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Background: The granular cell tumor (GCT) is a common, usually benign, neoplasm of uncertain histogenesis that may affect any organ in the body. It occurs rarely in ophthalmic sites, where orbital location is most frequent. The purpose of this report is to describe a unique case of an epibulbar, subconjunctival GCT in a child. Method: Histopathologic examination of the excised tumor included standard and immunohistochemical staining of formaldehyde-fixed, paraffin-embedded tissue. Results: Histopathologic study showed the characteristic morphology of GCT. Immunohistochemistry was strongly positive for vimentin, weakly and partly positive for neuron-specific enolase, and negative for S-100 and HAM-56. Conclusions: Granular cell tumor must be added to the differential diagnosis of epibulbar masses. Immunohistochemistry of the tumor in this case suggests an uncommitted mesenchymal cell origin.

A case of intraosseous orbital hemangioma is reported to alert surgeons to possible intraoperative hemorrhage during excision of such a lesion. A slowly enlarging mass was excised from the orbital rim of a 49-year-old woman. The clinical diagnosis was not suspected. In retrospect, roentgenographic findings included a focal honeycombed pattern of the zygomatic bone. Surgery was complicated by persistent low-volume bleeding. Histology showed endothelial-lined blood-filled channels within the bone. Intraosseous orbital hemangioma is a rare, benign neoplasm that can often be diagnosed clinically by characteristic roentgenographic findings. Observation should be considered as a therapeutic alternative when the radiographic diagnosis is established and when ocular function is not compromised.

Objective To report the clinical, histopathologic, and electron microscopic features of band-shaped and whorled microcystic corneal epithelial dystrophy. Design Two interventional case reports. Participants Two patients, two eyes. Intervention The involved area of corneal epithelium was scraped from each cornea. Results Histopathologic examination showed microscopic vacuoles in the epithelial cytoplasm in both cases. Electron microscopic examination revealed mainly empty cytoplasmic vacuoles with scant nonspecific osmophilic material. The process recurred clinically in one patient. Changes in corneal topography are documented in one patient. Conclusion Clinical findings and pathologic studies seem nearly identical to those in the original report. No pattern of systemic disorder or medication use was found. The cause of this condition remains unknown.

A patient noting a slowly enlarging bump at the orbital rim underwent surgical excision of the lesion. Pathologic examination showed a benign fibroma, a lesion that to the authors’ knowledge has never been previously reported in this location.

Hemangiopericytoma (HPCT) rarely originates in the lacrimal sac; 7 cases have been reported previously and only 1 contained an ultrastructural study. In this article we report an additional case and review the previous reports. While the initial biopsy specimen showed nonspecific cytologic abnormalities, light and electron microscopic studies on the subsequently excised tumor demonstrated that it had a structure characteristic of HPCT. The onset of lacrimal sac HPCT occurs in a younger age group than that of HPCT of other orbital locations. The tumor may recur locally but, to our knowledge, never has been reported to metastasize from a sac location. The treatment goal is complete surgical excision.

The authors report the clinical and ocular histopathologic findings in three patients with longstanding unilateral post-traumatic blindness. After one or more decades, acute pain associated with conjunctival hyperemia and apparent keratoprecipitates or a hypopyon developed in the affected eye of each individual. Phacoanaphylaxis was diagnosed preoperatively in two patients.Calcified granular lens fragments were dispersed throughout all three eyes. The anterior chamber in all patients contained extracellular calcified lens particles, but only one eye contained conspicuous macrophages. Two eyes showed elevated intraocular pressure (IOP), and in one patient calcified particles extended into a glaucomatous optic nerve head.To the authors' knowledge, this is the first report describing a rare condition involving the intraocular dispersal of calcified lens particles after disruption of the lens capsule. The authors have designated this entity as calcific phacolysis.

To report a unique case of intramuscular lipoma of the eyelid and to alert surgeons to this entity that simulates a common dermoid cyst. A slowlygrowing mass of the upper eyelid was excised from a 62-year-old man. Histology showed mature lipocytes interspersed with skeletal muscle. To our knowledge, this case represents the first report of intramuscular lipoma of the eyelid. Lipomas, including the intramuscular variety, are common tumors elsewhere in the body. The differential diagnosis of eyelid masses should include this entity. [Ophthalmic Surg Lasers 2000;31:340-341]

• We describe three patients with acquired immunodeficiency syndrome who presented with a bilateral coarse superficial epithelial keratitis due to infection with the protozoal parasite Microspora, Encephalitozoon cuniculi . Despite the extent of the corneal surface disease, conjunctival inflammation was minimal. Visual acuity ranged from 20/20 to 20/200. In one patient, the keratitis was complicated by the development of a surface defect with secondary Pseudomonas species infection. All patients had a history of exposure to household pets. Standard cultures were negative. Diagnosis was established in two of the three cases based on characteristic appearance of the protozoan in conjunctival scrapings. Electron microscopy of a conjunctival biopsy specimen in one patient confirmed the species. No recognized effective treatment is available for this infection.

Neurilemoma (schwannoma) of the conjunctiva is a rare ocular tumor. We report 3 cases of benign conjunctival neurilemoma occurring in women without other ocular or systemic disease. Two of these tumors arose from the bulbar conjunctiva and 1 from the tarsal conjunctiva. Immunoperoxidase staining for S-100 protein was positive in each case.

Calcific phacolysis has been previously described histopathologically in the enucleated eyes of three patients with unilateral posttraumatic total blindness. Each eye showed dissolution of a mature cataractous lens with dispersion of calcific lens material throughout the globe. We studied a patient in whom the signs of calcific phacolysis developed 19 months following intraoperative dislocation of a cataract into the vitreous. Partial removal of calcific particles was achieved by subtotal vitrectomy and lensectomy, with subsequent penetrating keratoplasty. Corneal histopathological findings showed a deposition of calcified lens particles between Descemet's membrane and retrocorneal fibrous membrane. Despite diffuse retinal calcification, the patient's visual acuity was 20/400. To our knowledge, this is the first report of calcific phacolysis occurring after surgical dislocation of a cataract into the vitreous and wherein treatment resulted in some preservation of vision. Early diagnosis and aggressive therapy may improve the visual prognosis in this condition.

Neoplastic angioendotheliomatosis (NAE) is a rare, fatal disease characterized by widespread intravascular proliferations of malignant cells of putative endothelial origin. Clinically, dermatologic and bizarre neurological manifestations predominate, but review of the reported cases of NAE reveals ophthalmic involvement to be frequent. To our knowledge, no reports of NAE have appeared in the ophthalmic literature. We describe three cases of NAE with the ocular manifestations of visual loss, cells in the vitreous, retinal artery occlusion, retinal vascular and pigment epithelial alterations, nystagmus, and cortical blindness. Autopsies (including eyes and central nervous system) revealed pancorporal involvement by intravascular anaplastic cells in each patient. In two patients massive extravascular involvement was also present. The tumor cells lacked ultrastructural features of endothelial cells and failed to stain for factor-VIII-related antigen. Common leukocyte antigen, a maker for hematopoietic cells, particularly lymphocytes, was detected on tumor cells in all cases, indicating that NAE is probably an extranodal lymphoma. The dramatic response of the central nervous system lesions to radiotherapy in one case supports this contention. It is suggested that this disorder be treated as a malignant lymphoma.

All eyelid lesions, because of their strategic proximity to the ocular and adnexal structures, should be considered as basal cell carcinomas until proved otherwise. The morphologic similarities of basal cell carcinomas to other common lid lesions are described. Following positive biopsy, successful therapy involves choice of an appropriate therapeutic modality for each individual patient and tumor.

Involvement of the posterior segment of the eye in Goldenhar-Gorlin syndrome is more cortimon than is generally appreciated. We examined seven patients with this syndrome. Abnormalities included diminished visual acuity, tilted optic disc, optic nerve hypoplasia, tortuous retinal vessels, macular hypoplasia and heterotopia, microphthalmia and anophthalmia. In one case, pathologic study showed agenesis of the optic nerve. It is proposed that retinal, optic nerve and craniofacial abnormalities in this condition may reflect an asynchrony in the migration of the neural crest cells in the early stages of embryonal development.

Juvenile pilocytic astrocytoma of the optic nerve (glioma of childhood) usually grows slowly. Rapidly evolving proptosis is rare and reflects the accumulation of mucinous or hemorrhagic material within the tumor. Rapid proptosis is more common in those patients beyond the first two decades of life who have long-standing tumors. We studied a 26-year-old woman with rapid proptosis caused by a large blood-filled cyst in a pilocytic astrocytoma of the optic nerve. The cyst was surgically removed along with the intraorbital portion of the optic nerve.

A 67-year-old woman, a retired writer, was ad­ mitted to the hospital on March IS, 1968. She stated that she had been in excellent health until the previous summer, when she noted an unusual lack of energy. In December, 1967, she experienced the abrupt onset of bitemporal aching pains which waxed and waned in severity and which radiated to her jaw. Examination revealed that both tem­ poral arteries were stony hard and very tender. An erythrocyte sedimentation rate (ESR) was 8.0/ mm/hour. In view of the history and physical ex­ amination, temporal arteritis was diagnosed. Be­ cause of personal fears, the patient refused to take corticosteroids, and aspirin, 10 gr every four hours, was prescribed. One week later she awakened in the morning and noted severe visual impairment in her left eye, pro­ gressing within a day to monocular blindness. Eye examination the following week revealed a visual

• The glomus tumor is a benign, vascular, hamartomatous derivative of the glomus body, a normal intradermal arteriovenous anastomosis. Most glomus tumors are solitary, tender lesions of the nail bed and are found only rarely on the eyelid. Occasionally, glomus tumors assume a multiple form, wherein they are nontender. I report a unique case of multiple glomus tumors involving the face, palate, eyelid, and anterior orbit.

• A 53-year-old black woman showed increasing epibulbar pigmentation following recurrent episcleritis at the same location. A biopsy was refused until vision was lost and a large black mass protruded from the globe and invaded the superficial cornea. Exenteration was performed and histological examination disclosed a malignant melanoma of the cornea with an adjacent intraepithelial conjunctival melanoma. We believe that the tumor arose in an area of acquired melanosis of the perilimbal conjunctiva and that secondary spread into adjacent superficial cornea occurred. Epibulbar malignant melanomas are uncommon conjunctival tumors in the general population and are especially rare in blacks.

• Two unrelated boys had a history of bilateral corneal clouding at birth following uncomplicated full-term gestations and spontaneous vaginal deliveries (without forceps). Clinical examinations disclosed bilateral corneal edema, no inflammation, and normal intraocular pressures. There was no history of similarly affected family members. The patients underwent penetrating keratoplasty at ages 4 months (patient 1) and 12 years (patient 2). Light and electron microscopic studies of the corneal buttons from both patients revealed areas of degeneration of the endothelium and separation of rounded endothelial cells. The morphologic features were strikingly similar to those in two acquired forms of corneal disorders—autoimmune endotheliopathy and "acute endotheliitis." Immunocytologic and in situ hybridization studies for herpes simplex virus were not consistent with either productive or latent corneal infection. Ultrastructural changes in Descemet's membrane reflect delayed or abnormal development of the postnatal nonbanded layer in patients 1 and 2, respectively. These suggest an intrauterine insult that resulted in endothelial dysfunction. The histologic and ultrastructural features of these two congenital cases are not typical of those seen in any of the recognized causes of congenital corneal clouding. We propose that these cases represent a unique congenital corneal endotheliopathy of undetermined origin.