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Biomedical subjects

R L Font

Publications and source records attributed to R L Font.

At least 199 records · Page 11Linked to original sources

Oncocytic lesions of the caruncle and other ocular adnexa.

This is a clinicopathologic study of 18 oncocytic lesions involving the ocular adnexa. These lesions most commonly occur in elderly patients (median age 73), and there is a striking predilection for women. The broad spectrum of lesions included oncocytic adenomatous hyperplasia, oncocytomas, and oncocytic carcinoma. Ten lesions, all of which were oncocytomas, involved the caruncle, four the conjunctiva, and one the mucocutaneous junction of the eyelid. Two tumors involved the lacrimal sac and one the lacrimal gland. A review of the literature disclosed only 17 previously reported oncocytic lesions of the ocular adnexa. A small yellowish-tan or reddish caruncular mass, especially in an elderly woman, should alert the ophthalmologist to consider the possibility of oncocytoma in his differential diagnosis.

Adenoma↗

Orbital involvement in 'sinus' histiocytosis. A report of four cases.

Sinus histiocytosis is a newly recognized benign disease affecting mainly children and young adults and usually having a protracted clinical course that is relatively unaffected by therapy. This paper describes four additional patients who had orbital involvement initially and reviews the salient clinical and histopathologic features of this entity. The outstanding clinical feature is cervical lymphadenopathy. Associated findings include low-grade fever, anemia, leukocytosis, and elevated IgG levels. A small percentage of patients develop proptosis with palpable orbital tumors. Such patients may not have appreciable lymphadenopathy. Progressive proptosis may lead to exposure keratitis, corneal ulceration, endophthalmitis, and loss of the eye. Histopathologically, the lymph nodes and orbital mass show a proliferation of large histiocytes intermixed with a variable proportion of lymphocytes and plasma cells. Lymphocytes and occasionally other cells derived from the hematopoietic system are commonly seen within the cytoplasm of the histiocytes.

Child↗

Mesectodermal leiomyoma of the ciliary body: a tumor of presumed neural crest origin.

Two benign tumors of the ciliary body diagnosed as neurogenic tumors by light microscopy were shown by electron microscopy to be composed of smooth muscle cells with unusual morphologic features. The light-microscopic appearance of a background fibrillary matrix imparting a "neural" appearance was the result of the interweaving of myriad cell processes filled with thin cytoplasmic filaments possessing fusiform densities. The perikaryon of the tumor cells was relatively free of filaments and displayed mitochondria and stacks of rough-surfaced endoplasmic reticulum suggestive of Nissl substance. The basis for these remarkable "hybrid" tumor cells--combining myogenic and neurogenic characteristics--is believed to reside in the neural crest origin of the smooth muscle of the ciliary body (mesectoderm). The histogenetic implications of mesectodermal tissue and the tumors that it spawns are discussed.

Adult↗

Unusual superficial variant of granular dystrophy of the cornea.

Two patients with a superficial variant of granular dystrophy of the cornea occurring primarily in nongrafted eyes developed rapid progression of clinical manifestations and visual deterioration at an early age. No other members of their family were known to be affected. A clinical diagnosis of superficial corneal dystrophy, type undetermined, was made. The histopathologic features of the corneas resembled the findings seen in Reis-Bücklers dystrophy and led to some confusion in the differential diagnosis. Electron microscopic studies in both cases unequivocally established the diagnosis of granular dystrophy.

Adolescent↗

Clinical and histopathologic observations in severe Vogt-Koyanagi-Harada syndrome.

Vogt-Koyanagi (V-K) syndrome is a severe anterior uveitis associated with alopecia, vitiligo, poliosis, and dysacousia. Harada's disease (HD) is primarily a posterior uveitis accompanied by signs of meningeal irritation and abnormalities of the cerebrospinal fluid. The overlapping of clinical manifestations between the two justified considering them as part of a spectrum of one disease (V-K-H). In a clinicopathologic study of nine cases four patients (three with V-K syndrome and one with V-K-H syndrome) histopathologically displayed a granulomatous uveitis. Five patients (two with V-K syndrome and three with HD) showed a nongranulomatous uveitis. We emphasized this latter finding, since in the past the diagnosis of this syndrome has been discarded both clinically and histopathologically because of the absence of a granulomatous uveitis. We established the clinical and histopathologic differences between V-K-H and sympathetic ophthalmia. The designation "uveomeningoencephalitic syndrome" stresses the key features of V-K-H, namely the uveal involvement and signs of meningeal irritation (that is, headaches, personality changes, and cerebrospinal fluid alterations).

Adult↗

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. The eye was enucleated and, histopathologically, showed necrotizing granulomatous retinochoroiditis and optic neuritis, numerous cigarshaped, yeast-like organisms located within the necrotic retina and subretinally, and a subretinal asteroid body. Organisms were identified as Sporotrichum schenkii by immunofluorescence techniques. Electron microscopical studies of the fungus disclosed an unusually thickened capsule with a well-developed cell wall, the outer portion of which exhibited a radiating pattern of granular filamentous material. The ability of S schenkii to cause endophthalmitis in a patient without apparent primary infection should be remembered in the differential diagnosis of a cryptogenic, slowly progressive intraocular infection.

Adult↗

Sebaceous gland carcinoma of the eyelid.

A 37-year old woman had received multiple radiation treatments between the ages of 6 months and 15 years for an extensive cavernous hemangioma of the left side of the face. At the age of 27 years, a biopsy specimen from a lesion of the left buccal commissure disclosed squamous cell carcinoma arising in chronic radiodermatitis. Multiple cutaneous lesions were excised surgically, and extensive skin grafting of the left side of the face was performed. At the age of 35, she developed a tumor of the left upper and lower eyelids that extended into the orbit, which proved to be a sebaceous gland carcinoma. Following left orbital exenteration, the tumor promptly recurred in the socket. The patient died with widespread metastatic lesions nine months after exenteration. This case, which, to our knowledge, represents the fourth example of postradiation sebaceous gland carcinoma of the eyelids, differs from the previously reported cases in that the radiation therapy had been given for a benign cutaneous condition.

Adolescent↗

Phakomatous choristoma of eyelid: electron microscopical confirmation of lenticular derivation.

This article presents the sixth recorded example of phakomatous choristoma of the eyelid. The tumor occurred in a 4-month-old boy and was located in the lower lid near the inner canthus. The findings from light microscopy included epithelial, tubular structures with associated patches of thick, irregular basement membrane. The results of electron microscopy (ie, epithelial cells with a scarcity of organelles, homogeneous type of basement membrane, cytoplasmic microfilaments, segments of fascia occludens, and degenerated cells with peculiar interdigitations of their plasmalemmas) are all consistent with lenticular derivation, as originally proposed by Zimmerman.

Basement Membrane↗

Cerebro-hepato-renal syndrome of Zellweger. Ocular histopathologic findings.

An infant who was the product of a full-term pregnancy was born with multiple congenital defects, including diffuse bilateral corneal opacification. He failed to thrive and died at the age of 4 days. Autopsy findings were consistent with those seen in the cerebro-hepato-renal syndrome. Histopathologic examination of both eyes, obtained at autopsy, demonstrated similar changes. The main pathologic findings in the anterior segment were bilateral, diffuse corneal edema associated with paracentral iridocorneal adhesions and focal attenuation of Descemet membrane. Both retinas disclosed a selective degeneration of the outer nuclear layers and photoreceptors, confined mainly to the macular region. Atrophy of the optic disc and nerve, with reactive gliosis and demyelination, were consistent with findings in the central nervous system described in this syndrome.

Abnormalities, Multiple↗

Carcinoma metastatic to the eye and orbit III. A clinicopathologic study of 28 cases metastatic to the orbit.

Two hundred and twenty-seven cases of carcinoma metastatic to the eye and orbit have been reviewed previously. The orbit alone was involved in 28 cases: theses lesions constitute the basis of this clinicopathologic study. The most common signs and symptoms produced by orbital metastasis included exophthalmos (75%1, pain (29%), decreased vision (29%), periorbital swelling (25%), a visible mass (21%), ophthalmoplegia, and diplopia (18%). The ophthalmologists' preoperative (or premortem) clinical diagnoses were: orbital mass, 36%; metastatic carcinoma, 29%; leukemia, 7%; melanosarcoma, 4%; mixed tumor of lacrimal gland, 4%; and meningioma, 4%. The sites of the primary tumors in the 28 patients with orbital metastasis were as follows: breast, eight; lung, four; genitourinary tract, four; pancreas, one; and ileum, one. In 10 patients the site of the primary carcinoma was not determined. In 17 of the 28 patients, symptoms of orbital metastasis preceded detection of a primary tumor elsewhere in the body. In 10 of the remaining 11 patients, detection of the primary tumor had preceded the onset of orbital symptoms. In one patient, symptoms of the primary tumor and of orbital metastasis appeared at about the same time. The median survival of patients with carcinoma metastatic to the orbit was 15.6 months from the time of orbital surgery. This was much better than the median survival of the 227 patients in the overall study (7.4 months) and far better than the median survival of the patients with metastasis to the anterior segment of the eye (only 5.4 months).

Adenocarcinoma↗

Mucoepidermoid carcinoma of the conjunctiva: a clinicopathologic study of five cases.

Five patients with mucoepidermoid carcinoma of the conjunctiva were studied. The tumors were located close to the limbus in three patients, in the bulbar conjunctiva in one, and in the lower cul-de-sac in the remaining patient. Histopathologically, they exhibited lobules of tumor cells showing an admixture of epidermoid and mucus-secreting cells. Histochemical stains for mucin were most useful in arriving at a correct diagnosis. Follow-up information revealed that all five tumors recurred rapidly, invading the corneal stroma, intraocular structures, and orbit. Biologically, these tumors appear to be locally aggressive and should be differentiated histopathologically from the conventional squamous cell carcinoma of the conjunctiva, which carries a better prognosis. They should be managed by wide local excision, and the margins should be carefully examined for residual tumor. Frequent follow-up is recommended to detect early recurrence.

Aged↗

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. The pericytes were completely surrounded by casement membranes and displayed no significant lipidization; in a cellular plaque of vasular tissue at the base of the lesion, however, some of the multilaminar pericytes showed evidence of early smooth muscle differentiation. The stromal cells contained abundant lipid vacuoles and a few organelles, and exhibited granular degeneration of cytoplasmic filaments between the lipid vacuoles. There was spotty basement membrane formation where the stromal cells abutted on the vascular elements. No interconversion could be demonstrated among the endothelial cells, pericytes, and stromal cells. A source for the stromal cells was discovered in the early lipisization of fibrous astrocytes. Analysis of the extracted lipid from the tumor by means of infrared spectroscopy, lipid chromatography, and x-ray diffraction disclosed that the lipid was mostly cholestrol stearate, a plasma lipid. It is suggested that in the retinal lesions the leaky (fenestrated) capillaries of the tumor allowed the passive imbibition of plasma lipid by the fibrous astrocytes, leading to their gradual transformation into the fully lipidized stromal cells.

Adult↗

Alterations of Descemet's membrane in interstitial keratitis.

Twenty corneas from patients with interstitial keratitis were examined by light microscopy and one of these by electron microscopy. Seventy percent of patients had either a positive serologic test for syphilis or a history of treatment for syphilis. Focal or diffuse multilaminar thickening of Descemet's membrane with secondary linear guttata was present in 88% of cases. Confluent linear cornea guttata formed retrocorneal hyaline ridges that clinically showed a central gray core surrounded by a translucent sheath. Microscopically, these ridges consisted of concentric laminations of newly formed Descemet's membrane. Some ridges hung into the anterior chamber as a bow-like strand or a spiderweb network. A possible pathogenetic sequence may include (1) inflammatory insult to a relatively young endothelium; (2) alteration of endothelial function to fibroblast-like activity; (3) the production of abnormal basement membrane and other collagenous material and formation of a new multilaminar Descemet's membrane; and (4) separation of some ridges from the multilaminar Descemet's membrane to hang into the anterior chamber as strands and networks.

Anterior Chamber↗

Experimental lens-induced granulomatous endophthalmitis.

Experimental lens-induced granulomatous endophthalmitis is an experimental autoimmune disease that is histopathologically identical to the human disease known as phacoanaphylactic endophthalmitis or lens-induced uveitis. The capacity to develop experimental disease after lens injury can be passively transferred to virgin animals with hyperimmune serum. Fluorescein-labeled anti-IgG and anti-C'3 antisera bind to the lenses of appropriately sensitized animals after lens injury but not to the injured lenses of unsensitized control animals. Cobra Venom factor which inhibits C'3 prevents the development of lens-induced granulomatous uveitis in appropriately sensitized animals. Hypersensitized animals develop a massive Arthus-like reaction. On the basis of these observations we feel that experimental lens-induced granulomatous endophthalmitis is an immune complex disease. Because the experimental disease is histopathologically identical to human lens-induced uveitis or phacoanaphylactic endophthalmitis we believe an immune complex mechanism of immunopathogenesis is important in phacoanaphylactic endophthalmitis.

Animals↗

Carcinoma metastatic to the eye and orbit II. A clinicopathological study of 26 patients with carcinoma metastatic to the anterior segment of the eye.

In 26 of 227 patients with carcinoma metastatic to the eye or orbit, metastasis to the anterior uveal tract was the predominating feature. There was a definite propensity for the tumor to involve the horizontal meridian of the iris or ciliary body, rather than the upper or lower portions. The site of the primary tumor in the 26 patients was as follows: lung 14; breast, 9; kidney, 2; and rectum, 1. Ocular symptoms and signs produced by the metastatic tumors at onset or during the course of the disease included decreased vision (80%), a visible mass (72%), redness of the eye (56%), pain (56%), glaucoma (56%), iridocylitis (44%), and hyphema (24%). The median survival of the 26 patients with metastasis to the anterior segment of the eye was only 5.4 months from the time of ocular surgery. This is poorer than the median survival (7.2 months) of the patients with metastasis confined to the posterior segment, and much worse than the median survival (15.6 months) of the 28 patients with orbital involvement.

Adenocarcinoma↗

Ophthalmologic manifestations of granulocytic sarcoma (myeloid sarcoma or chloroma). The third Pan American Association of Ophthalmology and American Journal of Ophthalmology Lecture.

The clinicopathologic review of 33 well-documented cases of granulocytic sarcoma on file in the Registry of Ophthalmic Pathology confirmed the facts that this tumor is encountered mainly in children, that boys are affected more frequently than girls, and that white Americans appear to be less vulnerable than other ethnic groups. While granulocytic sarcoma is a variant of granulocytic leukemia, the tumor may appear before, after, or concomitantly with hematologic evidence of leukemia. In the present series only four of the 33 patients were already known to have leukemia when they were first seen by an ophthalmologist for their orbital, ocular, or adnexal lessions. The Leder stain has proved extremely helpful in arriving at a definitive histopathologic diagnosis. Accurate diagnosis is important to prevent inappropriate medical or surgical treatment and to indicate the need for vigorous antileukemic chemotherapy. Prognosis at best is poor.

Adolescent↗

Uveitis and reticulum cell sarcoma of brain with bilateral neoplastic seeding of vitreous without retinal or uveal involvement.

A 65-year-old woman had been observed for more than four years with bilateral chronic nongranulomatous uveitis and vitreous clouding of unknown cause. Her death was from reticulum cell sarcoma of the brain (microglioma). Both eyes were obtained post mortem. Histopathologic examination revealed malignant cell in the vitreous of both eyes, but no other tumor was demonstrable in the ocular tissues. Reticulum cell sarcoma should be suspected in middle-aged or older persons with chronic unilateral or bilateral uveitis of unknown cause who develop cerebral manifestations during the course of the disease.

Aged↗

Pseudorheumatoid nodules of the ocular adnexa.

A clinicopathologic study of 21 cases revealed pseudorheumatoid subcutaneous nodules occurring in the eyelids and periorbital region of children and young adults. These nodules were most frequently located in the lateral upper eyelids and outer canthal region, and in five cases they were multiple. The lesions recurred in about 20% of the cases. In addition, new lesions located elsewhere were observed in 26% of cases. Histopathologically they resembled the subcutaneous nodules of rheumatoid arthritis and rheumatic fever. Follow-up information on 15 patients (from two to 12 years) and the results of laboratory studies on seven patients had failed to reveal any association with these two systemic diseases. Pseudorheumatoid nodules may be indicated when a healthy child or a young adult presents with solitary or multiple subcutaneous nodules, especially in the eyelids. These lesions pursued a benign clinical course and local excision, which is usually performed for diagnostic purposes, was the only treatment indicated. The lesions had a tendency to regress spontaneously; therefore, local recurrences should be treated conservatively by periodic follow-up examinations.

Adolescent↗