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OJHAS Vol. 8, Issue 4: (2009
Oct-Dec) |
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| Malignant Melanoma
of Nose and Paranasal Sinuses: 2 Case Reports |
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Sanjeev Bhagat, Saurabh Varshney, Rakesh Singh, Sampan Singh Bist, Nitin
Gupta,
Dept. of ENT, Himalayan Institute of Medical
Sciences, Swami Ram Nagar, Doiwala, Dehradun-248140, Uttarakhand, India |
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Address For Correspondence |
Assistant Professor,
Dept. of ENT,
Himalayan Institute of Medical
Sciences,
Jolly Grant, Dehradun-248 140
E-mail:
sbent224@gmail.com |
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Bhagat S, Varshney S, Singh R, Bist SS, Gupta N. Malignant Melanoma of Nose and Paranasal Sinuses: 2 Case Reports. Online J Health Allied Scs.
2009;8(4):13 |
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Submitted: Dec 4, 2009;
Accepted:
Apr 1, 2010; Published: Apr 30, 2010 |
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| Abstract: |
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Malignant
melanoma is one of the rare and highly aggressive diseases of the
sinonasal
cavity. High index of suspicion is required for diagnosis as the patient
usually presents with non specific signs and symptoms. In the natural
course of the disease, higher rate of loco regional recurrences and
distant metastasis are seen making the overall prognosis of disease
very poor. In reviewing the various treatment modalities used in the
past, surgical resection of the tumour with postoperative radiotherapy
is preferred one. Advances in surgery, radiotherapy and chemotherapy
don’t have any impact on improved survival, which remains poor in
this disease. We report two cases of malignant melanoma, which were
treated at our institute.
Key Words:
Malignant Melanoma, Nose, Paranasal sinuses |
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Malignant
melanoma
is rare and aggressive tumour of nose and paranasal sinuses with high
incidence of local recurrence and distant metastasis. They account
for less than 1% of all melanomas and less than 4% of sinonasal tumours.1
At intial presentation, the tumours are usually confined to the nasal
cavity.2 Diagnosis is often delayed because of onset of
symptoms is insidious and non specific. Early diagnosis is important
in management. Because of the rarity of disease, retrospective reviews
and case reports are important source of gathering information about
the course of disease, prognostic factors and response to treatment.
Out of various treatment modalities, which have been used in the past,
most of the surgeons prefer, surgical resection followed by
postoperative
radiotherapy.3 Survival rates are generally poor in spite
of aggressive treatment.
Case
I: A 50 year-old-female was referred to ENT OPD with history of nasal
obstruction and occasional epistaxis for the last 3 months. Earlier
patient presented to EYE OPD with the complaints of watering of left
eye for 3 months, swelling medial to the left medial canthus for 3
months.
On examination, there was epiphora of left eye, and a firm mass 1.0
X 1.5 cm medial to medial canthus causing lateral displacement of the
canthus. Best-corrected visual acuity of the left eye was 6/9. Slit
lamp examination showed a nuclear sclerosis of grade II and fundus was
normal. The patient was treated as a case of sub-acute dacryocystitis
for 1 month. On follow-up there was no improvement, hence, the patient
was referred to us for evaluation. On examination of face irregular,
elevated, tender swelling extending over the left side dorsum of nose
and extending to medial canthus area was seen. Anterior rhinoscopy
revealed
fragile soft tissue mass in the middle meatus area. Left side
of neck had a palpable lymph node 2 x 2 cm Ib (submandibular region).
Diagnostic nasal endoscopy revealed fragile pinkish vascular mass, in
the middle meatus area and septum, which bleeds on touch . Biopsy
from mass confirmed the diagnosis of malignant melanoma, while FNAC
from cervical lymph node was suggestive of amelanotic melanoma. Contrast
Enhanced Computed Tomography (CECT) of the sinuses (Fig.1)
revealed
soft tissue mass involving left nasal cavity, left ethmoid cell region
and causing erosion and destruction of lateral wall and protruding into
the left orbit. Patient underwent excision of naso-orbital tumor mass
by lateral rhinotomy approach with glabellar extension, and left side
selective neck dissection. Patient was advised postoperative
radiotherapy,
which patient did not take. After 4 months patient presented with
tonic-clonic
seizures. Visual acuity was reduced to 1/60. There was a firm, tender,
pigmented mass near the medial canthus 2 x 3 cm in size, proptosis,
inferolateral globe displacement and fundus examination showed disc
edema. CECT showed soft tissue mass in the ethmoid sinus extending into
the left orbit, invading the extraocular muscles, optic nerve and
maxillary
sinus with erosion of cribriform plate and going intracranially, thereby
suggesting recurrence of tumour. A craniofacial resection with left
orbital exenteration was done Patient was given postoperative adjuvent
chemotherapy (injection Dacarbamazine). Patient could tolerate only 2
cycles, before she expired.
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Fig.1 CECT
of nose and paranasal sinuses showing soft tissue mass involving left
nasal cavity, left ethmoid cell region and causing erosion and destruction of
lateral wall and protruding into the left orbit. |
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Fig.2 CECT
Scan Nose and PNS showing mass confined to the left nasal cavity, septum and
inferior turbinate |
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Fig.3 Microphotograph
(H/E - 40x) showing features consistent with Malignant Melanoma |
Case II: A
35-year-old female presented to ENT OPD with a 3-month history of
recurrent
epistaxis from right naris and gradually progressive nasal obstruction.
Anterior rhinoscopy revealed pinkish, friable, polypoidal mass involving
the right nasal cavity, septum, and inferior turbinate. Diagnostic nasal
endoscopy was done, findings were confirmed, and biopsy was taken.
Histopathology
confirmed the diagnosis of malignant melanoma. Metastatic work up was
within normal limits. CECT scan of nose and paranasal sinuses (Fig.
2) revealed enhancing soft tissue density mass lesion in right nasal
cavity, inferior turbinate and septum. The patient underwent excision
of the mass by lateral rhinotomy approach. Histopathology (Fig. 3)
of the excised mass was consistent with malignant melanoma. Patient
was given postoperative radiotherapy. Patient is under regular follow-up
for more than one year without any local recurrence and distant
metastasis.
In the head
and neck, the incidence of primary melanomas is 25-30%.4
However the incidence of mucosal melanomas arising from aerodigestive
tract varies from 0.4 to 4%, out of which nose and paranasal sinus is
the most common site.5,6 Nasal cavity is being the
commonest site of tumour presentation. Bridges et al2
reported
70% of melanomas start in nasal cavity and 30% from paranasal sinuses.
The incidence of the disease is roughly equal in both sexes, and
predominantly
affecting the age group over 60 years.7 This is not in
conformity
in one of our case as the disease was seen in early thirties. Clinical
presentation depends upon size and site of the lesion. Presenting
symptoms
include nasal obstruction, epistaxis, swelling of the nose, mass at
the vestibule, epiphora, diplopia and proptosis.7 Nasal
obstruction
with epistaxis are the two common symptoms with which majority of
patients
present.3,8 Despite its lower incidence these tumours invade
the orbit frequently because of easy access to the orbit from the
ethmoidal
sinus, given the thinness of lamina papaaracea.9 One of our
case presented with chief complaints of epiphora and swelling medial
to medial canthus and non specific complaints of nasal obstruction and
occasional bleeding. Patient was treated as a case of sub-acute
dacryocystitis,
and after persistence of symptoms after one month, the patient was
referred
to us for nasal endoscopy.
The presence
of positive lymph node and advanced clinical stage at presentation has
been implicated as poor prognostic indicators.10 This is
in conformity with one of our case where the patient presented with
early local and nodal metastasis. The primary treatment modality is
complete surgical excision with or without postoperative radiotherapy.2
Although radiotherapy alone has been shown to result in complete or
partial cure initially, neither radiotherapy nor chemotherapy, singly
or combined, has been shown to affect overall survival. Chemothearpy
should be reserved for patient with systemic disease. Temam et al11
reported that postoperative radiotherapy improved local control,
independent
of T stage, but distant metastasis free survival and overall
survival
were worse when compared with surgery only group. Conversely, Kingdom
and Kaplan12 reported that postoperative radiation therapy
was associated with better overall survival and better disease free
intervals. They recommended postoperative radiotherapy in all patients
and even those with negative surgical margin. Disease- related mortality
is associated with local or regional recurrence. The lung, liver, bone,
and brain are the most common site for metastasis.7 In
reviewing
the previous series on sinonasal melanomas high rates of local
recurrence
(31%-85%) and distant metastasis (25%-50%) and poor 5-year survival
rates (13%-45%) have been reported8. Close follow-up is
warranted
as local recurrence is common and resection of recurrent tumour may
result in longer survival.
- Manolidis
S, Donald PJ. Malignant mucosal melanoma of the head and neck: review
of literature and report of 14 patients. Cancer 1997;80:1373-86.
- Bridger AG, Smee D, Baldwin
MAR et al. Experience with mucosal melanoma of the nose and paranasal
sinuses. ANJ Surg. 2005;75:192-197.
- Dwivedi R, Dwivedi R, Kazi
R. Mucosal melanoma of nasal cavity and paranasal sinus: case report.
J Cancer Res Ther. 2008;4:200-202.
- Goldsmith HS, Melanoma:
An overview. CA Cancer J Clin 1979;29:194-215.
- McKinnon JG, Kokal WA,
Neifeld
JP et al. Natural history and treatment of mucosal melanoma. J Surg
Oncol.1989;41:222-5.
- Stern SJ, Guillamondegui
OM, Mucosal melanoma of the head and neck. Head Neck 1991;13:22-7.
- Sanderson AR, Gaylis B.
Malignant melanoma of the sinonasal mucosa: Two case reports and review.
ENT-Ear, Nose & Throat Journal. 2007;86:287-290.
- Dauer HE, Lewis JE,
Rohlinger
LA et al. Sinonasal melanoma: A clinicopathological review of 61 cases.
Otolaryngology-Head and neck Surgery: 2008; 238:347-352.
- Volpe NJ, Albert DM. Metastatic
and secondary orbital tumours. In Albert DM, Jackobeic FA, editors.
Principles
and practice of ophthalmology. 2nd ed. Phildelphia; W.B.
Saunders. 2000. pp 3218.
- Chang AE, Karnell LH, Menck
HR. The National Cancer Data Base report on cutaneous and noncutaneous
melanoma: A summary of 84,836 cases from the past decade. The American
college of Surgeons commission on Cancer and the American Cancer
Society.
Cancer. 1998;83(8):1664-78.
- Temam S, Mamelle G, Marandas
P, et al. Postaperative radiotherapy for primary mucosal melanomas of
the head and neck. Cancer 2005;103:313-9.
- Kingdom TT, Kaplan MJ.
Mucosal melanoma of nasal cavity and paranasal sinuses. Head Neck 1995;17:184-9.
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