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NR 10-12/2006
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Report of a case of
malignant melanoma of the limbus (30-thy years of observation)
Przypadek czerniaka rąbka
rogówki (30 lat obserwacji)
Dariusz Kęcik1, Monika
Turczyńska1, Iwona Świtka-Więcławska1,
Maja Marczewska2
1 Department of Ophthalmology Medical University of
Warsaw, Poland
Head: prof. nadzw. dr hab. Dariusz Kęcik
2 Department of Pathology Medical University of
Warsaw, Poland
Head: prof. dr hab. Aleksander Wasiutyński |
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| Summary: |
Purpose: To
describe a case of a malignant melanoma of the limbus.
Material and methods: A 49-year-old female
patient with a suspected malignant limbic melanoma in
the left eye. The first signs appeared in 1975,
manifested as reddening of the left eye on the temporal
side with markedly dilated conjunctival vessels. In
1993, the lesion became pigmented and nine years later
its progression was noted. In May 2003, lamellar
keratoplasty of the left eye was performed under general
anaesthesia.
Results: Histologic assessment – spindle cell
malignant melanoma. The entire tumour was resected and
the cosmetic result was very good. The results of
accessory diagnostic studies were within normal limits.
Over a 28-month follow-up period no local recurrence or
metastases to other sites were observed.
Conclusion: Patients with discoloration of the
conjunctivae should be regularly evaluated by an
ophthalmologist and an oncologist. |
| Słowa kluczowe: |
czerniak złośliwy rąbka,
knaloplastyka warstwowa, melanocyty. |
| Key words: |
malignant limbic melanoma,
lamellar keratoplasty, melanocytes. |
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Introduction
Malignant melanoma is a tumour arising from melanocytes, the
cells containing enzymes required to synthesize the pigment
melanin, which during embryogenesis displace from the neural
crest to the skin, mucous membranes, central nervous system and
the eye. About 10% of melanomas are localised in the eye. They
arise from the uveal melanocytes in the choroid (80%), the
ciliary body (12%), the iris (8%) or the conjunctiva (less than
1%), are the most common ocular malignancy in adults. The most
common sites within the conjuntiva are the lacrimal caruncle and
the semilunar fold. Malignant melanoma of the cornea, which most
commonly arises from the limbus or the adjacent bulbar
conjunctiva (1), is relatively rare.
Methods and material
A 49 years old Caucasian female patient was admitted to the
Department of Ophthalmology, Medical University in Warsaw, in
May 2003 with a suspected melanoma of the limbus in the left eye.
She was otherwise healthy. In 1975, when pregnant, she noticed
some reddening of the left eye on the temporal side with marked
dilatation of the blood vessels and sought an ophthalmologist’s
opinion. Since then she had been regularly evaluated by an
ophthalmologist. In 1993, the lesion (2x2 mm in size), became
pigmented. The patient did not give her consent to surgical
excision of the lesion and cryopexy was performed three times.
For the next nine years no progression of the lesion was
observed, but in 2002, the lesion was found to have enlarged
(2x3 mm), and become darker. B-mode ultrasonography (32 MHz) of
the left eye detected an echogenic homogenous lesion within the
limbus, affecting the corneal epithelium, which also involved
the bulbar conjunctiva, but did not invade the sclera. Another
B-mode ultrasonography performed six months later confirmed
progression of the lesion. Surgery was advised to which the
patient gave her informed consent. On admission: visual acuity
20/20 bilaterally, IOP 21 mmHg bilaterally. The right eye: the
anterior and posterior segments were normal. The left eye: a
pale eyeball, a network of dilated conjunctival vessels on the
temporal side, in the cornea, at the limbus an elevated,
vascularized, pigmented tumour measuring 2.7x6.5 mm was extended
outside the limbus and involved the cornea 2.5 mm from the
limbus. The remaining cornea and other structures of the eye
were normal, as was the posterior segment (Fig. 1A).
Lamellar keratoplasty of the left eye was performed under
general anaesthesia. After surgery, topical corticosteroids and
topical antibiotics were used. A histopathological examination
of the resected tumour was performed.
Results
In the early postoperative period the visual acuity was
20/30, the temporal aspect of the eyeball was deeply injected,
the graft in the temporal cornea as well as the own cornea were
transparent, the other segments were normal (Fig. 1B).
Histologic assessment: spindle cell malignant melanoma – pT1 pNx
pMx, Breslov 0.7 mm (tumour cells stained with S100 and HMB45).
Single tumour cells superficially invaded the cornea. The entire
tumour was excised with a margin of healthy tissue (Fig. 2A, 2B,
2C).
One month post surgery, the visual acuity was 20/20. The graft
became slightly opaque. After six months, extended network of
conjuntival vessels on the temporal side and the graft of
decreased transparency and with ingrowing blood vessels on the
limbic side could be seen. The corneal sutures were removed and
one month after that the cornea was transparent with subsiding
neovascularization on the temporal side (Fig. 1C). No recurrence
was found in 20-month follow-up (Fig. 1D).
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Discussion
Malignant melanoma of the cornea may arise from primary acquired
melanosis (PAM), a preexisting benign pigmented nevus or de novo
(2). Sudden appearance of one or more nodules in a so far flat
lesion and spread beyond Bowman’s membrane with involvement of
the corneal stroma, suggest malignant transformation of PAM (3).
In 20% of cases, malignant melanoma develops from a pigmented
nevus. Any lesion that increases in size, extending beyond the
limbus is suspected. Primary conjunctival melanoma usually
develops at the beginning of the fifth decade of life. In our
patient the abnormalities, such as conjuntival congestion and
dilated blood vessels were first noticed when she was in her
early twenties, while pigmentation was observed after 18 years
of follow-up. The standard treatment consists of surgical
excision of the tumour with an appropriate margin of healthy
tissue combined with cryo- or laser therapy. Pigmented tumours
limited to the epithelium may be treated by resection of the
epithelium with the lesion (3). A melanoma involving the
superficial layers of the corneal stroma may be excised using
superficial keratectomy combined with cryotherapy or lamellar
keratoplasty, which is a safer and less traumatizing method than
penetrating keratoplasty though technically more difficult. A
lamellar transplant allows a regular, smooth surface and
exercises a beneficial trophic effect on the cornea. The
cosmetic result is very good. When the melanoma invades the
cornea deeply, penetrating keratoplasty is required while
enucleation of the eyeball or orbital exenteration are the most
radical methods. The melanoma may spread haemato- or
lymphogenously or via the lacrimal duct into the nasal cavity
(2). When metastases are present, which may occur in any organ
(most commonly the lymph glands, brain, liver and lungs) (4),
palliative treatment and chemotherapy are used. In the case
presented in this report, the results of all accessory
diagnostic studies (NMR scan of the head, transaminase levels,
chest X-ray,) remained within normal limits. The essential
studies were performed every 6 months. Over a 20-months
follow-up period, no local recurrence or metastases to other
sites were observed.
Conclusion
The development of the condition over a long period time clearly
demonstrates that patients with discoloration of the conjunctiva,
should be regularly evaluated by an ophthalmologist and an
oncologist.
Referecnces:
1. Damato B. Ocular tumors: diagnosis and treatment. Butterworth
Heinemann, 2000, 1, 53-89.
2. Lommatzsch PK, Werschnik C. Malignant conjunctival melanoma.
Clinical review wiht recommendations for diagnosis, therapy and
follow-up. Klin. Monatsbl Augenheilkd, 2002, 219 (10), 710-721.
3. Paridaens ADA, Krikness CM, Garner A, et al. Recurrent
malignant melanoma of the corneal stroma: a case of black comea.
Brit.J.Ophthalmol., 1992, 76, 444-446.
4. Shields CL, Shields JA, Gunduz K, et al. Conjunctival
melanoma: risk factors for recurrence, exenteration, metastasis,
and death in 150 cansecutive patients. Arch. Ophthalmol., 2000,
118 (11), 1497–1507.
Praca wpłynęła do Redakcji 17.10.2005 r. (813)
Zakwalifikowano do druku 24.10.2006 r.
Adres do korespondencji (Reprint
requests to):
Prof. dr hab. n. med. Dariusz Kęcik
Katedra i Klinika Okulistyki I Wydziału Lekarskiego Akademii
Medycznej w Warszawie
ul. Lindleya 4
02-005 Warszawa |
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