Pathologic findings in a steroid-responsive optic nerve infarct in giant-cell arteritis.

OBJECTIVE: To investigate the pathophysiologic mechanism of optic nerve infarction in giant-cell arteritis (GCA). BACKGROUND: Previous pathologic reports of optic nerve infarction in GCA involved patients who were blind at the time of death. The optic nerve infarcts were primarily retrolaminar in localization. Simultaneous short ciliary and ophthalmic artery vasculitis was found in all patients. METHODS: Clinical neurologic and ophthalmologic examination, temporal artery biopsy, and neuroimaging tests were performed in a patient with an anterior ischemic optic neuropathy secondary to GCA. Pathologic examination of the viscera, eye, and brain were performed at autopsy 1 month later. RESULTS: A prelaminar/retrolaminar infarct was found in this patient. Subsiding vasculitis was limited to the short ciliary arteries, sparing the central retinal, pial, and ophthalmic arteries. CONCLUSIONS: The authors believe that the visual improvement observed in this patient was the result of preserved, anterior optic nerve collateral circulation, as well as the neuroprotective and anti-inflammatory effect of the corticosteroids.

Primary orbital melanomas.

PURPOSE: Primary orbital melanomas are rare tumors with a poorly defined biologic course. Most recorded experiences concern single case reports. The authors evaluated the applicability of several of the histopathologic prognostic indicators used for uveal melanomas to a series of primary orbital melanomas with known clinical follow-up. METHODS: Twenty-one primary orbital melanomas, each with at least a 1-year follow-up after diagnosis, were evaluated for (1) modified Callender cell type, (2) mitotic count per 40 high-power fields, (3) lymphocyte count (less than versus greater than 100/20 high-power fields), (4) blue nevus component, and (5) largest tumor diameter. RESULTS: All patients for whom race was recorded were white. The mean age at diagnosis was 42 years (range, 15-84 years). There was an associated blue nevus in 19 patients (90 percent), and in 10 patients (47.5 percent) there was some form of congenital melanosis. With a mean follow-up period of 4.5 years (range, 1-13 years), mortality from metastatic tumor occurred in 8 (38 percent) of 21 patients. Of these eight patients, there were liver metastases in seven (88 percent) and brain metastases in one (12 percent). Indicators of poor prognosis were tumors of mixed cell type with high mitotic count and greater patient age with underlying congenital melanosis. CONCLUSION: Most primary orbital melanomas occur in white patients and are associated with blue nevi. These tumors are similar to uveal melanomas with respect to prognostic indicators and pattern of metastasis.

William Thomson and Joseph Janvier Woodward.

William Thomson and Joseph Janvier Woodward were two of several exceptionally versatile and highly productive young physicians who volunteered for service with the Union Army at the outbreak of the Civil War, and then were subsequently assigned to the Washington Area where they played significant roles and made major contributions towards the development of the Army Medical Museum. Both pioneered in photomicrography. While Thomson deserves priority, Woodward was the more prolific contributor whose work and publications helped draw attention to the Army Medical Museum as a center for excellence in pathology. After the War Thomson returned to Philadelphia where his interests in photography stimulated his pursuit of optics and eventually his becoming one of the first American physicians to specialize in ophthalmology. He became Professor of Ophthalmology at Jefferson Medical College.

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.

Rhabdomyosarcoma of the ciliary body.

Rhabdomyosarcoma is the most common malignant orbital tumor of childhood. It has twice been reported to arise within the globe from the iris. In addition, teratoid medulloepithelioma, a tumor arising from the ciliary epithelium, can contain a rhabdomyoblastic component, often in combination with other heteroplastic elements. The authors report what may be the first recorded case of an embryonal rhabdomyosarcoma of the ciliary body, possibly representing a one-sided differentiation of a malignant teratoid medulloepithelioma.

Retinoblastoma. The relationship of proliferating cells to blood vessels.

In 150 retinoblastomas the authors found a uniform thickness of the cuff of viable retinoblastoma cells that surrounds blood vessels. The mean thickness was 98.7 microns with a standard deviation of 11.9 microns. The cross-sectional area of the cuff was negatively correlated with the mitotic activity in the cuff and positively correlated with the diameter of the central vessel. The mitotic activity in the cuff of cells was inversely related to the distance from the central blood vessel. When the cuff was divided into three concentric rings, the inner ring contained a mean of 6.2 mitotic figures, the middle ring contained a mean of 2.9 mitotic figures, and the outer ring contained a mean of 0.6 mitotic figures. This pattern of growth is similar to that observed in other rapidly growing neoplasms in humans and experimental animals. In these tumors this pattern results from reduction in oxygen tension with increased distance from the central blood vessel.

Pyogenic granuloma of the palpebral conjunctiva associated with contact lens wear.

We report a pyogenic granuloma of the upper palpebral conjunctiva in a soft contact lens user. Pathological examination revealed numerous foreign bodies embedded within the specimen. The contact lens may have contributed to development of the lesion.

A unique epibulbar osseous choristoma.

The case of a 14-year-old girl found to have a unique epibulbar osseous choristoma attached to the sheath of the superior rectus muscle is recorded. The bony mass was palpable through the upper lid and by computerized tomography, it appeared densely calcified and noncystic. There was no ptosis or abnormality in ocular motility preoperatively or postoperatively.

Reticulohistiocytoma of the limbus and cornea. A clinicopathologic study of two cases.

Reticulohistiocytoma is a rare, benign histiocytic lesion usually occurring as an isolated skin nodule or as part of a systemic disorder known as "multicentric reticulohistiocytosis." The clinical and histopathologic findings of two women who presented with a single, painless mass localized to the cornea and limbus without skin lesions or systemic disease are reported. Histopathologically, the lesions were composed predominantly of large mononuclear and a few multinucleated cells with finely granular, "ground-glass" cytoplasm and large nuclei with prominent nucleoli. Immunohistochemical and electron microscopic studies conformed the histiocytic nature of these cells. Reticulohistiocytoma should be included in the differential diagnosis of epibulbar benign histiocytic lesions.

Pleomorphic adenocarcinoma of the ciliary body. Immunohistochemical and electron microscopic features.

An 80-year-old man was evaluated for an epibulbar tumor on a phthisical eye. The initial biopsy diagnosis of the epibulbar tumor was poorly differentiated neoplasm. Exenteration of the phthisical eye and orbital contents showed an extensive pleomorphic adenocarcinoma of the nonpigmented epithelium of the ciliary body with extraocular extension. There was evidence of hyaluronic acid secretion and immunohistochemical staining was strong for vimentin, focal for epithelial membrane antigen and S-100 protein, and weak for neuron-specific-enolase. Electron microscopy demonstrated desmosomes between tumor cells, areas of thick, multilaminar basement membrane production surrounding individual tumor cells, and occasional intracytoplasmic intermediate filaments.

Bilateral orbital involvement in fatal giant cell polymyositis.

The case of a young woman with giant cell polymyositis is described. She had bilateral, severe, midly painful proptosis and ophthalmoplegia. Extensive pharyngeal, laryngeal, and cardiac muscle involvement occurred 18 months later coincident with fatal cardiac arrhythmia. At autopsy, extensive muscle necrosis and giant cells were noted in extraocular, pharyngeal, laryngeal, and cardiac muscle with only minimal involvement of other striated muscles.

Metastatic melanoma within and to the conjunctiva.

Two patients with epibulbar juxtalimbal primary conjunctival melanomas experienced local intralymphatic metastases to the inferior cul-de-sac, and a hematogenous metastasis to the conjunctiva developed in five other patients with cutaneous melanomas. Whether reflective of a local or distant metastasis, all of the lesions histopathologically were located in the substantia propria, and were separated from the overlying epithelium by a thin mantle of collagen. There was no evidence of atypical intraepithelial melanocytic proliferation, as would be expected in association with a primary conjunctival melanoma. Two of the cutaneous metastases exhibited a binodular or multinodular appearance that correlated histopathologically with variably confluent micronodules suggestive of the origin of the clinical lesion from a shower of tumor cell emboli. Patients with local intralymphatic spread from a primary conjunctival melanoma may experience additional lesions in the conjunctival sac or eyelid skin and are at risk for regional or distant metastases. They should be examined closely several times a year. The patients with the distant metastases all had their previously diagnosed primary cutaneous tumors on the truncal skin (a similar tendency emerges from a review of previous ocular cases), typically had myriad other cutaneous lesions, and two of them had a neoplastic iridocyclitis and vitreitis. These patients tended to die of the disseminated tumors within 1 year after conjunctival metastases developed.

Unusual eyelid tumors with sebaceous differentiation in the Muir-Torre syndrome. Rapid clinical regrowth and frank squamous transformation after biopsy.

An unusual eyelid tumor with sebaceous differentiation developed in two 70-year-old men and each had an earlier history of one or more colonic carcinomas--the so-called Muir-Torre syndrome. These eyelid tumors were both behaviorally and histopathologically different from previously documented cutaneous neoplasms in this syndrome. In the first case, a sebaceous adenoma took multifocal origin from the epidermis and exhibited an interanastomosing retiform growth pattern in the underlying dermis; the lesion rapidly recurred as a keratoacanthoma after subtotal excision. In the second case, a conjunctival tumor with features of a poorly differentiated squamous cell carcinoma manifested focal evidence of sebaceous differentiation. Again, after an incisional biopsy, the latter tumor rapidly regrew and transformed into a well-differentiated squamous cell carcinoma with massive central necrosis. Although sebaceous carcinoma of the eyelids does not appear to presage the Muir-Torre syndrome, any benign sebaceous or transitional squamo-sebaceous neoplasm should be considered a possible manifestation of this syndrome. Both clear-cut benign and transitional sebaceous neoplasms should also be recognized as having the potential to undergo an ominous clinical regrowth upon subtotal excision and a complete squamous transformation.

Incidence of second neoplasms in patients with bilateral retinoblastoma.

The cumulative incidence of second neoplasms in 215 patients with bilateral retinoblastoma was calculated using the life-table method. Second tumors developed in 4.4% of the patients during the first 10 years of follow-up, in 18.3% after 20 years, and in 26.1% after 30 years. The 30-year cumulative incidence was 35.1% for the 137 patients who received radiation therapy compared with an incidence rate of 5.8% for the 78 patients who did not receive radiation. In the 137 patients who received radiation, second tumors developed both inside and outside the field of therapy. There was a 30-year incidence rate of second tumors of 29.3% within the field of irradiation and 8.1% outside the field. The rate outside the field of irradiation (8.1%) was similar to that observed in nonirradiated patients (5.8%). Our findings indicate that carriers of the retinoblastoma gene have an increased incidence of second tumors, and that the incidence rate is further increased in patients who receive radiation therapy.