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Letters
medial palpebral conjunctiva probably stems from the
embryologic origin of the normal caruncle from this topographic site.4,5
The major differential diagnostic consideration is a solid
caruncular dermoid, which is also present at birth. There is only
1 persuasive caruncular dermoid in the ophthalmic literature,6
and it adhered to the superomedial eyelid margin (where colobomas may also occur with dermoids7,8). In contrast, abnormal caruncular lesions always involve the lower eyelid. The
caruncular dermoid was distinguishable from remnants of the
normal caruncle; our patient’s megacaruncle completely occupied the locus destined for a normal caruncle. Microscopically, the caruncular dermoid possessed a keratinizing epidermis-like surface and dense, thick collagen in place of a
substantia propria.6 In contrast, the present lesion exhibited
a goblet cell–rich nonkeratinizing squamous epithelium with
pseudoglands of Henle and subepithelial, thin collagen strands
typical of the caruncular substantia propria.
Gregory J. Bever, MD
Frederick A. Jakobiec, MD, DSc
Pia R. Mendoza, MD
Mark P. Hatton, MD
Author Affiliations: Boston University School of Medicine, Boston,
Massachusetts (Bever); David G. Cogan Laboratory of Ophthalmic Pathology,
Massachusetts Eye and Ear Infirmary, Boston (Jakobiec, Mendoza); Department
of Ophthalmology, Harvard Medical School, Boston, Massachusetts (Jakobiec,
Mendoza, Hatton); Ophthalmic Consultants of Boston, Boston, Massachusetts
(Hatton).
Corresponding Author: Frederick A. Jakobiec, MD, DSc, David G. Cogan
Laboratory of Ophthalmic Pathology, Massachusetts Eye and Ear Infirmary, 243
Charles St, Ste 328, Boston, MA 02114 ([email protected]).
Published Online: October 10, 2013.
doi:10.1001/jamaophthalmol.2013.4401.
Author Contributions: Dr Jakobiec had full access to all of the data in the study
and takes responsibility for the integrity of the data and the accuracy of the data
analysis.
Study concept and design: Bever, Jakobiec.
Acquisition of data: All authors.
Analysis and interpretation of data: Bever, Jakobiec, Mendoza.
Drafting of the manuscript: Bever, Jakobiec.
Critical revision of the manuscript for important intellectual content: All authors.
Administrative, technical, or material support: Jakobiec, Mendoza.
Study supervision: Jakobiec.
8. Jakobiec FA, Pineda R, Rivera R, Hsu-Winges C, Cherwek D. Epicorneal
polypoidal lipodermoid: lack of association of central corneal lesions with
Goldenhar syndrome verified with a review of the literature. Surv Ophthalmol.
2010;55(1):78-84.
Extrascleral Spread of Choroidal Melanoma via
Tantalum Marker Suture Track
Proton beam irradiation allows globe-sparing treatment of
uveal melanoma with excellent local control rates.1 Recurrence after radiotherapy is low, ranging from 2% to 5%. Treatment first requires delineation of the tumor by placement of
tantalum markers that are sutured to the sclera.2 We report a
case of multifocal, extrascleral spread of choroidal melanoma along the suture track of a tantalum marker after proton beam therapy.
Report of a Case | A 65-year-old woman presented 17 months after proton beam irradiation for choroidal melanoma of the right
eye with multiple, pigmented, subconjunctival lesions highly
suspicious for recurrence with extrascleral extension. Review of outside records revealed that the original mass extended from the fovea supratemporally with a collar button
configuration, measuring 16 × 14 mm at the base and 9 mm in
height on B-scan ultrasonography. The patient underwent tantalum marker placement followed by proton beam therapy 3
weeks later. A total dose of 56 Gy (to convert to rad, multiply
by 100) was given in 4 fractions. Thereafter, the patient was
lost to follow-up.
Visual acuity on presentation to our institution was no light
perception OD. Darkly pigmented subconjunctival nodules
were apparent (Figure 1). A secluded pupil and vitreous hemorrhage precluded a fundus examination. B-scan ultrasonography demonstrated remnants of the primary tumor and an
additional mass posterior to the globe that was confirmed with
Figure 1. Clinical Photograph
Conflict of Interest Disclosures: None reported.
1. Jakobiec FA, Lam H, Bhat P, Pineda R. Non-syndromic supernumerary
caruncles causing ocular irritation after cataract surgery: a critical review of
caruncular dysgeneses. Am J Ophthalmol. 2010;149(3):398-404; e1-e2.
2. Luthra CL, Doxanas MT, Green WR. Lesions of the caruncle: a
clinicohistopathologic study. Surv Ophthalmol. 1978;23(3):183-195.
3. Zamir E, Banin E, Chowers I, Arnon N, Peèr J. Ectopic caruncle. Arch
Ophthalmol. 1999;117(10):1446-1447.
4. Nijhawan N, Morad Y, Seigel-Bartelt J, Levin AV. Caruncle abnormalities in
the oculo-auriculo-vertebral spectrum. Am J Med Genet. 2002;113(4):320-325.
5. Duke-Elder S, Cook C. Normal and abnormal development: embryology. In:
Duke-Elder S, ed. System of Ophthalmology. St Louis, MO: Mosby;
1963:234-235.
6. Ghafouri A, Rodgers IR, Perry HD. A caruncular dermoid with contiguous
eyelid involvement: embryologic implications. Ophthal Plast Reconstr Surg.
1998;14(5):375-377.
7. Baum JL, Feingold M. Ocular aspects of Goldenhar’s syndrome. Am J
Ophthalmol. 1973;75(2):250-257.
jamaophthalmology.com
A 65-year-old woman presented with multifocal, extrasceral spread of choroidal
melanoma in the right eye 17 months after proton beam therapy. Multiple
pigmented, subconjunctival lesions of the nasal globe are noted.
JAMA Ophthalmology December 2013 Volume 131, Number 12
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Letters
Figure 2. Histopathologic Sections of the Exenteration Specimen
A
B
A, Primary posterior necrotic tumor
with overlying tantalum marker
suture (hematoxylin-eosin, original
magnification ×10). Inset, Melanoma
cells seen around the suture material
(hematoxylin-eosin, original
magnification ×40). B, Posterior
extrascleral extension of the tumor
(hematoxylin-eosin, original
magnification ×2). Inset, Melanoma
cells along the suture material
(hematoxylin-eosin, original
magnification ×20).
orbital magnetic resonance imaging. Findings on liver function tests, magnetic resonance imaging of the head and neck,
positron emission tomography, and chest radiography were
within normal limits. Given highly probable extraocular extension with multifocality, the tumor board recommended exenteration.
Gross pathology showed a large, posterior, pigmented choroidal mass with multiple extrascleral satellite lesions on the
anterior aspect of the globe and one near the posterior aspect. Histopathology of the choroidal tumor demonstrated a
necrotic central core with a peripheral edge of viable melanoma cells. The extrascleral sites of melanoma were not contiguous to the primary choroidal mass. Direct extension
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through episcleral channels was not observed. Instead, melanoma cells were seen neighboring and within suture material
adjacent to the primary tumor site and at the site of posterior
extrascleral extension (Figure 2).
Discussion | Nearly half of recurrences of uveal melanoma are
at the margin of the initial mass, probably as a result of treatment planning errors.1 Furthermore, larger melanomas are
more likely to recur because of poorer radiosensitivity.2 Extraocular tumor spread is typically through anatomical conduits including aqueous channels, ciliary arteries, vortex veins,
ciliary nerves, and the optic nerve.3 To our knowledge, this is
the first report of extrascleral extension of melanoma along a
JAMA Ophthalmology December 2013 Volume 131, Number 12
Copyright 2013 American Medical Association. All rights reserved.
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Letters
suture track created from tantalum marker placement. There
are no reports of scleral perforation during tantalum marker
or plaque placement. However, it is known that scleral perforation can occur during strabismus (0.8% to 1.8%) and scleral
buckle surgery (2.5%).4,5 Presumably, a needle pass penetrated the tumor to provide an avenue for tumor cells to spread
beyond the highly localized treatment area of the proton beam.
This case demonstrates the vigilance that must be taken in tantalum marker placement, tumor delineation, and timely delivery of an adequate dose of radiotherapy.
Shilpa J. Desai, MD
H. Jane Kim, MD
Robert C. Kersten, MD
Michele M. Bloomer, MD
M. Reza Vagefi, MD
Analysis and interpretation of data: Desai, Kim, Bloomer.
Drafting of the manuscript: Desai, Vagefi.
Critical revision of the manuscript for important intellectual content: All authors.
Administrative, technical, or material support: Desai, Bloomer, Vagefi.
Study supervision: Kim, Kersten, Vagefi.
Conflict of Interest Disclosures: None reported.
1. Gragoudas ES, Marie Lane A. Uveal melanoma: proton beam irradiation.
Ophthalmol Clin North Am. 2005;18(1):111-118; ix.
2. Brockhurst RJ. Tantalum buttons for localization of malignant melanoma in
proton beam therapy. Ophthalmic Surg. 1980;11(5):352.
3. Coupland SE, Campbell I, Damato B. Routes of extraocular extension of uveal
melanoma: risk factors and influence on survival probability. Ophthalmology.
2008;115(10):1778-1785.
4. Mills MD, Coats DK, Donahue SP, Wheeler DT; American Academy of
Ophthalmology. Strabismus surgery for adults: a report by the American
Academy of Ophthalmology. Ophthalmology. 2004;111(6):1255-1262.
5. Kuchenbecker J, Schmitz K, Behrens-Baumann W. Inadvertent scleral
perforation in eye muscle vs retinal detachment buckle surgery. Strabismus.
2006;14(3):163-166.
Author Affiliations: Department of Ophthalmology, University of California,
San Francisco (Desai, Kim, Kersten, Bloomer, Vagefi).
Corresponding Author: M. Reza Vagefi, MD, Department of Ophthalmology,
University of California, San Francisco, 10 Koret Way, San Francisco, CA 94143
([email protected]).
Published Online: October 3, 2013.
doi:10.1001/jamaophthalmol.2013.5042.
Author Contributions: Desai had full access to all of the data in the study and
takes responsibility for the integrity of the data and the accuracy of the data
analysis.
Study concept and design: Desai, Kersten, Vagefi.
Acquisition of data: Desai.
Increased Fundus Autofluorescence Related
to Outer Retinal Disruption
Fundus autofluorescence (FAF) imaging is able to map metabolic changes at the level of the retinal pigment epithelium
(RPE) noninvasively in vivo. However, the observed autofluorescence signal is a summation of not only the autofluorescence originating from the RPE but also that from more anterior ocular structures including the overlying neuroretina.1
Figure 1. Multimodal Imaging of a 30-Year-Old Man With Multifocal Choroiditis and a 50-Year-Old Woman With Multiple Evanescent
White Dot Syndrome
A
Presentation
B
C
7 mo later
D
A-D, Multimodal imaging of a 30-year-old man with multifocal choroiditis in the
left eye. A fundus autofluorescence (FAF) image at presentation using the
Optos system showed multiple hypoautofluorescent spots and a peripapillary
zonal hyperautofluorescent area (A), colocalizing with an area of disruption of
both the ellipsoid and retinal pigment epithelium–photoreceptor interdigitation
zones in the corresponding spectral-domain optical coherence tomographic
(SD-OCT) image (B). A FAF image 7 months later (C) showed resolution of the
peripapillary zonal hyperautofluorescent area concomitant with near-complete
restoration of both the ellipsoid and the digitation zones in the corresponding
SD-OCT image (D). E-H, Multimodal imaging of a 50-year-old woman with
multiple evanescent white dot syndrome. A 30° FAF image using the confocal
scanning laser ophthalmoscope system showed multiple hyperautofluorescent
jamaophthalmology.com
E
Before bleaching
F
G
After bleaching
H
spots (white arrow) (E), which corresponded to areas of focal disruption (arrow)
of both the ellipsoid and retinal pigment epithelium–photoreceptor
interdigitation zones on a horizontal SD-OCT scan (F). After bleaching, the FAF
signal of the surrounding retinal areas increased more than the FAF signal of the
spots, resulting in a markedly decreased difference in autofluorescence level
between the pathological spots and the relatively normal-appearing
surrounding retinal tissue (G). However, on the corresponding SD-OCT images
before (F) and after (H) bleaching, the retinal structure looked identical. One
hour later, the hyperautofluorescent spots reappeared just as in E (image not
shown). The green arrows in A, C, E, and G indicate the levels of the SD-OCT
scans in B, D, F, and H, respectively.
JAMA Ophthalmology December 2013 Volume 131, Number 12
Copyright 2013 American Medical Association. All rights reserved.
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