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Biomedical subjects

J A Shields

Publications and source records attributed to J A Shields.

At least 163 records · Page 9Linked to original sources

Diffuse ocular metastases as an initial sign of metastatic lung cancer.

A 68-year-old man with lung carcinoma and no systemic metastasis presented with a blind, painful right eye. Examination showed no perception of light in the affected eye, elevated intraocular pressure, marked epibulbar hyperemia, and a white placoid mass in the conjunctiva nasally. Although a cataract precluded a clear view of the fundus, ultrasonography disclosed a total retinal detachment and a diffuse thickening of the choroid. Metastatic carcinoma was suspected clinically and the eye was enucleated because of severe, intractable pain. Pathologic examination demonstrated extensively necrotic metastatic adenocarcinoma involving the conjunctiva, peripheral cornea, sclera, iris, ciliary body, choroid, optic nerve, subarachnoid space, and orbit. Metastatic disease usually affects a singular ocular tissue, and it is highly unusual for such widespread ocular involvement to be the first sign of systemic metastasis from a primary neoplasm.

Adenocarcinoma↗

External beam irradiation for choroid metastases: identification of factors predisposing to long-term sequelae.

PURPOSE: To improve overall quality of life, palliative treatments should attempt to minimize associated complications while effectively controlling specific symptoms. We reviewed our experience treating posterior uveal metastases with external beam radiotherapy (EBRT) to determine the complication rate and to identify the relationship between patient, tumor, or treatment-related factors and the development of ocular complications. METHODS AND MATERIALS: 483 consecutive patients (pts) (578 eyes) were diagnosed with intraocular metastatic disease from solid tumors between 1972-1995. Of these, 233 eyes (188 pts) had lesions of the posterior uveal tract and received EBRT. Median follow-up time was 5.8 months (range: 0.7-170.0 months). Follow-up information regarding the development of complications was documented for 230 eyes. Complete EBRT details were available for 189 eyes. Seventy-two percent of the patients received 30.0-40.0 Gy in 2.0-3.0 Gy fractions. Biologically effective dose (BED) was calculated to allow meaningful comparisons between various fractionation regimens and total doses. Concurrent chemotherapy and/or hormonal therapy was used for 101 eyes (44%). RESULTS: Median BED was 61 Gy3 (range, 6.7-105 Gy3), and 80% of treated eyes received BED 50-70 Gy3. EBRT energies included photons (70%), 60Co (19%), electrons (6%), mixed energies (3%), and orthovoltage (2%). Lens-sparing techniques were used in 136 eyes (71%). At last follow-up 28 eyes (12%) developed one or more significant complications, including cataracts (16 eyes), radiation retinopathy (6 eyes), optic neuropathy (5 eyes), exposure keratopathy (5 eyes), and neovascularization of the iris (4 eyes). Two eyes developed narrow-angle glaucoma, and one of these required enucleation. On univariate analysis, Caucasian race (vs. Black/Hispanic, p = 0.03), increased intraocular pressure at diagnosis (>21 mmHg, p = 0.02), and diagnosis by biopsy (vs. no biopsy, p = 0.03) predisposed toward the development of complications. Factors not correlated with complications included BED (p = 0.18), energy type (p = 0.81), lens-sparing technique (versus whole globe, p = 0.57), and concurrent systemic treatment (p = 0.60). The small number of complications did not support a multivariate analysis. CONCLUSIONS: Despite the employment of a variety of EBRT treatment techniques and the proximity of choroidal metastases to radiosensitive structures, significant complications of palliative EBRT were infrequent. Although complications do occur, they are related to host factors and do not appear to be a function of irradiation parameters. We conclude that the potential benefits of vision and globe preservation after palliative EBRT outweigh the small risk of treatment induced complications.

Adult↗

Plaque radiotherapy for the management of uveal metastasis.

BACKGROUND: Radiotherapy is effective for the management of most uveal metastases, and standard external beam radiotherapy is generally administered during a 3-to 4-week period. For those patients in whom external beam radiotherapy or other methods fail or those patients with solitary uveal metastases, plaque radiotherapy may be an alternative method. OBJECTIVE: To determine the effectiveness of plaque radiotherapy for the management of uveal metastasis. METHODS: A retrospective review of 36 patients with uveal metastases who were examined at the Oncology Service at Wills Eye Hospital, Philadelphia, Pa, and treated with plaque radiotherapy. The clinical findings and follow-up data of the primary tumor and the uveal metastasis were analyzed. RESULTS: Of the 36 patients, 27 (75%) received plaque treatment as primary therapy for the uveal metastasis and 9 (25%) received plaque treatment as secondary therapy after failure of the uveal tumor to respond to external beam radiotherapy, chemotherapy, or hormonal therapy. During treatment, 22 patients (61%) had no other systemic metastasis and 14 (39%) had controlled systemic metastasis. No patients had active metastasis elsewhere. The uveal metastasis was solitary and well circumscribed in all but 1 patient; it measured a mean of 11 mm in basal dimension and 4 mm in thickness. The mean time for treatment was 86 hours, and the mean therapeutic dose was 68.80 Gy to the tumor apex and 235.64 Gy to the tumor base. Regression of the uveal metastasis was documented in 34 patients (94%) during a mean follow-up of 11 months. As early as 3 months after treatment, the mean tumor thickness had decreased to 2 mm. Plaque radiotherapy salvaged 5 of the 6 eyes that had failed prior external beam radiotherapy. Radiation retinopathy, radiation papillopathy, or both were found in 3 patients (8%) and occurred at a mean of 8 months after treatment. At the last examination, 18 patients (50%) were alive (11 with and 7 without active systemic metastasis) and 18 (50%) were dead from systemic metastasis. CONCLUSIONS: Plaque radiotherapy is an effective method for treating selected solitary uveal metastasis. It offers a high degree of tumor control, especially for those eyes in which other methods have failed. Plaque treatment is provided during a short period, minimizing the time demand for these patients with a limited life expectancy.

Adult↗

Surgical management of conjunctival tumors. The 1994 Lynn B. McMahan Lecture.

To our knowledge, there are no articles that describe the specific step-by-step details of the surgical removal of premalignant and malignant conjunctival tumors. We describe our current approach to the surgical management of squamous cell carcinoma (intraepithelial or invasive), localized melanoma, and primary acquired melanosis of the conjunctiva. The surgical method differs with limbal tumors, extralimbal tumors, and primary acquired melanosis. Limbal lesions are managed by localized alcohol corneal epitheliectomy, removal of the main mass by a partial lamellar scleroconjunctivectomy, and supplemental cryotherapy. Tumors located in the extralimbal conjunctiva are managed by alcohol application, wide circumferential surgical resection, and cryotherapy. Primary acquired melanosis is managed by alcohol epitheliectomy, removal of suspicious foci, quadrantic staging biopsies, and cryotherapy from the underside of the conjunctiva. In all cases, a "no touch" method is used and direct manipulation of the tumor is avoided to prevent tumor cell seeding into a new area. We have employed this technique on 109 patients with conjunctival squamous neoplasms and 137 patients with conjunctival melanoma, about 80 of which neoplasms were associated with primary acquired melanosis. Our observations suggest that well-planned initial surgical management using this technique decreases the chance of tumor recurrence for conjunctival melanoma and squamous cell carcinoma. We describe a detailed stepwise approach to the surgical management of conjunctival neoplasms. It requires meticulous clinical evaluation and complete removal of the tumor in one operation using a specific technique.

Anesthesia, Local↗

Histopathologic study of eyes after iodine I 125 episcleral plaque irradiation for uveal melanoma.

OBJECTIVES: To describe the histopathologic findings attributable to irradiation in eyes with uveal malignant melanoma treated with iodine I 125 brachytherapy and to compare these findings with those reviewed in a previous study that compared histopathologic findings in eyes enucleated after proton beam teletherapy with those seen in eyes in a nonirradiated control group. METHODS: The slides from 22 eyes with uveal melanoma that had undergone enucleation after the administration of 125I brachytherapy were studied. The histopathologic features of the tumor and the retina were graded. Results were compared with findings from a previously reported group of 47 proton beam-treated eyes and its control group. RESULTS: Patient age, time between irradiation and enucleation, and ciliary body involvement were similar for the 125I brachytherapy-treated group and the proton beam-treated group and its control group. This allows comparison of the histopathologic findings. Comparing the 125I brachytherapy- and proton beam-treated groups, most histopathologic features were similar with nominally statistically significant differences only for cell type, number of mitotic figures, and fibrous metaplasia of the retinal pigment epithelium adjacent to the tumor. CONCLUSIONS: Irradiation of uveal melanoma induces changes in the tumor and in the surrounding retina. Brachytherapy and charged particle therapy are the 2 principal methods of irradiation. This study demonstrates that similar changes are produced by 125I plaque irradiation and proton beam irradiation.

Adult↗

Melanogenic neuroectodermal tumor of the retina (primary malignant melanoma of the retina).

A 35-month-old girl with leukocoria was clinically diagnosed with unilateral sporadic retinoblastoma. Macroscopic examination of her enucleated eye disclosed a white retinal tumor that appeared to be a retinoblastoma. Histopathologic examination, however, revealed that the tumor was composed of poorly differentiated neuroblastic cells, larger spindle-shaped cells, and anaplastic epithelioid cells, which is inconsistent with retinoblastoma. Immunohistochemical testing disclosed that the tumor cells were immunoreactive for melanoma-specific antigen HMB-45, while electron microscopy showed premelanosomes in the tumor cells, both of which are consistent with melanogenesis. To our knowledge, such an ocular tumor has not been reported previously.

Antigens, Neoplasm↗

Adenoid cystic carcinoma developing in the nasal orbit.

PURPOSE: To describe the clinical, imaging, and histopathologic findings of an adenoid cystic carcinoma that occurred in the medial orbit. METHODS: A 26-year-old man developed a painless soft tissue mass in the nasal aspect of the left orbit. Computed tomography was done, and incisional biopsy performed elsewhere disclosed adenoid cystic carcinoma. Orbital exenteration was performed. RESULTS: Computed tomography showed a 1.5cm mass in the antronasal region of the orbit without bone erosion. Histopathology disclosed adenoid cystic carcinoma. CONCLUSIONS: Although orbital adenoid cystic carcinoma usually originates superotemporally in the main lacrimal gland, it can develop in the nasal orbit, possibly from ectopic lacrimal gland tissue. Adenoid cystic carcinoma should be included in the differential diagnosis of tumors of the nasal orbit.

Adult↗

Conjunctival metastasis as the initial manifestation of lung cancer.

PURPOSE: To report a case of conjunctival metastasis as the initial manifestation of lung cancer. METHOD: A 55-year-old man without known systemic disease developed a conjunctival mass in his right eye. He underwent excision of the tumor and systemic evaluation. RESULTS: Histopathologically, the mass was a metastatic squamous cell carcinoma. Further evaluation disclosed a primary bronchogenic carcinoma with metastasis to ribs and brain. The primary and secondary tumors were treated with chemotherapy and irradiation. CONCLUSIONS: Conjunctival metastasis may be the initial manifestation of lung cancer. It should be considered in the differential diagnosis of a deep conjunctival mass.

Brain Neoplasms↗

Ewing sarcoma metastatic to the iris.

PURPOSE: To report a case of Ewing sarcoma metastatic to the iris. METHODS: A 19-year-old woman with metastatic Ewing sarcoma of the femur developed a diffuse, fluffy iris mass with a pseudohypopyon in the left eye. A fine-needle aspiration biopsy confirmed iris metastasis, and external beam radiotherapy was given to the affected eye. RESULTS: The iris mass responded initially to external beam radiotherapy and ongoing chemotherapy with vincristine, cyclophosphamide, doxorubicin, etoposide, and ifosfamide but recurred 5 months later. Subsequent radiotherapy with an iodine 125 plaque achieved further resolution of the iris tumor. CONCLUSIONS: Ewing sarcoma can rarely metastasize to the iris. The diagnosis can be confirmed by fine-needle aspiration biopsy. Plaque radiotherapy is a therapeutic alternative.

Adult↗

Retinal capillary hemangioma in Marshall-Stickler syndrome.

PURPOSE: Retinal capillary hemangioma, often associated with von Hippel-Lindau syndrome, is not characteristically seen in other conditions. We report a patient with Marshall-Stickler syndrome who had a retinal capillary hemangioma. METHOD: A 31-year-old man with Marshall-Stickler syndrome was evaluated for a retinal vascular tumor. RESULTS: Evaluation showed a retinal capillary hemangioma of the von Hippel type. Magnetic resonance imaging disclosed no tumors of brain, kidney, or other organs. The retinal tumor was treated with cryotherapy. CONCLUSION: Retinal capillary hemangioma is not pathognomonic of von Hippel-Lindau syndrome, and it may be associated with the Marshall-Stickler syndrome.

Adult↗

Trifocal uveal melanoma.

PURPOSE: To report the singular case of a patient who developed three noncontiguous uveal melanomas over a 30-year period. METHOD: Case report. RESULT: Systemic evaluation of a 50-year-old man with an iris melanoma and bilateral choroidal melanomas disclosed no evidence of metastases or other primary neoplastic disease. CONCLUSION: Although rare, the possibility of bilateral and multifocal uveal melanoma should be recognized.

Brachytherapy↗

Non-African Burkitt lymphoma presenting with oral thrush and an orbital mass in a child.

PURPOSE: To report an unusual case of orbital involvement with non-African Burkitt lymphoma in a child. METHODS: A 26-month-old boy developed oral thrush and painless right proptosis with eyelid swelling. Magnetic resonance imaging disclosed a diffuse mass involving the inferior, lateral, and superior right orbit and both maxillary sinuses. Incisional biopsy of the orbital mass was performed. RESULTS: Histopathology disclosed diffuse infiltration of the orbital tissues by a high-grade lymphoid neoplasm that showed positive immunoreactivity for B-cell markers. Immunoglobulin gene rearrangement studies disclosed a characteristic t(8;14) translocation consistent with a small, noncleaved malignant lymphoma of the Burkitt type. CONCLUSION: Non-African Burkitt lymphoma may manifest with orbital involvement.

Antineoplastic Combined Chemotherapy Protocols↗

Combined chemoreduction and adjuvant treatment for intraocular retinoblastoma.

OBJECTIVE: The purpose of the study is to investigate chemoreduction and adjuvant treatment (AT) for retinoblastoma and its effect on complete retinal tumor control, vitreous seed control, and subretinal seed control. DESIGN: The study design was a prospective, nonrandomized clinical trial. PARTICIPANTS: There were 130 intraocular retinoblastomas in 52 eyes of 32 consecutive patients observed for at least 1 year after initiation of treatment. INTERVENTION: Treatment with chemoreduction using vincristine, etoposide, and carboplatin (VEC) and adjuvant treatment (+ AT) (cryotherapy, laser photocoagulation, thermotherapy, chemothermotherapy, plaque radiation therapy, or external beam radiation therapy) were assessed. MAIN OUTCOME MEASURES: The effect of chemoreduction for 6 cycles (VEC x 6) versus fewer than 6 cycles (VEC x <6) on retinoblastoma control was analyzed. Furthermore, the impact of adjuvant treatment (+ AT) versus no adjuvant treatment (no AT) on retinoblastoma control was analyzed. RESULTS: Retinal tumors showed favorable initial regression with chemoreduction. Adjuvant treatment was applied to 93% of the retinal tumors after chemoreduction and only 2% recurred over the mean follow-up of 17 months (range 13-27 months). Vitreous seeds and subretinal seeds showed initial regression and often complete disappearance with chemoreduction. In those eyes with seeds before treatment, the addition of AT to VEC for 6 cycles decreased the vitreous seed recurrence from 75% to 0% (P = 0.04) and also decreased the subretinal seed recurrence from 67% to 0% (P = 0.003). More important, when considering that enucleation or external beam radiation therapy was the only other treatment option for these 52 eyes, the authors were successful in avoiding these methods in 42% of cases. Of the 36 eyes classified as Reese-Ellsworth group 5, there was 78% ocular salvage, and external beam radiation therapy was avoided in 25% of these eyes. There was a 100% ocular salvage in the group 5 eyes that received VEC for 6 cycles + AT to retinal tumors and seeds. CONCLUSIONS: Chemoreduction and AT to intraocular retinoblastoma and its seeds provides good retinal tumor control, even in eyes with advanced disease. Chemoreduction alone generally is not adequate to achieve complete tumor seed control. Cautious follow-up of affected patients is recommended because the risk for recurrent vitreous and subretinal seeds is substantial and proper treatment is critical for salvaging the eye.

Antineoplastic Combined Chemotherapy Protocols↗

Complications of motility peg placement for the hydroxyapatite orbital implant.

PURPOSE: The hydroxyapatite implant is an ocular motility implant designed to provide natural movement of the artificial eye. The movement of the prosthesis is maximized when the implant is coupled to the prosthesis via a peg. The purpose of this study is to determine the complications of the hydroxyapatite motility peg and the factors related to those complications. DESIGN: Retrospective review of 47 cases over 5 years. METHODS: A retrospective review was performed on all cases of hydroxyapatite motility peg placement. The technique of hydroxyapatite and peg placement, follow-up details, and complications of the peg were recorded. RESULTS: The complications of peg placement included peg extrusion in 26% (12 of 47), nonspecific conjunctivitis in 6% (3 of 47), audible click in 6% (3 of 47), temporary excessive conjunctival edema in 4% (2 of 47), and temporary excessive postoperative pain in 4% (2 of 47). There were no cases of infection, persistent pain, persistent edema, or discharge at peg site. The median time interval from peg placement to extrusion was 16 months (range, 1-52 months). The only statistically significant factor related to peg extrusion was age over 50 years (P = 0.04). There was a trend toward peg extrusion with use of a nonsleeved peg (versus sleeved peg) (P = 0.10). The extrusion rate was 32% (12 of 38) for nonsleeved pegs and 0% (0 of 9) for sleeved pegs. Factors unrelated to peg extrusion were patient sex, prior ocular surgery or radiotherapy, presence of giant papillary conjunctivitis, time interval from enucleation to peg placement, and degree of implant vascularization on magnetic resonance imaging. Of the 12 nonsleeved pegs that extruded, a sleeved peg system was subsequently successfully placed in 5 patients, a nonsleeved peg in 1 patient, and 6 patients remained without a peg system. CONCLUSIONS: Hydroxyapatite motility pegs have relatively few complications except for extrusion. The rate of extrusion can be minimized by employing a sleeved peg rather than a nonsleeved peg system.

Adolescent↗

Survey of 520 eyes with uveal metastases.

OBJECTIVE: The purpose of this investigation is to report the clinical features of patients with uveal metastases seen at a major ocular oncology center. DESIGN/PARTICIPANTS: A retrospective chart review was performed on all patients with uveal metastases evaluated at an ocular oncology outpatient facility over a 20-year period. MAIN OUTCOME MEASURE: To assess the systemic and ophthalmic features of uveal metastases. RESULTS: A total of 950 uveal metastases were diagnosed in 520 eyes of 420 consecutive patients. Of the 950 metastatic foci, the uveal involvement included iris in 90 (9%), ciliary body in 22 (2%), and choroid in 838 (88%). The total number of uveal metastases per eye was 1 (71%) in 370 eyes, 2 (12%) in 63 eyes, and 3 or more (17%) in 87 eyes. The mean number of uveal metastases per eye was two (median, one). Iris metastases presented most often as a yellow-to-white solitary nodule in the inferior quadrant. Ciliary body metastases typically presented as a solitary, sessile, or dome-shaped yellow mass in the inferior quadrant, but were difficult to visualize directly. The choroidal metastases typically were yellow in color, plateau shaped, and associated with subretinal fluid. In the 479 eyes with choroidal metastases, the epicenter of the main tumor was found in the macular area in 59 eyes (12%), between the macula and equator in 383 eyes (80%), and anterior to the equator in 37 eyes (8%). The mean size of the main (largest) choroidal tumor in each eye was 9 mm in base and 3 mm in thickness. At the time of ocular diagnosis, 278 patients (66%) reported a history of a primary cancer and 142 patients (34%) had no history of a cancer. Subsequent evaluation of these 142 patients after the ocular diagnosis of uveal metastasis showed a primary tumor in the lung in 50 patients (35%), breast in 10 (7%), others in 9 (6%), and no primary site was found in 73 patients (51%). Nearly half of the patients with no known primary site eventually died of diffuse metastatic disease. In the entire group of 420 patients, the uveal metastasis came from a primary cancer of the breast in 196 (47%), lung in 90 (21%), gastrointestinal tract in 18 (4%), kidney in 9 (2%), skin in 9 (2%), prostate in 9 (2%), and other cancers in 16 (4%). In 73 cases (17%), the primary site was never established despite systemic evaluation by medical oncologists. CONCLUSIONS: Iris, ciliary body, and choroidal metastases have typical clinical features that should suggest the diagnosis. The choroid is the most common site for uveal metastases, and the tumors occur most often in the posterior pole of the eye with an average of two tumors per eye. Approximately one third of patients have no history of primary cancer at the time of ocular diagnosis. Breast and lung cancers represent more than two thirds of the primary tumor sites.

Aged↗

Ocular manifestations of the organoid nevus syndrome.

BACKGROUND: The organoid nevus (sebaceous nevus) syndrome is characterized primarily by cutaneous sebaceous nevus, seizures, and epibulbar choristomas. Based on ophthalmoscopy and computed tomography (CT), a yellow fundus lesion recently observed in this syndrome has been called a coloboma by some authors or a choroidal osteoma by others. This study was undertaken to review the authors' personal experience with the organoid nevus syndrome, to review the English language literature on the subject, and to address some misconceptions regarding its ocular manifestations. METHODS: The authors reviewed the records of patients with the organoid nevus syndrome who were personally evaluated by the authors. The ocular findings were studied in more detail, with emphasis on the epibulbar and fundus lesions. RESULTS: The authors identified five patients with the organoid nevus syndrome. Four had a classic sebaceous nevus in the facial and scalp area and two had seizures and arachnoid cysts. All five patients had an epibulbar tumor, which proved to be a complex choristoma in one case that was studied histopathologically. A characteristic ophthalmoscopic feature, observed in the four patients with clear ocular media, was a flat, yellow discoloration of the posterior fundus, of variable size and shape, that appeared to correlate with a dense plaque noted on ultrasonography and CT. In one case, histopathologic studies showed that this posterior lesion contained intrascleral cartilage. CONCLUSIONS: The authors' observations and a review of the literature indicated that the organoid nevus syndrome has varied manifestations. Just like the closely related phakomatoses, it often occurs as a forme fruste, without full expression of the syndrome. The most important ocular manifestations are an epibulbar mass, compatible with a complex choristoma, and focal, yellow discoloration in the fundus, probably related to intrascleral cartilage.

Adolescent↗