Nasal mucosal melanoma presents usually with epistaxis, nasal obstruction and facial pain. However melanoma tends to give distant metastases at an early stage, having rare clinical presentations

Efstratiou, Ioannis

Konstantinidis, Iordanis

Nellas, Konstantinos

Printza, Athanasia

Zevgaridis, Alexandros

English

PubMed

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Case report Open Access
Nasal mucosal melanoma presenting as central type vertigo:
a case report
Konstantinos Nellas1, Iordanis Konstantinidis1*, Alexandros Zevgaridis1,
Athanasia Printza1 and Ioannis Efstratiou2
Address: 12nd Academic ORL Department, Papageorgiou Hospital, Thessaloniki, Greece and 2Pathology Department, Papageorgiou Hospital,
Thessaloniki, Greece
Email: KN - mpleka@yahoo.gr; IK* - jordan_orl@hotmail.com; AZ - azevgaridis@gmail.com; AP - nan@med.auth.gr; IE - jokons57@hotmail.com
*Corresponding author
Published: 5 May 2009 Received: 12 December 2008
Accepted: 11 March 2009
Cases Journal 2009, 2:7149 doi: 10.1186/1757-1626-2-7149
This article is available from: http://casesjournal.com/casesjournal/article/view/2/5/7149
© 2009 Nellas et al; licensee Cases Network Ltd.
This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0),
which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Abstract
Nasal mucosal melanoma presents usually with epistaxis, nasal obstruction and facial pain. However
melanoma tends to give distant metastases at an early stage, having rare clinical presentations.
We present a 74-year old female patient with symptoms of central type vertigo caused by brain
metastases. Clinical assessment for the detection of the primary site revealed a nasal mucosal
melanoma originating from the posterior end of the left inferior turbinate. The patient received a
combination of radio and chemotherapy being in relatively good condition 8 months later. This is the
first reported case of a nasal mucosal melanoma with vertigo as the first presenting symptom.
Introduction
Mucosal melanomas arising from the nasal cavity and
paranasal sinuses are rare and account for less than 1% of
all melanomas and less than 4% of all sinonasal tumours
[1]. The most frequent symptoms at first presentation are
epistaxis and unilateral nasal obstruction with a range of
85–90% followed by facial pain and facial deformity
usually in advanced cases [1]. Although the tumour has an
aggressive biologic behaviour, distant metastasis is not
frequent at the time of diagnosis accounting the 10% of
nasal melanoma cases [2]. We present the first reported
case in the literature of a nasal melanoma presenting as
central type vertigo.
Case presentation
This is a case study of a non-smoker 74-year old female
patient referred to ORL Department with a 2-months
history of vertigo. History and clinical findings indicated
the presence of vertigo with central type characteristics.
Specifically the patient had a feeling of unsteadiness
without spinning, absence of nausea and vomiting and a
direction-changing nystagmus. Otologic assessment had
normal findings and palpation of the neck did not reveal
nodal disease. Magnetic resonance imaging (MRI) of the
brain revealed 4 metastatic lesions. The location of
metastases was the cerebellum (Figure 1) and temporal
lobe on the left side and occipital and parietal lobe on the
right side. Interestingly a small hypodense lesion in T2
weighted sequences was also revealed on the posterior end
of the left inferior turbinate (Figure 2). Nasal endoscopy
showed a dark coloured mass originating from the
posterior end of the left inferior turbinate (Figure 3) and
a biopsy followed under local anesthesia. The histopatho-
logical examination of the nasal lesion biopsy revealed
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pleomorphic melanin pigmented cells (Figure 4A).
Immunohistochemistry showed that the tumour cells
stained positively for S-100 and HMB-45 (Figure 4B),
confirming the diagnosis of a malignant melanoma.
Further workup, included an MRI of the neck, abdomen
and chest, without evidence of other primary site or distant
metastasis. Thorough dermatological examination did not
reveal any site of primary cutaneous melanoma.
The presence of distant metastases and the absence of
nasal symptoms such as epistaxis were contra-indications
for surgical treatment. The patient followed a scheme of
radiotherapy with 3000 cGy whole brain and primary site
irradiation divided in 10 sessions followed by 3 cycles of
chemotherapy with Dacarbazine (850 mg/m2). The
patient 8 months after diagnosis still has no nasal
complains however she developed lung metastatic lesions.
Discussion
Nasal mucosal melanoma is a very rare malignancy with a
high tendency to develop both regional and distant
Figure 1.
(A) Sagittal and (B). Axial MRI T1 weighted sequence where
it is noted (black arrows) a metastatic lesion on the left side of
cerebellum.
Figure 2.
Axial MRI T2 weighted sequence in which a hypodense lesion
is seen on the posterior end of the left inferior turbinate
(white arrow).
Figure 3.
Endoscopic view of the left nasal cavity. A dark coloured mass
is seen originating from the inferior turbinate obstructing
partially the nasal choana.
Figure 4.
(A) Biopsy section showing nests of tumour cells with
brownish melanin pigment within the submucosa of the nasal
cavity. The arrowheads indicate the lining epithelia of the
tumour mass (H&E × 400) (B) Immunohistochemistry
positive stain of the tumor cells for HMB-45 marker × 400.
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Cases Journal 2009, 2:7149 http://casesjournal.com/casesjournal/article/view/2/5/7149
metastases. Amongst all melanomas, only 1.3% derives
from the mucous membranes. Head and neck mucosal
melanomas are approximately the half of them [1].
Both genders are affected equally, with the peak age
incidence between fifth and eighth decade [1]. The usual
clinical presentation of a nasal melanoma is this with nasal
symptoms such as unilateral nasal obstruction and epistaxis
(85–90% of cases) [1,2]. Other reported symptoms are
facial pain, facial deformity, secretory otitis media, diplopia
and dysphagia [3,4]. This is the first reported case to our
knowledge of a nasal melanoma without local or regional
symptoms and vertigo as the first presenting symptom.
If a balance disorder should be always investigated with
MRI of the brain is still debatable. The use of MRI in the
management of vertigo presents a low cost effectiveness
[5]. However if a thorough clinical examination indicates a
possible central nervous system (CNS) origin or reveal
suspected neoplastic lesions then the use of an MRI to
exclude among others and metastatic lesions is manda-
tory. In case of a CNS metastasis without nodal disease the
differential diagnosis should focus on neoplasms with
tendency for distant metastases such as mucosal melano-
mas of the head and neck.
The most frequent locations of sinonasal track melanomas
are the lateral wall and turbinates (39%), followed by the
septum (23%) and paranasal sinuses (22%) [4].
Nasal mucosa melanoma arises from the melanocytes that
have migrated during embryologic development from
neural crest to sinonasal mucosa. One third of these
lesions are amelanotic. The rest are usually rich in melanin
and appear pigmented (50–70%) [4].
Presence of melanin sets the pathologic diagnosis of
mucosal melanoma. Since melanin is not always present,
various markers are used to improve the accuracy of
diagnosis. The immunohistochemichal markers positive
for melanoma include vimentin (97.1%), S-100 protein
(91%), tyrosinase (78%) and HMB-45 (76%) [6]. In other
reports the HMB-45 marker is found to be even more
sensitive (98%) [7].
Opposing to squamous cell carcinoma, mucosal mela-
noma tends to develop distant metastases more frequently
to lungs and brain than cervical lymph nodes [8]. Sites of
nasal mucosal melanoma distant metastasis according to
their frequency are lung, liver, bone and brain [9].
The most fundamental treatment is wide resection of the
primary tumourwheneverpossible. Radiotherapy combined
with surgery is recommended in cases of local recurrence,
distant metastases or incomplete lesion removal [9],
The role of systemic therapy either chemotherapy or
immunotherapy still remains controversial [9]. Che-
motherapy should be utilized as an adjuvant therapy as
there is no evidence to be effective as the only modality of
treatment [9].
The elder patients present treatment dilemmas regarding
their prognosis, quality of life, risks of anaesthesia and
surgery, and prolonged postoperative care. Decisions with
respect to radical surgery in an elderly population must
encompass quality of life issues in addition to that of the
disease-free survival.
The prognosis of a nasal melanoma is poor, despite the
progress in the imaging techniques. The mean survival for
the mucosal melanoma at 5 years is reported 17.1%; with a
range of 0–48% [4]. Independent prognostic factors appear
to be the location, size, thickness, vascular invasion, positive
lymph nodes and development of distant metastasis of the
tumour [7,10]. Regarding the location some authors suggest
that nasal melanomas of the septumhave a better prognosis
than that of the lateral wall of the nose [10].
The development of distant metastases from the time of
diagnosis has a wide range (1–200 months) [1,2,8].
However the presence of distant disease is quickly
followed by death for most with a median survival in
several studies ranging from 3 to 6 months [1,2,4,9].
Conclusion
In conclusion the presented case except its rarity provides
information regarding the possible clinical presentation
with symptoms from the central nervous system of an
aggressive tumour such as the nasal mucosal melanoma.
List of abbreviations
ORL, Otorhinolaryngology; MRI, Magnetic resonance
imaging; CNS, Central nervous system.
Consent
Written informed consent was obtained from the patient
for publication of this case report and accompanying
image. A copy of the written consent is available for review
by the Editor-in Chief of this journal.
Competing interests
The authors declare that they have no competing interests.
Authors’ contributions
KNwas themajor contributor in writing themanuscript, IK
performed the clinical assessment, the endoscopic biopsy
and revised the manuscript, AZ performed the literature
review and contributed in writing the manuscript, AP gave
final approval and revised the article, IE performed the
histological examination and gave final approval.
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References
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Nasal mucosal melanoma presenting as central type vertigo: a case report

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