Peripapillary choroidal melanomas. Extension along the optic nerve and its sheaths.

We reviewed 26 cases of peripapillary melanoma, 21 (80.8%) of which extended into the optic nerve or its meningeal sheaths. Most melanomas that extended into the optic nerve were necrotic and of the mixed or epithelioid cell type; all invaded the overlying retina and occurred in blind eyes with secondary glaucoma. The extension of melanoma cells into the subarachnoidal space was only influenced by the presence of the melanoma around the optic nerve head (peripapillary). The prognosis of these patients was highly dependent on the presence of orbital extension. A long piece of optic nerve should be excised when eyes with peripapillary melanomas are enucleated.

Orbital extension of Choroidal and ciliary body melanomas.

In a review of 432 choroidal and ciliary body melanomas, histopathologic evidence of orbital extension was found in 45 cases (10.4 percent). The occurrence of orbital extension was influenced mainly by the cell type (P less than .05) and by the size of the choroidal mass (P less than .001). Local orbital recurrence occurred in 23 percent of cases with evidence of extrascleral extension. Survival rates were independent of cell type, pigmentation, and size and shape of the extension. Patients treated by exenteration had significantly better prognosis than nontreated patients or patients treated by chemotherapy, radiotherapy, or local excision (P equals .0002). Early exentration is the best treatment for choroidal melanomas with orbital extension.

Prognostic factors in choroidal and ciliary body melanomas.

We reviewed 293 cases of choroidal and ciliary body melanoma with a followup period of five years or more. Nine factors influenced significantly the prognosis: Age of the patient at the time of enucleation, location of the tumor and of its anterior border, largest tumor diameter in contact with the sclera, height of the tumor, integrity of the Bruch membrane, cell type, pigmentation, and scleral infiltration by tumor cells. Some of these factors can be evaluated clinically before the enucleation. Seven sets of three factors each were determined in order to have better prognostic values based on the clinical data and the cell type. The largest tumor diameter is the single most important clinical and pathological prognostic factor. The prognosis is relatively good when this diameter is 10 mm or less and becomes poor when it exceeds 10mm.

From Vienna to Iowa: an ophthalmologic odyssey.

In a warm and personal memoir, a world renowned ophthalmologist recounts experiences from his medical school days in pre-war Austria, through the tense and chaotic years of World War II, and the years of learning, practicing, and teaching in New York and Iowa after his immigration to the U.S.A. Special attention is given to the friends and colleagues who shaped and enhanced the author's career.

Ocular and orbital manifestations of neurofibromatosis.

The ocular and systemic manifestations of von Recklinghausen's neurofibromatosis are reviewed. A case is presented illustrating widespread involvement and the typical histopathologic changes of the eye and the ocular adnexa. The ophthalmologist should be aware of broad scope of this disorder so that a complete evaluation of the patient can be made.

Pathology of orbital bones. The XXXII Edward Jackson Memorial Lecture.

The orbital bones may show nearly all the pathologic changes observed in the skull and in the face. The congenital anomalies in this area are numerous and involve various forms of craniostenoses. Among the benign osseous tumors the osteoma is most frequently encountered in the orbit. Fibrous dysplasia is a tumefaction of indeterminate behavior that often involves the orbit. Osteosarcoma or other malignant neoplasms are rarely seen in this area. Eosinophilic granuloma and Hand-Schüller-Christian disease are tumor-like lesions that may involve the orbit.

Ocular melanocytosis and melanoma.

The hypothesis that ocular melanocytosis is a precancerous condition that may lead to a choroidal melanoma should be seriously questioned for the following reasons: The incidence of malignant degeneration in a hyperpigmented eye is unknown and overreported. If the hypothesis were correct a bilateral melanoma would occasionally occur in patients with bilateral melanosis. No such case has been reported. In patients with unilateral melanosis the blue, unaffected eye may also develop a melanoma. A 67-year-old white woman with one dark and one blue eye provided the first such instance, although previous cases may not have been reported if their histologic picture was not unusual. The incidence of melanoma in our patients with unilateral melanosis was rare compared with the many melanomas developing in normal pigmented eyes (4/418). I found no statistically significant difference in the incidence of choroidal melanomas originating in the hyperpigmented or in the blue eye in patients with unilateral ocular melanocytosis.

Localized conjunctival amyloidosis.

We treated a patient with localized amyloidosis of the bulbar conjunctiva, which strongly resembled a neoplastic growth. Although the patient suffered from a long-standing, debilitating rheumatoid arthritis, suggesting the possibility of a secondary systemic amyloidosis caused by the chronic effusive process, no specific clinical evidence of systemic amyloidosis was noted. The ocular lesion was, therefore, classified as a primary localized amyloidosis. However, the simple recognition of amyloid in the ocular adnexa should not be considered a complete diagnosis. For such cases in which a prominent underlying systemic disease is present, a thorough medical evaluation and future follow-up should be ensured to rule out the possibility that this represents the initial manifestation of a potentially life-threatening secondary systemic amyloidosis caused by rheumatoid arthritis.

Congenital generalized multicentric myofibromatosis with orbital involvement.

We studied a rare case of congenital myofibromatosis of the orbit as part of a generalized multicentric disease in a 5-month-old boy. The ultrasonographic and computed tomographic findings were those of a low-reflective, firm, vascularized, lesion with heterogeneous internal structure. The histopathologic findings showed vascular channels with large polygonal cells and transition toward myofibroblasts with interwoven collagen fiber bundles.

Standardized echographic-histopathologic correlations in liposarcoma.

A 57-year-old woman had metastatic liposarcoma of the left orbit that underwent transformation. The primary focus in the abdomen, resected in 1975, was a well-differentiated liposarcoma. Later metastases to the neck and orbit were poorly differentiated, pleomorphic, and highly anaplastic liposarcomas. The ultrasonographic and histopathologic correlations of the different stages of the tumor during the disease process were analyzed and compared. This patient's disease had echopathologic similarities to other orbital sarcomas and adult cavernous hemangiomas.

Localization of glial fibrillary acidic protein in retinal astrocytoma: an immunohistochemical study.

Glial fibrillary acidic protein (GFA) is found in glial filaments, and is specific for reactive or neoplastic astrocytes and ependyma. Using a commercially available antibody to GFA protein, a retinal neoplasm from a patient with neurofibromatosis is intensely stained with the brown immunoperoxidase reaction product. Rather than the less specific phosphotungstic acid hematoxylin (PTAH) or Holzer's stain, antibody to GFAP protein is a relatively rapid, simple, and reliable immunohistochemical technique for formalin-fixed and paraffin-embedded tissue.

Prognosis in retinoblastoma: influence of histopathologic characteristics.

One hundred twenty-eight cases of retinoblastoma were reviewed. The mean age at diagnosis, presenting signs, clinical, and pathological features were investigated. Patients with bilateral tumors presented earlier and had a higher mortality rate than those with unilateral tumors. Histopathological features such as calcification, seeding into the vitreous, and the degree of differentiation had no significant effect on prognosis. There was no predominance of exophytic, endophytic, or mixed tumor growth patterns. Extension of the tumor to extraretinal structures increased the mortality rate significantly.

The Tenth Frederick H. Verhoeff lecture. What else did 1864 contribute to ophthalmology?

In summary we can say that the AOS was founded at a time when ophthalmology established itself as an independent scientific medical specialty. A hundred years earlier, in 1750, ophthalmology became an independent surgical specialty when Jacques Daviel of Marseille had begun extracting a cataract instead of merely couching or dislocating the lens. Now in the middle of the 19th century a new era dawned on the ophthalmic horizon. An era which Julius Hirschberg calls "the reform of ophthalmology." It was effected mainly by a group of unusual, gifted and genial scholars. Hermann v. Helmholtz, who not only invented the ophthalmoscope, but established with his handbook physiologic optics as an advanced, sophisticated branch of optics and mathematics; F.C. Donders, who put refraction, refractive errors and accommodation on a sound scientific footing, the great A. v. Graefe, who contributed so much to the concept and treatment of glaucoma, to strabismus, to various diseases of the fundus, to neuro-ophthalmology and to many other fields and finally William Bowman, the great investigator, clinician and surgeon. It was during this time of reform, of fermentation, of maturation, that a group of farsighted American ophthalmologists decided to establish a society to further the aims and objectives of our specialty in America. The time was right; the effort succeeded and our society developed into one of the decisive forces of American ophthalmology. I hope that my address has met the objectives which I had outlined earlier: To present and illuminate the circumstances and external conditions which were effective in 1864 when our society was founded. At the same time I hope I have done justice to the memory of this outstanding American ophthalmologist, Frederick Verhoeff, who contributed so much to the American Ophthalmological Society. On this the quasquicentennial jubilee of the AOS we find the Society healthy and flourishing. May it continue as an association of the most prominent and most promising American ophthalmologists who consider the practice of our specialty a scholarly profession and not a mercenary trade. I can only conclude with wishing the AOS a happy birthday, many successful returns, ad multos annos!