Mr. Bhandarkar Ramesh, a 56-year-old male, presented with pain and swelling on the right side of his nose and below his right eye for two months. Imaging showed a soft tissue mass in the right nasal cavity and paranasal sinuses that had destroyed bone and extended into the right orbit. Biopsy revealed the mass was a primary mucosal melanoma, which are rare but can originate from melanocytes in the nose and sinuses. The preferred treatment is wide local excision but prognosis is poor as these tumors are aggressive and can metastasize rapidly.

1. CASE 2
Dr Mohit Goel
JR 1
9/7/2012

2. HISTORY
 56 yrs male ( Mr Bhandarkar Ramesh )
presented with pain and swelling of right
lateral side of nose and right lower
periorbital area since 2 mnths. There is also
redness of right eye with headache since 2
mnths.

3.  Soft tissue density
mass is seen in the
right nasal cavity
causing infundibular
widening and
extending into right
maxillary antrum.
There is also
destruction of right
nasal turbinate.

4.  Extension of mass into
the ethmoid sinus on
both sides with right
side lamina papyracea
destruction and invasion
of intraorbital fat
causing right eye
proptosis.

5.  Cranially the mass
extends upto the
frontal and ethmoidal
sinuses on both sides
and destroys the
floor of anterior
cranial fossa on right
side, extending
intracranially into the
frontal region.

6.  The mass shows fairly homogenous enhancement on post contrast
images with few small non-enhancing areas within. A well defined
non-enhancing area within the right frontal sinus laterally could
represent a mucocele.

7. MRI
The mass appears
inhomogenously
hyperintense on T1 WI.

8.  It turns hypointense with
hyperintense areas
within, on T2W_FLAIR.

9.  A fairly defined non
enhancing area witihin
the right frontal sinus
laterally shows
peripheral enhancement
and could represent
mucocele.

10. MELANOMA
 Primary mucosal melanoma of the head and neck is an uncommon
neoplasm and accounts for 20% of all malignant melanomas. Within
the head and neck region, the most common site of origin is the nasal
cavity, where the anterior septum is most often involved, followed by
inferior and middle turbinate . The second most common site is the oral
cavity, where a predilection for the hard palate and maxillary alveolar
gingivae has been found.
 The peak age for mucosal melanoma is during sixth to seventh decade.
The majority of patients with nasal cavity primaries present with nasal
obstruction and epistaxis. Proptosis, diplopia, pain and facial deformity
are less common and are indicative of advanced disease.

11. Malignant melanoma is relatively rare
malignancy and constitutes approximately 1-2 %
of all malignancies arising in the body. 90% of
these occur in the skin. Primary malignant
melanoma involving the nasal and paranasal
sinus mucosa are rare, accounting for less than
1% of all melanomas and also carries poor
prognosis.
Nasal Malignant Melanoma arises from the
melanocytes that have migrated during
embryologic development from the neural crest
to the mucosa of the nose and sinuses.
The preferred treatment for Sino nasal mucosal
melanoma is wide local excision with tumor free
margin.

12. On CT
Melanomas shows bone remodeling , although element
of frank erosions may be present . They are highly
vascular tumor so they enhance well on post contrast
study.
On MRI
Melanomas have high T1W signal intensities primarily
because of the presence of haemorrhage and to a
lesser degree because of the paramagnetic melanin.

13. PNS radiograph shows homogenous opacity in the
right nasal cavity.

14. CORONAL CT shows the mass involving the
ethmoid sinus and extension into the orbit.
POST CONTRAST study shows intense
enhancement

15. CORONAL CT (BONE WINDOW)
shows bony
destruction.
AXIAL CECT showing the extent of mass.

16. The Differential Diagnosis of Nasal
melanoma includes-
Anaplastic carcinoma, Lymphoma,
Embroyonal rhabdomyosarcoma,
Esthesioneuroblastoma and
Extramedullary plasmocytoma.
Esthesioneuroblastoma (ENB),
also known as olfactory
neuroblastoma, is a rare
neoplasm originating from
olfactory neuroepithelium.
These tumors often display
varying biologic activity ranging
from indolent growth, with
patient survival exceeding 20
years, to a highly aggressive
neoplasm capable of rapid
widespread metastasis, with
survival limited to a few
months.
Esthesioneuroblastoma. Coronal CT scan of the orbits
and sinuses shows a large, enhancing, and expansile
mass occupying the ethmoid air cells that is invading the
cribriform plate and breaking through to the left anterior
cranial fossa.

17. Esthesioneuroblastoma. A 39-year-old man presented with 1 month of
decreased vision, left facial numbness, and swelling. Physical examination
demonstrated left-sided exophthalmos and blindness. He had also lost his
sense of smell. Contrast-enhanced T1-weighted MRI demonstrated a large
lesion that originated in the paranasal sinuses and extended through the
cribriform plate into the anterior cranial fossa.

18. Esthesioneuroblastoma
The MRI appearance is often that of a large, soft tissue,
dumbbell-shaped mass centered within the superior nasal cavity
and extending intracranially. The tumor is generally hypointense
on T1-weighted images and iso- to hyperintense on proton
density and T2-weighted images. Contrast enhancement is
usually mild to marked and can be uniform or mildly
heterogenous.
On CT, the tumour is iso- to slightly hyperdense to muscle and
enhances homogenously. CT is most useful in demonstrating
bone destruction. In addition, calcifications within the mass are
reported to be a relatively specific diagnostic indicator of
esthesioneuroblastoma.

19. Solitary extramedullary
plasmacytoma
 Extramedullary plasmacytomas are rare neoplastic lesions that
can appear in the head and neck. They are characterized by
monoclonal proliferation of plasma cells. The nasal cavity and
nasal septum are probably the most common locations.
It shows nonspecific CT and MR imaging features. However, features
that may suggest the diagnosis are a bulky soft-tissue mass or
infiltrative lesion. The tumour does not usually become disseminated,
but it may be locally aggressive and demonstrate marked
involvement and destruction of the adjacent structures.
SEP should be included in the differential diagnosis of a nasal tract
lesion because it has imaging findings similar to those of other more
common lesions such as nasopharyngeal carcinoma and lymphoma.

20. THANKU