Painful ophthalmoplegia caused by neurotropic malignant melanoma.

Progressive cranial nerve palsies and painful ophthalmoplegia developed in a 76-year-old man with lentigo maligna of the forehead 18 months after the excision of a spindle cell tumour of the forehead that proved on immunohistochemical study to be a malignant melanoma. The signs and symptoms were caused by neurotropic spread of the malignant melanoma into the cavernous sinus and subsequent neurosarcomatous transformation of the orbital metastasis.

Ghost-cell tumor of the optic chiasm. Primary CNS lymphoma.

A case of steroid-responsive malignant lymphoma of the optic chiasm is reported in a 58-year-old woman presenting with subacute vision loss, headaches, and decreased hearing. The exquisite sensitivity of magnetic resonance imaging (MRI) of the parasellar area and the usefulness of MR-directed stereotactic biopsy in identifying a chiasmatic lesion are shown. Primary CNS lymphomas are briefly reviewed. Ophthalmologists will be asked to evaluate patients with malignant lymphoma of the optic chiasm with increased frequency in the future, as CNS lymphomas are expected to be the most common neurological neoplasm by 1991.

"Empty sella" with invasive microprolactinoma.

We describe the unique association of an aggressive prolactin secreting pituitary microadenoma invading through the sphenoid sinus with an empty sella, in which the pituitary tumor remained clinically undiagnosed. The postmortem anatomy of this empty sella syndrome is presented. It is concluded that the phrase "empty sella", although universally accepted, correlates poorly with the underlying disease, and that terms such as "intrasellar arachnoidocele" or "intrasellar subarachnoid herniation" would be more properly descriptive.

Immunohistochemistry of capillary hemangioblastoma. Immunoperoxidase-labeled antibody staining resolves the differential diagnosis with metastatic renal cell carcinoma, but does not explain the histogenesis of the capillary hemangioblastoma.

We used a battery of antigens to determine whether immunohistochemistry can (a) contribute to resolving the histogenesis of the stromal component of the capillary hemangioblastoma, and (b) answer cases of difficult pathologic differential diagnosis with metastatic clear cell carcinoma. The stromal cells of the capillary hemangioblastoma are antigenically polymorphous and may express immunoreactive erythropoietin, renin, keratin, Leu M1, Leu 7, actin, neuron-specific enolase, S100 protein, and glial fibrillary acidic protein. However, the use of epithelial membrane antigen allows certain histopathologic distinction between capillary hemangioblastoma and metastatic clear cell carcinoma.

Malignant glioma of the optic chiasm eight years after radiotherapy for prolactinoma.

A 41-year-old man had rapidly progressive visual loss caused by a malignant glioma that developed in the optic chiasm eight years after radiation therapy for a recurrent prolactinoma. Radiation-induced glioma should be considered as a cause of progressive visual loss in patients who have received irradiation in the region of the sella turcica.

Hemangiopericytoma of the ciliary body.

A 60-year-old woman had a ciliary body tumor that resembled an amelanotic malignant melanoma by clinical examination. The magnetic resonance imaging scan correlated with the clinical diagnosis. Because of apparent rapid growth, her eye was enucleated. However, histopathologic examination revealed a highly vascularized, spindle-cell tumor harboring immunoreactive actin and vimentin. Electron microscopy further supported the diagnosis of hemangiopericytoma with smooth-muscle differentiation. To our knowledge, this is the first report of a hemangiopericytoma of the ciliary body.

[Microangioma and optochiasmatic apoplexy. Description of an anatomo-clinical entity associating spontaneous hemorrhages of the anterior optic pathways and rupture of cryptic vascular anomalies]. / Microangiomes et apoplexie opto-chiasmatique. Description d'une entité anatomo-clinique associant hémorragies spontanées des voies optiques antérieures et rupture d'anomalies vasculaires cryptiques.

A 39 year-old women presented for the third time in 9 years, with an acute chiasmal syndrome caused by hemorrhage within a ruptured intra-chiasmatic cryptic angioma. We have reviewed this entity described as "chiasmal apoplexy" and defined its clinicopathological characteristics. Sub-frontal microsurgical approach to the optic chiasm is recommended to confirm the diagnosis and to decompress the optic chiasm. However, surgery does not prevent the possibility of recurrences. We have precised the concept of cryptic vascular malformation.

Ciliary body neurilemoma recurring after 15 years.

A 57-year-old white female presented with a recurring neurilemoma (benign solitary schwannoma) of the ciliary body 15 years after the primary modified iridocyclectomy. As neurilemoma are encapsulated, successful "tumorectomy" was twice accomplished with total anatomical and functional preservation of the globe. The pathological diagnosis of spindle cell tumors of the uvea is discussed.

Desmoplastic fibroma of parietal bone simulating eosinophilic granuloma. Case report.

Desmoplastic fibroma is a distinctive and rare neoplasm of bone. Only one previous example has been reported in the calvaria. The diagnostic and surgical aspects of a case of desmoplastic fibroma of the skull that radiographically simulated eosinophilic granuloma are reported.

Immunohistochemical and ultrastructural studies on the exenterated orbital tissues of a patient with Graves' disease.

The exenterated orbital contents obtained post mortem from a 47-year-old man who had an eight-month history of treated hyperthyroidism and a two-month history of visual complaints were examined histopathologically, immunohistochemically, and by electron microscopy. All of the extraocular muscles were massively enlarged, due to early diffuse endomysial fibrosis, mucopolysaccharide deposition, and a predominantly perivascular lymphocytic and plasmacytic infiltration. Histochemical stains revealed that the intramuscular mucopolysaccharides were weakly sulfated and polycarboxylated, establishing that they were products of fibroblastic activation rather than derived from mast cells. Subsarcolemmal deposits in the myofibers were shown by electron microscopy to be collections of glycogen rosettes with intermixed lipid deposits. These inclusions were interpreted as secondary phenomena due to abnormal muscle energetics from restrictions in contractility, and not as evidence of a primary degeneration of the extraocular muscle fibers themselves. The sarcomeric organization of the striated muscle cells was undisturbed, and therefore the pathologic changes occurred int he interstitial space of the endomysium. A monoclonal antibody specific for neurofilaments (antibody 4.3 F9) revealed, in comparison with normal controls, a preferential loss of large-type axons of the proximal segment of the orbital portion of the optic nerve as well as in the intracanalicular portion. The axonal loss was associated with a mildly increased number of astrocytes as demonstrated by an antibody against glial fibrillary acidic protein. The meninges of the optic nerve, the orbital fat, and the tendons of the extraocular muscles were uninflamed. The foregoing findings are compatible with a compressive optic neuropathy mediated by the massively swollen extraocular muscles impinging upon the optic nerve at the orbital apex.