Norman C. Charles

Neoplastic angioendotheliomatosis: a variant of malignant lymphoma immunohistochemical and ultrastructural observations of three cases

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.

Pilocytic Astrocytoma of the Optic Nerve with Hemorrhage and Extreme Cystic Degeneration

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.

Retinall and Optic Nerve Findings in Goldenhar-Gorlin Syndrome

Involvement of the posterior segment of the eye in Goldenhar-Gorlin syndrome is more common 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.

Epibulbar molluscum contagiosum in acquired immune deficiency syndrome. Case report and review of the literature.

BACKGROUND While molluscum contagiosum of the eyelid skin is commonly complicated by conjunctivitis, primary involvement of the conjunctiva or cornea by molluscum lesions is exceedingly rare. The authors studied a 34-year-old man with acquired immune deficiency syndrome (AIDS) in whom multiple molluscum lesions of the lids and a single nodule of the limbus developed. METHODS The nodular lesion was excised from the limbus and processed for histologic examination by light microscopy. Pertinent literature concerning epibulbar molluscum contagiosum was reviewed. RESULTS Histopathology of the excised lesion showed molluscum bodies within the acanthotic, hyperkeratotic conjunctival epithelium with surrounding chronic, nongranulomatous inflammation. Only 10 previous cases of conjunctival or corneal primary lesions have been reported, half of which showed associated cutaneous involvement. Lesions tended to be single, noninflamed, dome-shaped, and umbilicated, often with a yellowish central core. Patients were otherwise well and ranged in age from 3 to 55 years. Simple excision was effective in eradicating the lesions. CONCLUSION Primary epibulbar molluscum contagiosum is rare. Although cutaneous molluscum is common in AIDS, this report is the first to document conjunctival molluscum in a patient with AIDS.

Ganciclovir Intraocular Implant: A Clinicopathologic Study

BACKGROUND Surgical implantation of the intraocular sustained-release ganciclovir device is a safe and effective treatment for cytomegalovirus (CMV) retinitis. Previous histopathologic studies on eyes containing such implants have been limited by the necessity of removing the device before processing. Microtome sectioning of hard plastics within paraffin-embedded blocks is infeasible, and the anatomic relations of implant to eye are destroyed. METHODS The authors studied four eyes from three patients who had undergone implant insertion. Globes with implants in place were fixed in neutral 10% formation, embedded in methylmethacrylate, sectioned on a special microtome, and stained with hematoxylin-eosin. RESULTS After methacrylate embedding, the precise anatomic relations of the implant to the neighboring uveoscleral coats were preserved. In two eyes, the suture tab of the implant protruded through the sclera, exiting subconjunctivally. In two eyes, the implant was totally intravitreal. In all patients, the device was supported by fibrous tissue which emanated from a surgical coloboma of the pars plana ciliaris. Focal granulomatous inflammation adjoined suture and implant materials but no other inflammation or deleterious effects on the ocular structures were noted. CONCLUSION This report is the first to document the intraocular histopathology of the ganciclovir implant. The subconjunctival location, enhancing the potential for endophthalmitis, may be avoided by trimming of the suture tab close to the anchoring suture and not tying it too tightly. Methylmethacrylate embedding is a useful technique for preserving the microanatomy of intraocular implants.

Cryptococcosis of the anterior segment in acquired immune deficiency syndrome

Cryptococcosis is a rare form of human ocular infection, usually occurring in immunocompromised patients. Unilateral or bilateral posterior segment involvement is the rule. The authors describe a unique case of intraocular cryptococcosis presenting as an iris inflammatory mass in a patient with acquired immune deficiency syndrome. Diagnosis was established by anterior chamber paracentesis and confirmed by histopathologic examination of the enucleated eye.

Band-shaped and whorled microcystic dystrophy of the corneal epithelium.

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.

Carcinoma metastatic to the anterior ocular segment: Response to chemotherapy

Metastatic carcinoma involving the anterior ocular segment is an exceedingly rare complication of disseminated malignant disease. Non‐surgical treatment is ineffective and patients may require enucleation for relief of pain. We describe a response to chemotherapy in a patient having a metastasis from small cell carcinoma of the lung to the iris. By one month following commencement of therapy, complete gross tumor resolution had occurred.

Intramuscular lipoma of the eyelid.

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 slowly-growing 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.

Intraosseous hemangioma of the orbit.

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.