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Malignant melanoma of the conjunctiva with intraocular extension: a
clinicopathological study of three cases.Graefes
Arch Clin Exp Ophthalmol. 2007
Mar;245(3):431-6.
BACKGROUND:
Malignant melanoma of the conjunctiva is a rare tumour with an
unpredictable behaviour, characterised by the high risk of local
recurrence and metastatic spread. Intraocular extension of a
conjunctival melanoma is rare. We present three cases of primary
conjunctival melanoma with intraocular extension. METHODS: Three
patients presented with primary conjunctival melanomas, which either
arose at or later involved the limbus on a background of primary
acquired melanosis. Despite adequate primary local excision and
adjuvant chemotherapy, all three patients experienced several
recurrences, requiring further surgery. Two of the patients had
excision of the tumour that required superficial keratectomy. One of
these patients then required two further procedures in which the
anterior chamber was breached. RESULTS: All three patients ultimately
required enucleation or exenteration to control local disease. All
three specimens showed intraocular extension. CONCLUSIONS: The
management of conjunctival melanoma can be difficult. In addition to
local recurrence and metastases, limbal melanomas may rarely show
intraocular extension, particularly if surgery to excise the tumour
requires the removal of Bowman's membrane. Regular follow-up of these
patients is mandatory.
Amelanotic
conjunctival melanoma.Cutis.
2006 Jun;77(6):377-81.
Conjunctival
melanoma is a rare condition of the eye pigment predominantly
affecting white adults. We describe a 32-year-old white man with an
amelanotic malignant melanoma of the conjunctiva that is not
associated with primary acquired melanosis (PAM) or melanocytic nevus.
The patient presented with a 2-year history of nonpigmented
vascularized nodules of the right eye. Results of hematoxylin and
eosin (H and E) staining of the lesion showed an invasive nodule with
vertical spreading, invasion of the substantia propria corneae, and
ulceration. S100 protein was expressed in the cells of the invasive
nodule. HMB45 protein was highly positive in the melanoma cells. The
de novo amelanotic malignant melanoma of the conjunctiva we describe
is an extremely uncommon tumor mainly affecting white adults.
Malignant melanoma of
the conjunctiva.Cancer
Control. 2004 Sep-Oct;11(5):310-6.
BACKGROUND:
Conjunctival melanoma is a relatively rare ocular malignancy with
substantial associated morbidity and mortality. METHODS: More than 100
articles on conjunctival melanoma were reviewed, including most of the
relevant recent publications cited in a current MEDLINE search. The
author's experience with conjunctival melanomas is also incorporated
in this review. RESULTS: Recognition of their precursor lesions at an
early stage is important. Staging of the disease by sentinel lymph
node biopsy is now advocated in some centers. Surgical excision with
adjuvant cryotherapy and alcohol corneal epithelialectomy is usually
effective in eradicating most of these lesions. Extensive cases of
flat primary acquired melanosis with atypia may be managed with
mitomycin C. Multifocal and advanced melanoma, especially in cases
showing intraocular or orbital invasion, may require exenteration
and/or radiotherapy to adequately extirpate the neoplasm locally.
However, systemic metastases already may have occurred in these
patients with advanced disease. CONCLUSIONS: Conjunctival melanoma is
a condition of concern because of its rarity and lethal potential.
Advances in the understanding and management of this neoplasm have
markedly reduced the mortality and possibly the morbidity associated
with this malignancy.
Malignant
conjunctival melanoma. Clinical review with recommendations for
diagnosis, therapy and follow-up.:
Klin
Monatsbl Augenheilkd. 2002 Oct;219(10):710-21.
BACKGROUND:
Malignant conjunctival melanoma is a rare disease with an incidence of
0.03 - 0.08. This tumour is potentially lethal, even after prompt and
proper treatment, especially after delayed onset of therapy.
Conjunctival melanoma arises from primary acquired melanosis (PAM),
from a preexisting nevus or "de novo" without any precursor at all. In
contrast to uveal melanomas, conjunctival melanoma metastasizes via
the ipsilateral lymph nodes and in rare cases through the lacrimal
duct into the nasal cavities. RESULTS: Removing the local tumour with
preservation of visual functions and avoidance of metastases is the
therapy of choice. Excision alone is followed by a high rate of
recurrence. To minimize local recurrence rate surgical excision should
be combined with an additional procedure such as cryotherapy,
irradiation, or local chemotherapy with MMC. Surgical technique is
characterized by a so-called "no-touch" method avoiding any direct
manipulation of the tumour to prevent tumour cell seeding into a new
area. The behaviour of conjunctival melanomas is individually
unpredictable. Prognostic factors are tumour size and tumour location.
Tumours growing extralimbal especially at the fornix, plica and
caruncle have a significantly poorer prognosis than limbal tumours. In
our own patients the 5-year, 10-year, and 15-year cumulative
melanoma-specific survival rate was 84.4 %, 77.7 %, and 75.0 %,
respectively. Up to now there is no effective treatment of the
metastatic disease. CONCLUSION: In all cases with pigmented lesions of
the conjunctiva exclusion of a malignant melanoma has to be the first
aim. A patient suffering from a conjunctival melanoma should be
referred to an ophthalmo-oncological center for proper treatment. An
indefinite follow-up including photodocumentation is necessary since
the rate of recurrence is high. An international prospective study
would be worthwhile to answer open questions and to develop new kinds
of treatment of this potentially fatal tumour.
Corneally
displaced malignant conjunctival melanomas.
Ophthalmology.
2002 May;109(5):914-9.
PURPOSE: To
characterize and classify malignant conjunctival melanomas with
exclusively corneal invasive growth. DESIGN: Population-based,
nationwide retrospective cross-sectional study. PARTICIPANTS: Patients
with primary malignant conjunctival melanoma diagnosed between 1967
and 2000 in Finland. METHODS: On the basis of all available clinical
and histopathologic data of tumors diagnosed during the study period,
malignant conjunctival melanomas that first demonstrated invasive
growth on the cornea without evidence of conjunctival tumors other
than primary acquired melanosis were identified, their prevalence
calculated, and their characteristics reviewed. On the basis of these
cases and literature data, a classification for "corneal melanoma" was
developed. MAIN OUTCOME MEASURES: Frequency and type of corneal
involvement, recurrence, and survival. RESULTS: Patients with
exclusively corneal invasive tumor accounted for 5% (95% confidence
interval, 1-12) of 85 consecutive primary conjunctival melanomas. Two
were separated from the limbus by clear cornea (type I), and two
paralleled but did not invade the limbal conjunctiva (type II). Two
were associated with clear evidence of primary acquired melanosis.
None of the tumors recurred after local excision, and no metastases
were observed during a median follow-up of 2 years 5 months (range, 1
year 8 months-7 years 10 months). CONCLUSIONS: Primary malignant
conjunctival melanomas can grow on the cornea without conjunctival
involvement other than acquired melanosis. They are easily removed and
do not cause lymphatic metastases. The term "corneally displaced
malignant conjunctival melanoma" would best describe their supposed
conjunctival origin and actual corneal location.
Malignant
melanoma of the conjunctiva with intraocular extension.Arch
Ophthalmol. 2000 Apr;118(4):557-60.
Intraocular
extension of a malignant melanoma of the conjunctiva is a rare entity.
A 75-year-old woman underwent repeated surgery after receiving the
diagnosis of a multilocular recurrent malignant melanoma arising from
a primary acquired melanosis. Treatment included 2 lamellar
sclerokeratectomies and percutaneous radiotherapy. Five years after
initial surgery, intraocular extension of the melanoma was observed,
and enucleation was performed. Findings from histopathological
examination revealed a malignant melanoma occupying part of the
ciliary body, the trabecular meshwork, and the iris. Eyes with
recurrent malignant melanoma of the conjunctiva should be carefully
monitored for intraocular extension. Deep excision of conjunctival
melanoma, including lamellar sclerokeratectomy, may abolish the
natural barrier against intraocular extension of malignant melanomas
of the conjunctiva.
Conjunctival
amelanotic malignant melanoma arising in primary acquired melanosis
sine pigmento.Ophthalmology.
1998 Jan;105(1):191-4.
BACKGROUND:
The authors describe an amelanotic malignant melanoma of the
conjunctiva in association with primary acquired melanosis (PAM) sine
pigmento, and highlight the clinical and pathologic features of this
rare entity. METHODS: Histopathologic and immunohistochemical studies
were performed on a conjunctival tumor in a 54-year-old white woman.
STUDY DESIGN: Case report. RESULTS: Histopathologic examination
revealed an invasive amelanotic melanoma of the conjunctiva, with
anterior orbital extension arising from intraepithelial dysplastic
melanocytes that lacked melanin pigment (PAM sine pigmento). Both the
malignant melanoma cells and the intraepithelial dysplastic
melanocytes in the areas of PAM exhibited S-100 and HMB-45 positivity.
The patient underwent an orbital exenteration that disclosed tumor
within the anterior orbit inferiorly. CONCLUSIONS: Amelanotic invasive
malignant melanoma can arise in association with PAM sine pigmento, as
seen in our patient who had orbital invasion necessitating
exenteration. This aggressive form of conjunctival melanoma is often
associated with a poor prognosis and risk of metastatic disease.
Absence of conjunctival pigmentation in PAM sine pigmento prevents
early clinical detection of this variant of PAM. This lack of
pigmentation also makes clinical diagnosis virtually impossible, and
diagnosis can only be established histopathologically. Awareness of
this nonpigmented variety of PAM is crucial for early recognition and
appropriate management of the associated melanoma.
Prognostic
factors in primary malignant melanoma of the conjunctiva: a
clinicopathological study of 256 cases.Br
J Ophthalmol. 1994;78(4):252-9.
A series of
256 consecutive cases of invasive primary conjunctival malignant
melanomas was examined to identify clinical and histopathological
prognostic factors. The follow up period varied between 0.3 and 45.9
years (mean 9 years, median 6.3 years). The 5 year survival rate was
estimated at 82.9%, the 10 year survival rate at 69.3%. Multiple
regression analysis with the Cox proportional hazards model was used
to assess sex, age, and a number of baseline features of conjunctival
malignant melanoma as possible prognostic factors influencing melanoma
related mortality. In assessing each potential prognostic factor, the
effects of all other factors were taken into account in the modelling
process. Tumours in unfavourable locations--that is, those involving
the palpebral conjunctiva, fornices, plica, caruncle, and lid margins,
were associated with 2.2 times higher mortality compared with (epi)bulbar
melanomas. Patients with mixed cell type tumours had about three times
higher mortality compared with those with pure spindle cell melanomas,
and histological evidence of lymphatic invasion by tumour cells was
also a prognostic feature, carrying a fourfold increase in the death
rate. Multifocal tumours were associated with a fivefold increase in
mortality among those with tumours in favourable (epi)bulbar
locations, but were not prognostic in patients with melanomas in
unfavourable sites. The death rate was significantly higher in those
with initial tumour thickness of more than 4 mm, but only among
patients with unfavourably located melanomas. Sex, age, and clinical
origin of the tumour (primary acquired melanosis, pre-existing naevus,
or de novo) were not useful prognostic indicators in this study.
Clinical
predictive factors for development of recurrence and metastasis in
conjunctival melanoma: a review of 68 cases.Br
J Ophthalmol. 1993 Oct;77(10):624-30.
Sixty eight
cases of histologically proved conjunctival melanoma were reviewed in
order to determine the clinical factors that were predictive of local
recurrence and distant metastasis. All patients were treated with
surgical excision and most had supplemental cryotherapy. The mean
follow up was 7.5 years. Histopathologically, the conjunctival
melanoma arose from primary acquired melanosis in 56%, from naevus in
26%, and de novo in 18%. Of the 68 patients, 38 (56%) developed at
least one local tumour recurrence and 22 (32%) developed more than one
recurrence. The method of initial treatment and the eventual
development of metastasis were the two parameters statistically
associated with tumour recurrence. Those patients treated initially
with tumour excision alone had a statistically significant higher
recurrence rate than those treated initially with excision and
supplemental cryotherapy (p = 0.001). Fourteen patients (21%)
developed metastasis and the mean period between treatment and
metastasis was 3.6 years. Twelve (18%) died from metastatic melanoma
with a mean interval of 4.4 years from the time of initial surgery
until death. The only clinical parameter that was statistically
associated with distant metastasis was local tumour recurrence (p =
0.015). Based on these observations, the authors make recommendations
regarding the treatment of conjunctival malignant melanoma. It appears
that initial complete excision of the tumour with supplemental
cryotherapy offers the patient the best chance of remaining free of
recurrence and metastasis.
Recurrent
conjunctival melanoma with neuroidal spindle cell features.Ophthalmology.
1987 Jan;94(1):56-60.
A 66-year-old
white woman had had a 20-year history of flat primary acquired
melanosis involving the left inferior forniceal and palpebral
conjunctiva. Over the ensuring 12 years, the patient experienced
multiple recurrences of invasive malignant melanoma that emerged from
the progressive primary acquired melanosis. Two of these recurrences
were composed of nonpigmented spindle cells, and in the most florid
invasive malignant melanoma that developed, the spindle cells formed a
nodule 7.5 mm thick. The spindle cells were organized into fascicles
and small bundles, the latter separated by a loose stroma that was
devoid of mucopolysaccharides. The fascicular and neuroidal features
in this case were sufficiently well developed to suggest the incorrect
diagnosis of a neural tumor or a neurofibroma. However, the presence
of intraepithelial atypical melanocytes at the edge of the spindle
cell lesion, the absence of mucopolysaccharides in the stroma, the
mitotic activity, and the absence of intercellular reticulin fibers
favored the diagnosis of a spindle cell invasive malignant melanoma.
This morphologic variant of conjunctival melanoma is compared with
related cutaneous lesions of melanoma featuring a spindle cell
population.
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