National Cancer Institute®
Last Modified: March 1, 2002
1
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.
2
UI - 11815331
AU - Shields CL
TI -
Conjunctival melanoma.
SO - Br J Ophthalmol 2002 Feb;86(2):127
3
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. gerasimos.anastassion@uni-essen.de
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.
4
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.
iscovich@netvision.net.il
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.
5
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.
6
UI - 11587196
AU - Anonymous
TI -
Similar survival rates for ocular melanoma therapies.
SO - Optom Vis Sci 2001 Sep;78(9):626
7
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. hayyamk@ttnet.net.tr
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.
8
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.
9
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.
10
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.
jc.schmidt@gmx.de
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.
11
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.
12
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.
13
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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