Juniper Publishers-Journal of Ophthalmology
Abstract
The Ciliary body is an unusual location of uveal
melanomas and usually these kinds of tumors appear with a reduction of
vision due to the formation of sectorial cataract or retinal detachment
when the tumor spread to a posterior position. We present a 57 years old
woman with sudden and severe ocular pain. Ophthalmologic examination
showed a hyper mature cataract and superior displacement of the lens
with sectorial angular block. The intraocular pressure was 22 mmHg.
After papillary dilation aciliary body tumor was observed. The
ultrasound study and magnetic resonance imaging confirmed the diagnosis
of uveal melanoma.
Keywords: Ocular Pain; Melanoma; Ciliary Body; CataractAbbreviations: BCVA: Best-Corrected Visual Acuity; NMR: Nuclear Magnetic Resonance
Introduction
Uveal melanomas are the most frequent intraocular
primary tumors in adulthood. However, ciliary body location is very
uncommon regarding iris or choroidal melanomas. Furthermore, ciliary
body location gives symptoms later, which also gives a worse prognosis.
Materials and Methods
Materials and Methods
Biomicroscopy, dilated indirect ophthalmoloscopy, Goldmanntonomter,
B-Mode echography and ultrasound biomicroscopy were performed.
Subsequent RMN imaging was also obtained.
Case report
We report a case of a 57 years old woman who came to
the emergency department with a sudden and severe pain in her right eye.
The patient also related that she had had a decrease in her visual
acuity for last two months. She was a nonsmoker, but would occasionally
drink alcohol. Family history was unremarkable for malignancy. Medical
history only included controlled hypertension and cholesterolemia.
Best-corrected visual acuity (BCVA) at first was 20/200 in the right eye
and 20/30 in the left eye.
The examination of the right eye showed a brown
pigmented lesion in the lower region of iris, without satellites lesions
or papillary deformity. She also presented a hyper mature cataract
antero-superiorly displaced endangering the anterior chamber space and
the iridocorneal angle (Figure 1). The intraocular pressure was 22 mmHg
in the right eye and 16 mmHg in the left eye. After papillary dilation,
we found a pigmented tumoral mass behind the iris which was situated
between 4 to 6 hours and produced the dislocation of the lens (Figure
2).The retinal examination was of no value because of the presence of
the mature cataract. The ophthalmoscopic examination of the left eye was
normal.
Results
The study with B-Mode echography and ultrasound
biomicroscopy (OTI HF 35-50 Ultrasound System ®)
demonstrated a 15 X 10 mm tumor which came from ciliary
body and displaced the lens (Figure 3). The nuclear magnetic
resonance (NMR) with gadoline confirmed the existence of an
intraocular tumor with hyper intense signal in T1 and hypo
intense in T2 (Figure 4) with a size of 15 x 10 x 8 mm and
heterogeneous catchment of contrast (Figure 5). With these
results a ciliary body melanoma was suspiciously diagnosed.
The extensive study was negative and a brachytherapy with of
iodine 125 (I125) treatments was decided. Actually and after
cataract surgery our patient is asymptomatic and without signs
of recurrence.
Discussion
Ocular melanoma is the most common intraocular primary
tumor in adulthood, which mainly affects white males between
fifty and sixty years old. It represents 3.7% of cases of melanoma
and it is the second most usual location after cutaneous
melanoma [1]. Its incidence is nearby 4.3 cases per million
populations [2]. And its most common position is the uveal
tract, which is supposed the origin of the 82.5% of intraocular
melanomas [1]. Although inside this rate, ciliary body melanoma
only represents 12% of uveal melanomas its location is a factor
of poor prognosis [3].
The majority of these tumors are asymptomatic until
their
growth produces a decrease of visual acuity due to the formation
of a sectorial cataract or retinal detachment [2]. The presence of
dilated episcleral vessels near the tumor (sentinel vessels) is
also frequent [4]. Unusually these tumors may also produce an
increase in intraocular pressure because of the angular closing
by the pigment dispersion produced by myeloma [5] a uveitis
due to necrosis of tumor [6] or aleukocoria in children [7]. There
is no existing record of a case of ciliary body melanoma that
appears with sudden and severe eye pain without a significant
increase of intraocular pressure. In the case I am talking about
this circumstance happens as well as other manifestations
like the presence of a mature cataract, lens subluxation and a
sectorial angular closure.
The diagnosis of this pathology is clinical using the slit lamp,
accompanied by additional tests such as mode A ultrasound
(presence of kappa angle); mode B ultrasound or ultrasonic
biomicroscopy (the size, location and extent of tumor is
characterized) and orbital NMR (show tumor mass as a hyper
intense on T1 and hypo intense on T2) [8]. The confirmation of
the diagnosis is obtained by the histological study of the lesion.
Pathological study is also important to establish the
prognosis of these melanomas because there are some factors
that can add to a worse prognosis for this kind of tumors like
epithelioid cell line, high mitotic activity, necrosis of tumor,
lymphocytic infiltration or extra sclera extension [9]. Genetic
factors are other important prognostic. The mutation in genes 3
and 8 is related to poor prognosis in these tumors [10].
Regarding the treatment of these tumors, there are several
alternatives such as radiotherapy, brachytherapy or external
beam radiation; local excision (iridocyclectomy) or enucleation.
Depending on the size of the tumor and the presence or absence
of distant metastases we would choose a treatment or another.
Our patient has been treated with brachytherapy due to the
characteristics of the tumor and the absence of extraocular
disease.
Ciliary body melanomas have a rate of distant metastasis
of19% and 33% at 5 and 10 years respectively, being the most
frequent liver, lung or bones metastases [1]. This rate is the
highest of uveal melanomas, which makes the survival from these tumors, also lower [3].
Conclusion
In conclusion we suggest that sudden ocular pain is a very
unusual presentation of already unusual ciliary body melanoma.
The objective of this article is describing this way of atypical
debut. These tumors are the most metastatic inside the group of
intraocular melanomas. However the brachytherapy treatment
can be a good choice for this kind of tumors if we detect them
before extra ocular dissemination.
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