          Background: To report on an 11-year-old boy with a painless slow-growing temporal bulbar conjunctival mass of his left eye. Methods: A case report and review of the literature. Results: An 11-year-old boy presented with a painless slow-growing mass in the temporal bulbar conjunctiva of his left eye, which had been noted for 1 year. After ophthalmic and systemic evaluations, the clinical differential diagnosis at the time included amelanotic naevus, amelanotic melanoma, myxoma, fibrous histiocytoma, reactive lymphoid hyperplasia and lipoma. This lesion was excised under local anaesthesia. From the histopathological features, conjunctival myxoma was confirmed. After 12 months, the patient remained healthy, with no recurrence or metastasis of the conjunctival lesion or evidence of a systemic abnormality. Conclusion: We report this case to emphasize that conjunctival myxoma can appear as a well-circumscribed, translucent, yellow-pink conjunctival mass in teenage patients. Successful healing is usually achieved by complete surgical resection. Cardiac, endocrine, and family screening is required to reduce the risk of morbidity and mortality.

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          Most cited references 9

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          The complex of myxomas, spotty pigmentation, and endocrine overactivity.

          Of 40 patients (16 males and 24 females), 29 had cardiac myxoma(s), 14 had skin pigmentation (lentigo and several types of nevi) which also commonly affected the lips, 6 had skin myxoma(s), and 12 had both pigmentation and myxoma(s); 18 had primary pigmented nodular adrenocortical disease (Cushing syndrome was present in 11); 10 had myxoid mammary fibroadenomas; 9 had testicular tumor(s) (large-cell calcifying Sertoli cell tumor, Leydig cell tumor, or adrenocortical rest tumor, or a combination); and 4 had pituitary adenoma with gigantism or acromegaly. The maximum number of conditions present together was five, occurring in two patients; each of the remaining patients had at least two of the conditions. The overlap, in this sizeable number of patients, of various combinations of the same rare or very rare conditions unlikely to occur together by chance with any degree of frequency is striking evidence for a unique syndrome. The patients were young (mean age at diagnosis of the first component, 18 years). Pathologic involvement tended to be multicentric (heart and skin) and bilateral in paired organs (adrenal, breast, and testis). Thirteen patients (32%) are alive and well. Twelve patients are alive but with complications of cardiac myxoma (in 8), testicular tumors (in 2), residual Cushing syndrome (in 1), or bilateral pulmonary nodules (in 1). Twelve patients are dead: 9 of cardiac myxoma, 1 of intracranial (nonpituitary) tumor, and 2 postoperatively. The status of three is unknown.
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            Clinical survey of 1643 melanocytic and nonmelanocytic conjunctival tumors.

            To report the spectrum and frequency of melanocytic and nonmelanocytic conjunctival tumors in an ocular oncology practice. Retrospective noninterventional case series. One thousand six hundred forty-three consecutive patients with a conjunctival mass evaluated at an ocular oncology department. A chart review was conducted to obtain the clinical features of the patient and tumor and to tabulate and categorize the diagnoses. Tumor diagnosis overall and relative to patient age, race, and gender and relative to tumor location and laterality. In 1643 consecutive patients, the tumor was classified as melanocytic in 872 cases (53%) and nonmelanocytic in 771 cases (47%). The nonmelanocytic categories included congenital choristomatous (n = 40 [2%]), epithelial (n = 219 [13%]), vascular (n = 63 [4%]), fibrous (n = 7 [ 60. Of the 219 patients with epithelial tumors, 80% occurred in males, whereas the incidence of melanocytic lesions was equal in males and females. African-American patients represented only 7% of epithelial tumors, <1% of melanomas, and 8% of lymphoid tumors. Conjunctival tumors were of melanocytic origin in 53% of cases and nonmelanocytic origin in 47%. Overall, melanocytic tumors, epithelial tumors, and lymphoid tumors accounted for 74% of all cases. These tumors were far more common in Caucasian patients, and epithelial tumors were found more frequently in men.
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              Conjunctival Lesions in Adults


                Author and article information

                S. Karger AG
                February 2008
                22 February 2008
                : 222
                : 2
                : 136-139
                Departments of aOphthalmology and bPathology, Tri-Service General Hospital, National Defense Medical Center, Taipei, Taiwan, ROC
                112632 Ophthalmologica 2008;222:136–139
                © 2008 S. Karger AG, Basel

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                Figures: 2, References: 26, Pages: 4
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