Retinoblastoma and intracranial malignancy.

The occurrence of independent brain tumors in two patients with retinoblastoma is described. One patient with well-differentiated biliteral retinoblastomas developed, over two years later, a pineal tumor but no other metastatic lesions. The pineal tumor was composed of small neuroblastic cells and a second population of larger cells with vesicular nuclei and more abundant cytoplasm.

Granulomatous dacryoadenitis caused by Schistosoma haematobium.

An 11-year-old boy from Sierra Leone developed a mass in the left lacrimal gland a year after trauma to the left side of the brow. Biopsy of the mass led to the diagnosis of schistosomiasis caused by Schistosoma haematobium, which had not been suspected previously. The initial occurrence in the orbit of such a rare, ectopic lesion in schistosomiasis and the possible relationship to previous trauma are features of special interest.

Angiomatosis retinae. An ultrastructural study and lipid analysis.

A nonfamilial case of agiomatosis retinae (retinal hemangioblastoma) was studied by electron microscopy. In addition to the three major types of cells previously identified within the tumor (endothelial cells, pericytes, heavily lipidized stromal cells), fibrous astrocytes in different stages of lipidization were also found. The endothelial cells were fenestrated, providing the basis for the extravasated exudate that is characteristic of the tumor.

Ophthalmic striated muscle neoplasms.

Rhabdomyosarcoma, the most common primary malignant childhood orbital tumor, is composed of neoplastic striated muscle cells (rhabdomyoblasts) in various stages of differentiation and in patterns suggestive of neoplastic analogs of normal muscle embryogenesis. Orbital rhabdomyosarcoma is most commonly seen in children and adolescents, the average age of onset of symptoms being 7.8 years.

Granulomatous necrotizing retinochoroiditis caused by Sporotrichum schenkii. Report of a case including immunofluorescence and electron microscopical studies.

A middle-aged man had blurred vision, redness, and pain in the right eye. Ophthalmoscopic examination revealed slowly progressive necrotizing retinitis in the peripheral superonasal quadrant. The clinical impression was toxoplasmic retinochoroiditis, but lesions failed to respond to steroids, pyrimethamine, and sulfonamides.

Choroidal leiomyoma of vascular origin.

A 57-year-old man had a clinically suspected malignant melanoma in his left eye. On microscopic study, the enucleated eye harbored an unusual choroidal tumor that had extended extraocularly. This tumor had been variously interpreted microscopically as an angiosarcoma, an atpical angioma, a glioma, or a neurilemoma.

Tumors of the optic nerve.

This is a review of primary and secondary tumors of the optic nerve. The emphasis is an optic nerve gliomas and meningiomas. Optic nerve gliomas are slowly growing astrocytic neoplasms of the anterior visual pathways, the majority of which occur within the first two decades of life, with equal sex incidence in about 1 of 200,000 patients presenting with eye complaints.

Leiomyoma and leiomyosarcoma of the orbit.

Comparison of the clinical and pathologic features of two orbital leiomyomas, two leiomysarcomas, and one embryonal rhabdomyosarcoma showed the leiomyomas occurred in young individuals and the leiomyosarcomas in older patients. The histopathologic diagnosis rested on the intense cytoplasmic eosinophilia and nostriated longitudinal cytoplasmic filaments demonstrated by means of the trichrome stain. The leiomyosarcomas disseminated 15 months and seven years after their orbital presentations.

Rhabdomyoma of the orbit.

To our knowledge, this is the first fully documented case of an adult-type orbital rhabdomyoma in an 8-year-old white boy, which originated in the belly of the medial rectus muscle and grew into the anterior orbit to incorporate the trochlea. Incomplete excision resulted in recurrence; the lesion was misinterpreted as a rhabdomyosarcoma and exenteration was performed. The patient was free of disease 25 years later.

Sector hamartoma of the iris.

A 15-year old girl with a jet black-pigmented lesion on the anterior surface of the upper nasal quadrant of her iris was followed up for 12 years, during which interval growth of the lesion was documented. Histopathological evaluation of the excised segment of the iris revealed a pigment epithelial proliferation originating from posterior pigment epithelium. Additionally, there was a band of spindle shaped smooth muscle cells in the posterior stroma and a zone of plump melanocytes in the anterior stroma beneath the proliferating pigment epithelial cells.

Amyloidosis of the orbit and adnexae.

Five patients representing a spectrum of orbital and adnexal amyloidosis are presented. One patient with conjunctival amyloidosis did not have antecedent infection nor systemic disease (primary localized amyloidosis), while another patient with conjunctival amyloidosis had a long history of repeated attacks of bacterial conjunctivitis (secondary localized amyloidosis). The third patient presented with serial bilateral lacrimal gland amyloid without systemic disease (primary localized orbital amyloidosis).

Kaposi's sarcoma of the conjunctiva.

A 75-year-old man with known Kaposi's sarcoma of three-years' duration developed chronic hemorrhagic thickening of the conjunctiva. Biopsy demonstrated histopathologically that the conjunctiva thickening represented sarcoma involvement. Although surgically untenable, the conjunctival lesion responds satisfactorily to radiotherapy.

Electron microscopic diagnosis of medulloepithelioma.

A 20-month-old boy with a ciliary body tumor presented with two white flocculi floating in the anterior chamber of his left eye. This material was examined by electron microscopy. Both the clinical appearance of the tumor and the ultrastructural findings suggested the diagnosis of medulloepithelioma.