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Biomedical subjects

J A Shields

Publications and source records attributed to J A Shields.

At least 217 records · Page 12Linked to original sources
PubMed1996

Diagnosis and treatment of uveal melanoma.

Most malignant melanomas in the ocular region arise in the uveal tract (iris, ciliary body, and choroid). Uveal melanoma generally has characteristic clinical features and the diagnosis can usually be made by an experienced ophthalmologist using slit lamp biomicroscopy or indirect ophthalmoscopy. Ancillary studies such as fluorescein angiography, ultrasonography, magnetic resonance imaging, and fine needle biopsy can occasionally be used to establish the diagnosis in atypical cases. Today, most affected patients are managed by specialists in ocular oncology. The management of uveal melanoma has been the subject of considerable controversy. Iris melanoma can usually be excised without enucleation of the affected eye. With regard to posterior uveal melanoma (ciliary body and choroid), enucleation of the affected eye was once the undisputed method of treatment. More recently, however, removal of the eye is performed less often and alternatives to enucleation have gained popularity. Several years ago, laser photocoagulation and plaque brachytherapy were the most popular alternatives to enucleation. Now, techniques of local tumor excision and transpupillary thermotherapy are gaining popularity in selected cases. Even more recently, various combinations of these methods have been judiciously used in many instances. The selected method of treatment in a given case depends on a number of complex clinical factors. Philosophies regarding the management of these lesions continue to change. This review covers the current diagnosis and management of uveal melanoma with emphasis on methods of management.

Brachytherapy↗
PubMed1996

Genetic aspects of uveal melanoma: a brief review.

Uveal melanoma usually occurs sporadically in the absence of obvious genetic predisposing factors. However, in rare patients, there is a suggestion that there may be genetic predisposition. Rare occurrences of familial uveal melanoma are believed to be inherited in an autosomal dominant mode. There are a few clinical conditions that can predispose to or be associated with uveal melanoma, including ocular melanocytosis, neurofibromatosis type I, and familial atypical mole and melanoma syndrome. Nonrandom cytogenetic changes in uveal melanoma are characterized by monosomy 3, trisomy 8, and structural or numerical abnormalities of chromosome 6. Alterations of chromosome 9p are less frequently observed. CDKN2 gene, a cutaneous melanoma predisposition gene, is probably not a uveal melanoma predisposition gene as evidenced by the lack of somatic mutations involving this gene in uveal melanoma samples and the absence of germline mutations in familial uveal melanoma patients. Transgenic mouse models developed using a tyrosinase promoter tagged with a mutated ras gene or SV40-Tag oncoprotein develop retinal pigment epithelium tumors that resemble uveal melanoma. We propose that uveal melanoma cases be categorized on genetic basis according to a new classification system. This classification scheme will help to identify and uniformly categorize uveal melanoma patients with genetic predisposition. Such patients offer unique opportunities for studying the genetic aspects of uveal melanoma and, therefore, appropriate tissue samples should be obtained from them for molecular genetic studies. Further studies are needed to fully understand the genetic aspects of uveal melanoma.

Animals↗
PubMed1995

Vasoproliferative tumors of the ocular fundus. Classification and clinical manifestations in 103 patients.

OBJECTIVE: To describe the clinical features of vasoproliferative tumors of the ocular fundus and to propose a comprehensive classification of these tumors. METHODS: A retrospective review of all cases that were diagnosed as acquired retinal hemangioma or vasoproliferative retinal tumor was conducted on the Ocular Oncology Service at Wills Eye Hospital, Philadelphia, Pa. RESULTS: There were 129 vasoproliferative tumors in 113 eyes of 103 patients. The tumors were classified as idiopathic in 84 eyes (74%) and secondary to preexisting ocular disease in 29 (26%). Subclassification into solitary (88 eyes), multiple (17 eyes), and diffuse (eight eyes) involvement was made. Of the 84 eyes with idiopathic tumors, 73 (87%) had solitary tumors, five (6%) had multiple tumors, and six (7%) had diffuse tumors. The lesion was located in the inferior, inferotemporal, or temporal region of the fundus in 78% and developed within 6 mm of the ora serrata retinae in 88%. Associated vitreoretinal findings included intraretinal exudation (82%), secondary exudative retinal detachment (48%), vitreous cells (46%), vitreous hemorrhage (21%), preretinal macular fibrosis (31%), and macular edema (18%). Of the 29 eyes with secondary tumors, the tumor was solitary in 15 (52%), multiple in 12 (41%), and diffuse in two (7%). The most common preexisting ocular disease included intermediate uveitis (pars planitis) in eight eyes (28%), retinitis pigmentosa in six (21%), toxoplasmic retinitis in two (7%), toxocariasis in two (7%), retinochoroidal coloboma in two (7%), and traumatic chorioretinopathy in two (7%). Retinal pigment epithelial hyperplasia was a prominent feature that was adjacent to 58% of the secondary tumors. Overall, management of the 129 tumors consisted of observation in 63 (49%), cryotherapy in 54 (42%), laser photocoagulation in seven (5%), plaque radiotherapy in three (2%), and other modes of treatment in two (2%). CONCLUSIONS: Vasoproliferative retinal tumors can be idiopathic, or they can develop secondary to congenital, inflammatory, vascular, traumatic, dystrophic, and degenerative ocular diseases. They can produce a variety of complications. Awareness and recognition of these tumors and differentiation from other retinal vascular tumors are important.

Adolescent↗
PubMed1995

Melanoma-associated retinopathy.

PURPOSE/METHODS: A 64-year-old man with a history of maxillary antrum melanoma had abrupt-onset night blindness and photopsia. RESULTS/CONCLUSIONS: Ophthalmologic examination and electroretinogram suggested melanoma-associated retinopathy. Serum produced a weak but specific labeling of human bipolar cells by indirect immunofluorescence. Complete excision of melanoma may have contributed to low levels of circulating autoantibodies. The identity of the retinal bipolar antigen recognized by melanoma-associated retinopathy autoantibodies is needed for more accurate diagnosis.

Electroretinography↗
PubMed1995

Metastatic tumors to the iris in 40 patients.

PURPOSE: Metastatic cancer to the iris is rare, and the best approaches to clinical recognition and treatment of this neoplasm are not widely known. We reviewed a series of patients with metastatic cancer to the iris and elucidated the clinical variations, diagnostic approaches, and treatment of this neoplasm. METHODS: The files of the Wills Eye Hospital Ocular Oncology Service were surveyed, and cases of metastatic cancer to the iris were extracted for further analysis. General data, systemic data, tumor characteristics, diagnostic methods used, treatment, and prognosis were tabulated. RESULTS: Of 512 patients with uveal metastasis, the iris was involved in 40 (7.8%). The metastasis originated from breast carcinoma in 16 cases, lung carcinoma in 11, carcinoid tumor in three, melanoma in three, colonic carcinoma in two, and esophageal, laryngeal, prostate, and kidney carcinoma in one case each. In one case the primary tumor was never identified. There was no history of primary cancer in 13 (32%) of 40 patients. All iris metastases were unilateral, and secondary glaucoma was present in 15 (38%). Fine-needle aspiration biopsy of the iris lesion helped to establish the diagnosis in 11 cases. Treatment with chemotherapy, radiotherapy, or both, achieved local tumor control in all patients. The overall mean survival was 20 months and the median survival was 13 months after diagnosis of the iris metastasis. CONCLUSIONS: Iris metastasis can show a spectrum of clinical variations, but its features are generally distinctive enough to differentiate it from other intraocular neoplasms and inflammations. Treatment consists of chemotherapy, which was sometimes combined with ocular irradiation. The visual prognosis was good but the systemic prognosis was generally poor.

Adult↗
PubMed1995

Multinucleate cell angiohistiocytoma of the orbit.

PURPOSE: A 31-year-old had a five-year history of painless progressive swelling of her right upper eyelid. METHOD: A mass was removed from the superonasal aspect of the right orbit. RESULTS: The orbital mass was composed of a collagenous stroma with capillary channels and irregular giant cells. The findings were characteristic of multinucleate cell angiohistiocytoma. CONCLUSION: Multinucleate cell angiohistiocytoma should be included in the differential diagnosis of circumscribed orbital tumors.

Adult↗
PubMed1995

Natural course and histopathologic findings of lacrimal gland choristoma of the iris and ciliary body.

PURPOSE: To show a well-documented clinicopathologic correlation of lacrimal gland choristoma of the iris and ciliary body, to review the literature on the subject, and to recommend treatment of this condition. METHODS: A congenital iridociliary mass was removed by iridocyclectomy when a female infant was 19 months old because it had produced a large cyst, secondary glaucoma, a vascularized corneal pannus, focal cataract, and iris heterochromia secondary to pigment dispersion. The excised mass was studied by routine histopathologic methods. RESULTS: The mass proved histopathologically to be a lacrimal gland choristoma composed of mature lacrimal gland tissue associated with a large epithelial-lined cyst and several smaller cysts. Postoperatively, the glaucoma resolved and the child had visual acuity of 20/40 14 months after surgery. CONCLUSIONS: Lacrimal gland choristoma of the anterior uvea has typical clinical features that should indicate that correct diagnosis. It is a fleshy, often cystic, lesion with an irregular, cerebriform surface that appears identical to the lacrimal gland in the orbit. Although the mass itself is generally stable, secondary cysts in the lesion can enlarge and secondary glaucoma and other complications can develop. Consequently, affected patients should be followed up at close intervals and surgical excision performed if such complications begin to develop.

Choristoma↗
PubMed1995

Transesophageal atrial pacing (TAP) for sinus bradycardia during coronary artery bypass grafting: comparison of TAP to intermittent bolus gallamine.

OBJECTIVE: To assess the relative efficacy of a pacing esophageal stethoscope and intermittent boluses (40 mg) of gallamine in correcting sinus bradycardia (SB) during coronary artery surgery. DESIGN: The study was prospective, randomized, and controlled. SETTING: A community hospital. PARTICIPANTS: Fifty patients scheduled for elective coronary artery surgery. INTERVENTIONS: The patients were randomly allocated to receive treatment for an SB (less than 60 BPM) with either transesophageal atrial pacing (TAP) or gallamine. MEASUREMENTS AND MAIN RESULTS: Heart rate, blood pressure, and systemic hemodynamics were measured. The electrocardiogram was monitored for rate, rhythm, and conduction abnormalities. Twenty-four of the 25 TAP patients could be paced at a rate of 70 BPM after SB. Cardiac index increased from 1.90 to 2.56 L/min/m2. In the gallamine group, heart rate was increased from 50 to 66 BPM, but cardiac index only increased to 2.2 L/min/m2, and 2 patients developed nodal rhythms. Eight of these patients had peak heart rates over 80 BPM, and two were over 90 BPM. CONCLUSIONS: The ability to reliably and precisely control heart rate was superior with TAP compared with intermittent bolus dosing with gallamine.

Arrhythmia, Sinus↗
PubMed1995

Orbital varix presenting as a subconjunctival mass.

A 39-year-old woman presented with a vermiform superior conjunctival mass that progressively enlarged during 4 years of follow-up. Although magnetic resonance imaging suggested that the lesion was located in the superior forniceal conjunctiva, surgical exploration disclosed a vascular anomaly (varix) that extended deep into the orbit. The clinical features and management of orbital varices are discussed.

Adult↗
PubMed1995

Patterns of indocyanine green videoangiography of choroidal tumours.

BACKGROUND: Indocyanine green video-angiography is a recently popularised technique for imaging the retina and choroid. Little is known about the value of this technique in the evaluation of choroidal tumours. METHODS: Indocyanine green video-angiograms of 51 patients with choroidal tumours, including 32 with choroidal melanoma, five with choroidal naevus, four with choroidal metastasis, four with choroidal haemangioma, one with choroidal osteoma, one with choroidal vortex vein varix, and four with irradiated choroidal melanoma were reviewed in a masked fashion. RESULTS: The choroidal melanoma group achieved maximal fluorescence at an average of 18.2 (range 0.4 to 60) minutes after injection. At maximum fluorescence, the pattern varied from hypofluorescent in 17 cases, to isofluorescent in eight cases, and hyperfluorescent in seven cases. Non-pigmented choroidal melanoma generally showed an earlier average onset of fluorescence than the pigmented choroidal melanoma (mean 28 versus 185 seconds, respectively) (p = 0.04). The two subgroups otherwise overlapped substantially in angiography characteristics. The choroidal metastasis group demonstrated maximal fluorescence at an average of 8.9 (range 1.7 to 13) minutes. All choroidal metastases had a homogeneous diffuse fluorescence with late isofluorescence. The choroidal metastases allowed a subtle blurred perception of the normal choroidal pattern through the tumour. The choroidal haemangioma group revealed very characteristic findings with an onset of filigree fluorescence at an average of 0.6 (range 0.4 to 0.7) minutes with little variation. The maximal fluorescence was typically hyperintense in all cases and was achieved at an average of 1.2 minutes and was reached by 1.8 minutes in all cases. In these cases the fluorescence appeared as a lacy diffuse 'fluorescent mulberry' pattern with visibility of the vascular channels and demonstrated 'washout' of the dye in the late frames. CONCLUSION: Indocyanine green may be a useful adjunct in the differentiation of amelanotic choroidal melanoma, choroidal metastasis, and choroidal haemangioma. The different patterns of fluorescence may be explained by the intrinsic choroidal vascular architecture in these three tumours.

Brachytherapy↗
PubMed1995

Treatment of non-resectable malignant iris tumours with custom designed plaque radiotherapy.

BACKGROUND: Plaque radiotherapy is the most common method of managing posterior uveal melanoma but its use for iris melanoma and iris metastases has not yet been evaluated. METHODS: Fourteen patients with non-resectable iris melanoma and four with iris metastasis were treated with plaque radiotherapy. The tumour response to treatment and the local side effects of the radioactive plaque were evaluated. RESULTS: In the iris melanoma group over a mean follow up of 26 (range 6-75) months, the tumour regressed in 13 of the 14 patients (93%) and recurred as diffuse seeding in one patient (7%). Despite large doses of radiation given transcorneally, the cornea developed epitheliopathy, abrasion, and oedema in only one case each. The major radiation side effects were localised iris vasculopathy without glaucoma in two cases, posterior synechiae in five cases, and cataract in six cases. In the iris metastasis group, tumour regression was observed in all four patients (100%) and radiation side effects were not evident over the relatively short mean follow up period of 8 (range 4-9) months. All of the 14 patients with irradiated iris melanoma have remained systemically healthy without metastasis while three of the four patients with irradiated iris metastases have died of metastases from the primary neoplasm. CONCLUSION: Custom designed plaque radiotherapy appears to be an effective alternative method of controlling non-resectable diffuse iris melanoma and solitary iris metastasis and has relatively few side effects.

Brachytherapy↗
PubMed1995

Uveal melanoma and familial atypical mole and melanoma (FAM-M) syndrome.

We conducted this study to determine whether occurrence of primary uveal melanoma in the setting of familial atypical mole and melanoma (F A M-M) syndrome (an autosomal dominant cutaneous preneoplastic syndrome) follows a pattern of a hereditary cancer predisposition syndrome. A retrospective review of 4600 consecutive patients with primary uveal melanoma revealed eight patients with biopsy-proven F A M-M syndrome. The clinical profile of these patients was studied and their kindreds analyzed. In patients with F A M-M syndrome, the uveal melanoma occurred at a relatively young age (mean 40 years; range 10-52 years). The diagnosis of F A M-M syndrome preceded or followed the diagnosis of uveal melanoma by as much as 10 years. None of the patients had an associated nonmelanocytic malignancy. Three of the eight patients had a positive family history of melanoma (cutaneous melanoma (2) and uveal melanoma (1). The authors conclude that the occurrence of primary uveal melanoma in the setting of F A M-M syndrome does not follow a clear pattern of a hereditary cancer predisposition syndrome.

Adult↗