National Cancer Institute®
Last Modified: March 1, 2002
UI - 11642491
AU - Shields JA; Shields CL; Brotman HK; Carvalho C; Perez N; Eagle RC Jr
TI - Cancer metastatic to the orbit: the 2000 Robert M. Curts Lecture.
SO - Ophthal Plast Reconstr Surg 2001 Sep;17(5):346-54
AD - Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania 19107, USA.
PURPOSE: To report the demographics and clinical features of a large series of patients with orbital metastasis. METHODS: Retrospective chart review on 100 consecutive patients and a literature review on orbital metastasis. RESULTS: Of 100 patients, the primary tumor site was breast in 53 (53%), prostate gland in 12 (12%), lung in 8 (8%), skin (melanoma) in 6 (6%), kidney in 5 (5%), gastrointestinal tract in 5 (5%), choroid (melanoma) in 2 (2%), parotid gland in 1 (1%), and adrenal gland (neuroblastoma) in 1 (1%). Of patients in whom a detailed history was available, there was no history of cancer at the time of presentation in 19%. In 10%, the primary tumor remained undetected despite systemic evaluation. There were 36 male patients and 64 female patients whose mean age at diagnosis was 62 years (median 60 years, range 5 to 91 years). Both the right and left orbits were affected equally, and 4 cases (4%) were bilateral. The most frequent clinical findings were limited ocular motility (54%), proptosis (50%), and palpable mass (43%). The diagnoses were established by history, systemic survey, imaging studies, and biopsy. Treatment included chemotherapy, hormone therapy, irradiation, surgical excision, or observation, depending on clinical circumstances. Among patients with sufficient follow-up, 95% died of metastasis, with overall mean survival of 15 months (median 15 months; range 3 to 96 months) after orbital diagnosis. CONCLUSIONS: The most common primary cancers that metastasize to the orbit are breast, prostate gland, and lung cancer. In 19%, there is no history of cancer when the patient presents with ophthalmic symptoms and in 10% the primary site remains obscure despite systemic evaluation. The systemic prognosis is generally poor.
UI - 11815341
AU - Anastassiou G; Heiligenhaus A; Bechrakis N; Bader E; Bornfeld N; Steuhl
TI - KP Prognostic value of clinical and histopathological parameters in conjunctival melanomas: a retrospective study.
SO - Br J Ophthalmol 2002 Feb;86(2):163-7
AD - Department of Ophthalmology, University Hospital Essen, University of Essen, Germany. firstname.lastname@example.org
AIM: To determine prognostic factors for recurrence of disease and tumour related mortality in patients with conjunctival melanoma. METHODS: A retrospective analysis of clinical and histopathological data of 69 patients with histologically verified conjunctival melanoma. RESULTS: As univariate analysis showed, significant risk factors for the development of recurrence were: irregular pigmentation (RR = 2.0, p = 0.0007), incomplete surgical excision (RR = 3.5, p = 0.008), tumour invasion deeper than in substantia propria (RR = 3.9, p = 0.008), and presence of epithelioid tumour cells (RR = 2.9, p = 0.05). For tumour related mortality a significantly increased risk was found for tumour location in palpebral conjunctiva, caruncle, plica, or fornices (RR = 5.9, p = 0.001), for tumour infiltration deeper than the substantia propria (RR = 5.5, p = 0.001), for incomplete surgical excision (RR = 4.4, p = 0.05), and for nodular or mixed (nodular and superficial) growth pattern of the tumours (RR = 1.2, p = 0.002). The use of an adjuvant therapy for the surgical excision of the melanomas had no statistically significant influence upon the development of recurrent disease nor upon the tumour related mortality. CONCLUSION: These data present similar clinical and histopathological risk factors for patients with conjunctival melanoma as reported previously. The present study also addresses the failure of retrospective studies on conjunctival melanoma to prove the efficacy of a supplementary therapy to surgical excision.
UI - 11857383
AU - Iscovich J; Abdulrazik M; Cour C; Fischbein A; Pe'er J; Goldgar DE
TI - Prevalence of the BRCA2 6174 del T mutation in Israeli uveal melanoma patients.
SO - Int J Cancer 2002 Mar 1;98(1):42-4
AD - Selikoff Center for Environmental Health and Human Development and the International Fertility Institute, Ra'anana, Israel. email@example.com
Substantial differences exist in the incidence rates of uveal melanoma (UM) among Israeli Jewish subpopulations: high in immigrants from North America and Europe (Ashkenazic) and low in immigrants from Africa and Asia (Sepharadic). This trend persists in Israeli-born individuals when stratified by their ancestral place of birth. There have been several anecdotal reports of uveal melanoma occurring in breast cancer families with mutations in the BRCA2 gene as well as one systematic study reporting BRCA2 mutations in UM. A single BRCA2 mutation, 6174 del T, occurs in about 1% of the Ashkenazic population and rarely in non-Ashkenazic. To assess the contribution of this germline mutation to uveal melanoma in Jewish Israeli patients, we tested this relationship through analysis of blood samples from a series of UM patients. A total of 153 cases (84 female, 69 male) were available for study, which represents 30% of all cases of UM diagnosed in Israel during the period 1984-1999 (82% for the period 1992-1999). Of the 143 UM patients for which a result could be obtained (4 due to refusals, 6 due to damage to the blood sample), 4 (2.8%, 95% confidence interval [CI] 0-5.6) carried the 6174 del T mutation. Assuming a population frequency of the mutation of 1% as estimated among Ashkenazic Jews in the United States, the probability of observing 4 or more carriers with the 6174 del T mutation, assuming no relationship between uveal melanoma and BRCA2, is 0.057. Although our study confirms the relationship between uveal melanoma and BRCA2, it is clear that the 6174 del T mutation accounts for only a small fraction of all Israeli UM cases. Therefore, BRCA2 mutations are likely to account for an even smaller proportion in populations with low frequencies of BRCA2 alterations. Copyright 2001 Wiley-Liss, Inc.
UI - 11821693
AU - Girkin CA; Goldberg I; Mansberger SL; Shields JA; Shields CL
TI - Management of iris melanoma with secondary glaucoma.
SO - J Glaucoma 2002 Feb;11(1):71-4
AD - Department of Ophthalmology, University of Alabama at Birmingham, Birmingham, Alabama 35203, USA.
UI - 11688673
AU - Kiratli H; Bilgic S; Gedik S
TI - Late normalization of melanocytomalytic intraocular pressure elevation following excision of iris melanocytoma.
SO - Graefes Arch Clin Exp Ophthalmol 2001 Sep;239(9):712-5
AD - Department of Ophthalmology, Hacettepe University School of Medicine, Ankara, Turkey. firstname.lastname@example.org
BACKGROUND: Melanocytoma of the iris is a rare tumor which may sometimes undergo necrosis that could result in elevated intraocular pressure through pigment dispersion. Only nine similar patients have been previously reported. METHODS: A 27-year-old woman presented with a dark brown iris stromal mass located between the 4 and 8 o'clock positions in the inferior quadrant. Her left visual acuity was 20/60. The tumor encroached on the lens and caused focal cataract. There was massive pigmented debris over the iridocorneal angle and the intraocular pressure was 42 mmHg. RESULTS: Fine needle aspiration biopsy did not suggest malignancy. A wide sector iridectomy was performed and histopathological examination of the lesion revealed melanocytoma of the iris. There was no ciliary body involvement. In the postoperative period, intraocular pressure, which persisted in the mid-twenties, was successfully lowered with topical dorzolamide and betaxolol drops. These drugs were continued for 2 years while the angle pigmentation gradually disappeared. There has been no documented glaucomatous damage to the optic nerve and visual fields. A year after the cessation of the drops, the left intraocular pressure stabilized and did not rise above 15 mmHg. Her left visual acuity remained 20/25. CONCLUSION: In contrast to previously reported cases, the normalization of intraocular pressure in this patient took 26 months, a period that could be associated with the self-clearing process of pigment from the iridocorneal angle. Close follow-up with medical treatment averted a pressure lowering surgical procedure in this case.
UI - 11725771
AU - Sarah; Srinivasan S; Sampat V; Kemp E; Parks S; Keating D
TI - Optical coherence tomography in the diagnosis of subclinical serous detachment of the macula secondary to a choroidal nevus.
SO - Ophthalmic Surg Lasers 2001 Nov-Dec;32(6):474-6
AD - Electrodiagnostic Imaging Unit, Tennent Institute of Ophthalmology Gartnavel General Hospital, Glasgow, Scotland.
We present a case in which optical coherence tomography (OCT) aided in the diagnosis of subretinal fluid (SRF) secondary to a choroidal nevus. A patient with a raised lesion above the left optic disc and distorted central vision was referred to our unit because of suspected choroidal melanoma. Fundus fluorescein angiography and slit-lamp investigation did not indicate any areas of SRF; however, OCT showed serous detachment in the macular area. The lesion was treated with laser photocoagulation and, when reviewed at four weeks post-treatment, repeat OCT scans showed that the area of SRF had cleared. In this case OCT was able to detect a subclinical serous detachment in the macula. This may have important implications in the monitoring of patients with small suspicious lesions.
UI - 11874758
AU - Chang EL; Rubin PA
TI - Bilateral multifocal hemangiomas of the orbit in the blue rubber bleb nevus syndrome.
SO - Ophthalmology 2002 Mar;109(3):537-41
AD - Ophthalmic Plastic, Orbital and Cosmetic Surgery Service, Harvard Medical School, Massachusetts Eye and Ear Infirmary, 243 Charles Street, Boston, MA 02114, USA.
PURPOSE: To report the clinical findings and surgical treatment of multifocal, bilateral orbital hemangiomas in a patient with the blue rubber bleb nevus syndrome, a rare vascular disorder characterized by distinctive cutaneous and visceral hemangiomas. DESIGN: Interventional case report. METHODS: Review of clinical findings, radiologic studies, and treatment of the patient. RESULTS: A 36-year-old female with multifocal, bilateral orbital lesions was seen with severe proptosis and decreased visual acuity of the left eye. Surgical debulking of multiple cavernous hemangiomas of the orbit resulted in decreased proptosis and an improvement in visual acuity. Subsequent histopathologic analysis and the results of a systemic workup revealing multiple, distinctive, cutaneous and visceral lesions were found to be consistent with the blue rubber bleb nevus syndrome. CONCLUSIONS: Although hemangiomas of the orbit are relatively common occurrences, multifocal, bilateral lesions may exist in the setting of underlying systemic hemangiomatosis. Bilateral or multifocal hemangiomas associated with cutaneous or visceral lesions should alert the clinician to the possibility of a number of syndromes that may have life-threatening consequences.
UI - 11805872
AU - Schmidt JC; Brieden-Azvedo S; Nietgen GW
TI - [Therapy of radiation resistant malignant uveal melanoma with endoresection by pars plana vitrectomy in two patients]
SO - Klin Monatsbl Augenheilkd 2001 Dec;218(12):800-4
AD - Zentrum fur Augenheilkunde, Philipps-Universitat Marburg. email@example.com
BACKGROUND: Since introduction of brachytherapy using (106)ruthenium plaque therapy for treating malignant choroidal melanomas a number of comparative studies have shown that survival rates are equal between patients who underwent radiation or enucleation. When radiation treatment fails, the patient might be determined to choose removal of the tumor by pars plana vitrectomy. PATIENTS AND METHODS: Between 1995 and 1998 a total number of 48 patients underwent (106)ruthenium plaque irradiation. In 9 patients tumor regression was not sufficient. Two of these nine patients decided explicitly against enucleation and choose to have the tumor removed from within the eye employing pars plana vitrectomy. RESULTS: Intraoperatively, the previously radiated melanoma was easily removed with the vitrectomy cutter without significant bleeding. Postoperative recovery was without complications. Silicone oil removal was carried out in one patient after one year. Vision 9 months and 2.5 years after surgery was 0.2 and 0.02, respectively. The cosmetic results with parallel axis of the globes were very good and one patient gained binocular vision. No recurrent tumor growth was seen so far. Histologically, no mitotic figures were found. CONCLUSION: Malignant choroidal melanomas can be safely removed by pars plana vitrectomy with stable intraocular pressure. Previous radiation therapy alters the tumor, inducing fibrous tissue growth and necrosis of tumor cells. These tissue types are not prone to bleed when they are resected by the vitrectomy cutter. Long time studies have to elucidate the difference in survival rates between patients undergoing enucleation of the eye and patients who chose endoresection of the melanoma by pars plana vitrectomy.
UI - 11805873
AU - Schmidt M; Maier M; Schmidt T; Klaehsen D; Merte RL
TI - [The treatment of a primary acquired melanosis of the conjunctiva with atypia by low dose of Mitomycin C 0.02 % - case report with follow up of 2 years]
SO - Klin Monatsbl Augenheilkd 2001 Dec;218(12):805-9
AD - Augenklinik rechts der Isar, Technischen Universitat Munchen.
BACKGROUND: About 70 % of the melanomas of the conjunctiva develop from an primary acquired melanosis (PAM) with atypia. This is the reason why excision, radiation, cryotherapy or a combination of them are proposed for therapy. But, if the acquired melanosis is very large, serious complications will be found with that treatment. PATIENT AND METHOD: A 65-year-old woman has shown a large melanosis of the conjunctiva of her left eye. Biopsies, taken from different sites of the conjunctiva, have confirmed the diagnosis of an acquired melanosis of the conjunctiva with atypia. This has been the reason for our Mitomycin C 0.02 % therapy for one week. It has been repeated once again after half a year. RESULTS: Atypia of the cells was shown in the upper cell layer of the conjunctiva after the first therapy. But after the second therapy no atypia of the cells was present within two years after the last treatment, examining several biopses. CONCLUSION: Looking for an effective treatment without complications, a new therapy with Mitomycin C has been introduced in the last years. As it is not yet clearly known how to use Mitomycin to have maximal effectiveness with minimal complications, the reports about doses and frequency are differing a lot in the literature and are more often considerably lying over the introduced effective dose of our case. If Mitomycin is used in that low dosage and in that special application as shown, it could be a very efficient therapy with less complications.
UI - 11801268
AU - Honavar SG; Shields CL; Singh AD; Demirci H; Rutledge BK; Shields JA;
TI - Eagle RC Jr Two discrete choroidal melanomas in an eye with ocular melanocytosis.
SO - Surv Ophthalmol 2002 Jan-Feb;47(1):36-41
AD - Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, PA 19107, USA.
We report a case of multifocal choroidal melanoma arising in an eye with ocular melanocytosis and review the pertinent literature. A 63-year-old Caucasian male with ocular melanocytosis in the left eye was found to have two discrete choroidal melanomas in the same eye. Histopathology of the enucleated eye confirmed the diagnosis of two discrete choroidal melanomas of mixed cell type within a region of choroidal melanocytosis. It is estimated that 1 in 160,000 patients with unilateral ocular melanocytosis may develop two uveal melanomas, based on the reported data. On the basis of random chance, patients with two melanomas in the same eye would be expected to have approximately 1000-fold greater likelihood of underlying ocular melanocytosis than the general Caucasian population. In conclusion, multifocal choroidal melanoma is rare and may be related to underlying ocular melanocytosis.
UI - 11815892
AU - Byrne SF; Marsh MJ; Boldt HC; Green RL; Johnson RN; Wilson DJ
TI - Consistency of observations from echograms made centrally in the Collaborative Ocular Melanoma Study COMS Report No. 13.
SO - Ophthalmic Epidemiol 2002 Feb;9(1):11-27
AD - COMS Echography Center, Mars Hill, NC 28754, USA.
PURPOSE: To describe the methods used by the Collaborative Ocular Melanoma Study (COMS) Echography Center for grading tumor echograms and to assess reliability of the grading system. METHODS: Tumor echograms were graded at the COMS Echography Center using a specific protocol. To assess consistency, a five-percent random sample of all echographic gradings received as of June 30, 1996 was selected by the COMS Coordinating Center for re-evaluation at the COMS Echography Center. The results were compared with original data. Agreement between the two sets of gradings was evaluated by calculating overall percent agreement, and by using the kappa statistic for categorical features and intra-class correlation coefficients (ICC) for continuous measures. RESULTS: Overall agreement between original gradings and regradings was high. Agreement, based on the kappa statistic, between the original grading and the regrading was classified as 'moderate,' 'substantial,' or 'almost perfect' for nearly every variable graded. Kappas ranged from 0.47 for extrascleral extension to 0.82 for tumor shape. Intra-class correlation coefficients were 0.99 or higher for tumor height measurements and for the clock hour designation of the tumor apex. The only variable graded by the COMS Echography Center that did not have good agreement was 'confidence in tumor measurement.' CONCLUSIONS: The level of agreement (after adjusting for chance agreement) ranged from 'moderate' to 'almost perfect.' Grading for 'confidence of tumor measurement' differed between the original grading and the regrading but there was little difference in the tumor measurements. The COMS Echography Center has demonstrated that its grading protocol is consistent over time.
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