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SINONASAL Pres Tari

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SINONASAL CARCINOMA

Dwi Utari Pratiwi


VISI Prodi Kesehatan THT-KL
» Program Studi berstandar global yang inovatif dan unggul,
serta mengabdi kepada kepentingan bangsa dan
kemanusiaan dengan dukungan sumber daya manusia yang
profesional dan dijiwai nilai-nilai Pancasila pada tahun 2020
MISI Prodi Kesehatan THT-KL
1. Meningkatkan kegiatan pendidikan, penelitian dan pengabdian
masyarakat berlandaskan kearifan lokal
2. Mengembangkan sistem tata kelola program studi Kesehatan
THT-KL yang mandiri dan berkualitas (Good Governance)

3. Membangun kemitraan dan kerjasama dengan rumah sakit yang


berkepentingan dalam rangka mendukung kegiatan pendidikan,
penelitian dan pengabdian masyarakat

3
INTRODUCTION
• Cancers of the nasal cavity and paranasal
sinuses are rare
• <1 % of all human malignancies and only
3 % of those arising in the head and neck
• Males : females = 2:1
• Often diagnosed in patients 50 to 70 years
of age
Incidence
• The highest incidence of sinonasal
malignancies found in Japan is 2-3,6 per
100.000 population per year
• In ENT Department in Medical Faculty of
University of Indonesia Ciptomangunkusumo
Hospital found in 10-15% of all malignant ENT
tumors.
NOSE (EXTERNAL)

(Drake, et al, 2007)


PARANASAL SINUSSES
8 paranasal sinusses, 4 in each side:
Frontal sinusses right and left
Ethmoid sinusses right and left
(anterior and posterior)
Maxillary sinusses right and left
(antrum highmore)
Sphenoid sinusses right and left
All of its coated by mucous which is
part of nasal mucous and containsof
air. Each of it has its own ostium as
an exit door.

(Drake, et al, 2007)


HISTOLOGY
Respiratory epithel consist of basal cells, goblet cells, and
columnar cells. It has a role in mucousproduction.

Epithel is supported by basal membrane which is


arranged by collagen and fibrous tissue (lamina propia)

Lamina propia is enriched by vascularization.

Bailey. 2015
• Superior part: anterior and
posterior etmoidalis artery
which is branch of ophtalmic
VASCULARIZAT I O N
art e ry and external carotid
artery.
• Inferior part : branch of
internal maxillary artery, like
greater palatine artery and
sphenopalatine artery which is
exit from sphenopalatine
foramen and get into nasal
cavity from the back of
posterior medial turbinate.
• Anterior part of septum : there
is anastomosis from the
branch of sphenopalatine
artery, anterior ethmoidal
aratery, superior labialis artery,
and greater palatine artery
which is called as
Kiesselbach’s area.

(Drake, et al, 2007)


Vascularization

(Drake, et al, 2007)


Innervation
Nervus olfactorius : carry sense of smell +
supply olfactory region of nose
Nervus of common sensation
n. ethmoidalis anterior : supply anterior and superior part of nasal cavity (lateral wall+septum
r. n. ganglion sphenopalatina : supply 2/3 posterior of nasal cavity (septum+lateral wall)
r. n. Infraorbita : supply vestibule of nose (medial+lateral side)
Nervus autonom
n. Vidianus : supply the nasal glands + control nasal secretion + supply blood vessels
Innervation
• Olfactory carry sheaths of dura,
arachnoid and pia with them into
the nose
• Olfactory nerve dendrits exit into
mucous membrane and forms
cillia which is called as Olfactory
hairs
• Olfactory center area cortex
cerebri:
 Lateral olfactory
centertemporal lobes cortex
 Medial olfactory
centerfrontal lobes cortex

(Drake, et al, 2007)


Lymphatic Drainage
• The anterior nose has the
same lymphatic drainage
as the external nose. These
tend to spread to the
submental or level I area.
• The posterior nose tends to
drain to the
retropharyngeal nodes as
well as the lateral pharyngeal
nodes, which eventually drain
into the level II.

(Standring, et al, 2008)


SINONASAL TUMOR
• The majority of these tumors are
squamous cell carcinoma,although a wide
variety of other malignancies including
sarcoma, adenoid cystic carcinoma,
lymphoma, melanoma, and olfactory
neuroblastoma
Etiology
• Not known certainly yet,
• But is suspected by exposure to some chemical
subtances and industrial material dusts:
o Nickel o formaldehyde,
o chromium, o isopropyl oil,
o vapor of mustard gas
o asbestos,
solvents used in
o wood dust, furniture and footwear
o skin industries and etc.
PATHOLOGY
Squamous Cell Carcinoma
• The most common malignancy of the sinonasal
tract.
• These tumors are more common in men than in
women with a peak incidence between 60 to 70
years of age.
• There is an association between sinonasal
squamous cell carcinoma and nickel exposure.
• Workers at a nickel refinery in Norway
developed squamous cell carcinoma at 250
times the expected rate, with a latent period
varying from 18 to 36 years.
• The most frequent site of origin for
squamous cell carcinoma is the maxillary
sinus followed by the nasal cavity, ethmoid
sinus, and sphenoid sinus
• Within the nasal cavity, squamous cell
carcinoma most commonly arises from the
turbinates, followed by the nasal septum,
floor, and vestibule.
• Approximately 80 percent of these tumors
are keratinizing carcinomas, with the
remaining 20 percent the non-keratinizing
subtype.
Pathology
STAGING
• Öhngren described a difference in the
prognosis of maxillary sinus malignancies
depending on their anatomic location
within the sinus.
• He divided the maxillary sinus into 2
halves by an oblique, imaginary plane
passing through the medial canthus of the
eye and the angle of the mandible
• Öhngren observed that this plane separates the
topographically more favorable tumors which are anterior
and inferior to the plane, from those of more unfavorable
character which are superior and posterior to the plane.
• Öhngren’s early insight into the topographical
importance of maxillary sinus carcinoma proved to be
highly significant.
• Cancers of the maxillary sinus originating anterior and
inferior to this plane (in the “infrastructure”), present
earlier with symptoms and are more amenable to
surgical resection with a better overall prognosis.
• In contrast, malignancies originating posterior
and superior to this plane, (in the
“suprastructure”), tend to develop symptoms
later in the course of the disease and are
challenging to resect surgically due to the
anatomic proximity of the pterygopalatine fossa,
infratemporal fossa, orbit, and skull base.
• These principles of maxillary sinus carcinoma
behavior continue to be reflected in the
American Joint Committee for Cancer (AJCC)
staging system for maxillary sinus carcinoma
Anatomical diagram illustrating Öhngren’s line.
DIAGNOSIS
Symptoms
• Nasal cavity and paranasal sinus
malignancies often do not cause
symptoms until they have expanded to a
significant size or have extended through
the bony confines of the sinus cavity.
• These tumors therefore tend to present at
a more advanced stage.
• Symptoms may initially include nasal
obstruction, epistaxis,pain, and episodes
of sinusitis.
• Tumor expansion inferiorly towards the oral
cavity may be associated with swelling of the
gingiva or palate with loose teeth, while orbital
invasion may lead to ocular symptoms such as
proptosis, diplopia, decreased acuity, and
restriction of ocular motion.
• Extension laterally into the pterygoid
musculature may cause trismus and deeper
invasion into the infratemporal fossa.
• Anterior extension through the anterior maxillary
wall may cause visible cheek swelling and
numbness from involvement of the infraorbital
nerve.
• In rare cases, posterior and superior extension
into the skull base, dura and brain may lead to
headache, cerebrospinal fluid leak, and central
nervous system deficits.
Physical Exam
• A complete head and neck physical exam
should always be performed, beginning with an
assessment of overall facial symmetry and any
areas of swelling or fullness.
• An eye examination should be performed with
attention to the range of extraocular motion,
visual acuity, pupillary response, and signs of
globe displacement.
• Proptosis may be evident from the anterior
displacement of the globe from a mass
impinging on the orbit.
• An ear examination should inspect the tympanic
membrane to assess middle ear aeration and to
evaluate possible eustachian tube dysfunction or
obstruction.
• Intraoral inspection of the hard palate,
gingiva, and anterior maxillary wall should
assess for fullness, signifying an
expanding mass within the maxillary sinus
or nasal cavity
• Mandibular excursion should be assessed
for trismus, a possible sign of pterygoid
musculature invasion.
• Cranial nerves should be tested with
particularattention to nerves I through VI
along with a general neurologic evaluation
• A thorough intranasal exam is essential,
using a flexible or rigid endoscope for
optimal visualization of the nasal cavity
and nasopharynx.
• Although gross tumor may be
obvious,subtle irregularities in the nasal
mucosal lining or fullness in the lateral or
superior nasal cavity wall should also be
carefully assessed.
• A thorough neck examination should be
performed to evaluate for palpable lymph
node metastases.
A hard palate and gingival bulge results from the inferior extension
of a left maxillary sinus mass into the oral cavity.
Imaging
• Imaging studies are an essential
component in the diagnosis, staging, and
follow-up of sinonasal malignancies.
• CT scans give a good initial overview of
the tumor’s location with excellent bone
detail
• Because the paranasal sinuses and nasal cavity
are mucosallined bony chambers, CT is helpful
in determining whether a tumor remains
confined within these natural boundaries or has
eroded through the surrounding bone.
• CT provides details of the extent of local bone
invasion, and is particularly useful in assessing
the lamina papyracea, orbital floor, fovea
ethmoidalis,cribriform plate, pterygoid plates,
hard palate, and skull base.
Axial CT scans accurately demonstrate the extent of bone destruction from a left-
sided maxillary
Coronal CT scans accurately demonstrate the extent of bone
destruction from a left-sided maxillary
• MRI allows a better distinction of tumor
from adjacent soft tissue.
• MRI is particularly useful for determining
invasion of the orbital contents, dura,
brain, and cavernous sinus.
• MRI may also be better for assessing
carotid artery invasion
• CT and MRI therefore complement one
another in the assessment of sinonasal
tumors.
• CT provides excellent bone detail,while
MRI offers better soft tissue imaging.
saggital MRI scan with gadolinium contrast shows a large ethmoid
malignancy completely filling both ethmoid sinuses and the right nasal
cavity, with extensiveintracranial extension.
coronal MRI scan with gadolinium contrast shows a large ethmoid
malignancy completely filling both ethmoid sinuses and the right nasal
cavity, with extensiveintracranial extension.
Biopsy
• An optimal procedure for biopsy of
sinonasal malignancies is an endoscopic
approach through the nares.
• This approach offers several advantages,
including excellent visualization, low
morbidity, and minimal alteration of the
tumor and its surrounding structures.
• Even small, lateral tumors within the
maxillary sinus may be accessible with the
creation of a middle meatal antrostomy,
visualization with a 30° or 70° endoscope,
and biopsy using a long,curved giraffe
instrument.
• In cases where a maxillary sinus tumor is
not accessible transnasally with the
endoscope, a canine fossa puncture can
be combined with endoscopic visualization
and biopsy.
• Open biopsy may rarely be necessary for
poorly accessible tumors, through either a
Caldwell-Luc approach or an external
ethmoidectomy(Lynch) incision.
Treatment
• Surgery
• More often along with other therapeutic
modalities such as radiation and
chemotherapy
• I.IDENTITY :
Name : Mrs. DP
Sex : Female
Age : 59 years old
MR : 01.82.64.82
II. ANAMNESIS
• MAIN COMPLAINT: Obstructed on right nose

THE PRESENT HISTORY OF THE DISEASE


Since one year ago the patient has been feeling nasal
obstruction on the right nose. The obstructed nose has been
felt continuously. The patient also complained of a thick yellow
discharge out of the right nose and smell. Complaints with
nosebleeds. There was a complaint of nasolalia. The patient
also complaining a facial pain. Nuisance impairment
complaint are denied. The patient had gone to the doctor but
the nasal obstruction complaint was not reduced. Double
vision is denied. Complaints of hearing loss, ringing ears and
throat are denied.
• THE PAST HISTORY OF THE DISEASE.
The allergic to the food (-).
History of the asthma (-), DM (-)
The patient had a history of lump surgery on the right
neck 2 years ago but no AP results

• THE FAMILY HISTORY OF THE DISEASE.


There is no family ill like the patient. Asthma (-), DM (-)
• RESUME :
 nasal obstruction on the right nose, felt
continuously
 thick yellow discharge and smell
 Nosebleeds
 nasolalia
 facial pain
III. PHYSICAL EXAMINATION.
• General status : good
Conciousness : compos mentis
Vital sign
BP : 130/80 mm Hg
Pulse : 80 x/m
RR : 20 x/m
T : 36,5 oC
III. Physical Examination
Ear examination : within normal limits
Anterior and posterior rhinoscopy : the bumpy
reddish mass in the right nasal cavity, easily bleed.

Oropharynx examination : within normal limits.


Indirect laryngoscopy : within normal limit
Head examination : lump on the right nose.
Neck examination : not palpable lymph node
enlargement and not palpable mass.
Patient Photos
Nasoendoscopy

Dextra Sinistra
• IV. Supporting examination
• MSCT Scan on
October, 12th 2017
CT-SCAN
• MSCT Scan on
October, 12th 2017
• MSCT Scan on
October, 12th 2017
Anatomical Pathology
• NO: AM-1709-147 on October 4, 2017
• Microscopic:
the preparation of swollen tissue with PMN
leukocyte infiltrate, found several groups of round
cells, oval, and atypical and pleomorphic spindles,
mitosis is sufficient, lymphocyte infiltrate and
leukocytes are numerous among atypical and
polymorphic cells.
Conclusion: Sinonasal carcinoma tumor poorly
differentiation until undifferentiated with supurativa
inflammation.
Thorax X-ray
MSCT thorax
V. Diagnosis:
Right Sinonasal Carcinoma (AP: SCC undiff)
T4aN0M1 stage IV C
VI. Therapy
– Media maksilektomi media + Tumor debulking
with lateral rhinotomy approach.
– Planned radiotherapy on December 6, 2017
VII. Problem :
Prognosis
Discussion
• Sinonasal malignancies are very difficult
tumors to treat and traditionally have been
associated with a poor prognosis.
• One reason for these poor outcomes is the
close anatomic proximity of the nasal
cavity and paranasal sinuses to vital
structures such as the skull base, brain,
orbit, and carotid artery.
Sites of Tumor Origin
TREATMENT GOALS AND ALTERNATIVE
FACTORS AFFECTING CHOICE
OF TREATMENT
• These decisions should include
careful consideration of many factors
including
(1) histology of the tumor,
(2) tumor stage,
(3) feasibility of a complete
surgical resection
(4) the patient’s underlying medical
condition,
(5) associated treatment risks and
morbidity,
(6) reconstructive options for the
restoration of form and function,
(7) socioeconomic issues,
(8) the surgeon’s technical ability,
(9) each patient’s personal wishes.
(American Academy of Otolaryngology -
Head and Neck Surgery American Head
and Neck Society, 2014)
(American Academy of Otolaryngology -
Head and Neck Surgery American Head
and Neck Society, 2014)
(American Academy of Otolaryngology -
Head and Neck Surgery American Head
and Neck Society, 2014)
(American Academy of Otolaryngology -
Head and Neck Surgery American Head
and Neck Society, 2014)
Surgery or Radiation Therapy Alone

• Early squamous cell carcinoma of


the maxillary sinus (T1) and
small, contained, anterior nasal
cavity malignancies may be
amenable to surgical resection
alone as definitive therapy.
• Radiation therapy alone for
localized (T1) squamous cell
carcinoma of the maxillary sinus
and nasal cavity may also be
effective.
• Radiation therapy alone for larger
(T2 to T4) squamous cell
carcinoma gives significantly
inferior results in comparision to
combination therapy.
• Several series have shown that
radiation therapy alone used for
all T stage lesions yields 5-year
survival rates of only 0 to 16
percent.
• Vestibular carcinomas have a
more favorable prognosis than
sinonasal malignancies.
• Primary radiation alone as external
beam therapy and/or brachytherapy
has been shown to be effective for T1
and T2 lesions, with a 94 percent 5-
year local control rate and a 94
percent 5-year causespecific survival
rate.
• T4 lesions are best treated with a
combination of surgery and radiation
therapy.
Surgery with Postoperative
Radiation Therapy
• Surgery combined with postoperative
radiation therapy is the standard treatment
for most sinonasal malignancies.
• Most squamous cell carcinoma of the
maxillary sinus (T2, T3, and most T4),
nasal cavity,and ethmoid sinus may be
treated by surgical resection followed by
radiation therapy if the tumor is confined to
an anatomic region that makes it
removable in its entirety.
• Massive parenchymal brain invasion,
extensive skull base invasion,
massive tumor volume with trismus,
and carotid artery invasion are
contraindications to surgical
resection.
• Localized anterior skull base invasion
does not necessarily preclude
consideration of surgery, as these
tumors may still be removed with
craniofacial resection.
• The advantages of postoperative
radiation therapy include no delay in
tumor resection, dissection through
normal tissue planes during the
surgery,improved postoperative
wound healing, and less dose
restriction.
• In addition, radiation therapy may be
more effective as a postoperative
treatment of microscopic disease,
rather than as an initial treatment for
massive, bulky disease.
Surgical Management of the
Orbit
• To resect involved periorbita and
preserve the orbital contents in cases
where there is no invasion of orbital
fat, orbital musculature, or
involvement of the orbital apex.
• The invasion of any of these
structures is an indication for orbital
exenteration.
Chemotherapy for Advanced
Tumors
• In attempts to improve the
survival rates and local control of
advanced sinonasal tumors,
chemotherapy has been
administered as an adjuvant
treatment in a wide variety of
methods.
Treatment of the Neck

• Clinically positive metastatic disease


in the neck is generally managed with
neck dissection, the type depending
on the extent and location of the
nodal metastases.
• Postoperative radiation therapy to the
neck is indicated for multiple positive
nodes, any single node> 3 cm in size,
or extracapsular spread.
• Some studies have supported the
use of elective neck irradiation for
maxillary squamous cell
carcinoma.
• To not treat the N0 neck electively
in cases of sinonasal squamous
cell carcinoma.
Radiation Therapy for Palliation
• Palliation is an appropriate treatment
goal for patients with massive
unresectable tumors, distant
metastatic disease, extensive medical
co-morbidities, and poor physical
constitution precluding more
aggressive therapy.
• Radiation therapy alone may be of
benefit in decreasing local morbidity
from advanced sinonasal tumors
Surgical Treatment
• Tumors originating in the
maxillary sinus are removed by
some form of maxillectomy.
• Several different subtypes of
maxillectomy have been
described, each characterized by
the extent of the maxilla resected
with tumor.
• The limited maxillectomy removes
primarily one wall of the maxilla, while
the subtotal maxillectomy removes at
least two walls, including the palate.
Total maxillectomy is a term reserved
for procedures resecting the entire
maxilla.
Anteroposterior views of a skull delineating the osteotomies required for
a medial maxillectomy.
oblique views of a skull delineating the osteotomies required for a medial
maxillectomy.
Lateral views of a skull delineating the osteotomies required for an
“infastructure” maxillectomy.
Palatal views of a skull delineating
the osteotomies required for an “infastructure” maxillectomy.
Oblique views of a skull delineating the osteotomies required for a total
maxillectomy.
Palatal views of a skull delineating the osteotomies required for a total
maxillectomy.
NONSURGICAL TREATMENT
Chemotherapy with Maxillary
Debridement
• In the 1970s several groups in Japan began
trying a combination of
chemotherapy,radiation therapy and routine
tumor débridement or
cryosurgery within the maxillary sinus.
• Difficulties of these routine débridements
included significant pain as well as risk of
injury to adjacent structures such as the orbit
and brain.
Selective Intra-arterial Chemotherapy
• The selective administration of
chemotherapy through intra-arterial
infusion offers the advantage of a
more direct drug delivery to the tumor
site at a higher local concentration
than can be achieved withsystemic
therapy.
Neoadjuvant or Concurrent
Chemotherapy
• Cisplatin and 5-FU are currently the
most effective chemotherapy agents
in the treatment of sinonasal
malignancy
• Their adminstration may be most
effective as neoadjuvant agents prior
to other treatment modalities, or
concurrently with hyperfractionated
radiation therapy.
SEQUELAE, COMPLICATIONS AND
THEIR MANAGEMENT
Surgery
• Complications which may accompany
surgical resection include intraoperative
or postoperative bleeding, wound
infection, cerebrospinal fluid leak, and
visual or orbital injury if orbital preservation
was planned.
• Visual complications including diplopia,
lacrimal duct dysfunction,ectropion, and
exposure keratopathy may occur in cases
of maxillectomy with orbital preservation.
Intraoperative photograph of the radial forearm free flap placed in a
right partial maxillectomy defect.
Postoperative intraoral view of a hard and soft palate reconstruction
using a radial forearm free flap.
Radiation Therapy
• The complications that may arise
from radiation treatment of the
paranasal sinuses can be quite
significant and are related to the
anatomic proximity of adjacent
structures.
• Soft-tissue complications may include
septal perforation, nasal cartilage
necrosis, fistula, epiphora, nasal
synechiae, nasal stenosis, trismus,
pituitary insufficiency, and brain
necrosis.
• Osteoradionecrosis, bone exposure,
hearing loss, and loss of dentition
may also occur.
Chemotherapy
• Systemic cisplatin toxicity most
commonly includes nausea and
vomiting, renal failure,
myelosuppression—with repeated
treatment cycles, alopecia, and
ototoxicity and neurotoxicity as a
cumulative effect.
• 5-Fluorouracil may cause
myelosuppression,mucositis, gastritis
and diarrhea.
Outcomes and Results of Treatment

• The prognosis for sinonasal


malignancies has remained poor for
the past several decades despite
refinements in both surgical technique
and radiation therapy.
• Predictor of poor prognosis is the T
category. For example, although the
overall total 5-year survival for patients
with paranasal sinus malignancy may
range from 40 to 50%, patients
categorized as T1 and T2 may have a 5-
year survival as high as 70%. Conversely,
patients with a tumor category of T3 or T4
commonly have 5-year survivals as low as
30%. Chalian AA, Litman D in
Ballenger and Snow, 2003
• This patient was included into stage IV C
criteria with T4aN0M1 and it made a
worse prognosis to the patient as stated
before was as low as 30% of 5-year
survival. T4 lesions have been divided into
T4a (resectable) and T4b (unresectable),
leading to the division of stage IV into
stage IVA, stage IVB, and stage IVC.
• Overall 5-year survival rate in stage I, II
and III tumors 50% while stage IV tumor
by 20%.
CONCLUSION
• We reported a female, 59 years old patient
with a diagnosis of Right Sinonasal
Carcinoma (AP: SCC undiff) T4aN0M1
stage IV C.
• In this patient, has been done therapy
Media maksilektomi + Tumor debulking
with a lateral rhinotomy approach
Thank you
Suggestions please
Adenocarcinoma
• Exhibit a male predominance with a
peak age incidence between 55 and 60
years.
• Adenocarcinomas tend to arise
superiorly in the sinonasal region and
commonly involve the ethmoid sinuses.
• There is a significantly higher incidence
of adenocarcinomas in workers exposed
to wood dust particles
• Sinonasal adenocarcinomas may assume
three basic histologic forms:
papillary,sessile, and alveolar-mucoid.
• Although one pattern may dominate a
particular tumor, a combination of all three
types also may coexist.
• The papillary form is the type associated
with woodworkers and tends to be more
localized with a better prognosis.
• In contrast,the sessile and alveolar-mucoid
forms are locally more aggressive with a
greater metastatic potential and carry a
poorer prognosis.
Adenoid Cystic Carcinoma
• Minor salivary glands lining the
sinonasal tract may give rise to
malignancies of salivary origin.
• Adenoid cystic carcinomas account
for 5 to 15 percent of sinonasal
malignancies and occur equally in
both sexes with a peak age incidence
between 40 and 60
years.
Mucosal Melanoma
• Melanomas constitute about 3.5
percent of all neoplasms in the
sinonasal tract, and orignate from
neural crest-derived melanocytes
present in the mucosa and
submucosa.
• The nasal cavity is more
commonly affected than the
paranasal sinuses.
• Although some melanomas may
appear heavily pigmented,others
may be non-pigmented and
appear pink or tan in color;
melanin may or may not be
present in these tumors.
• Histologically, sinonasal
melanomas are quite varied, with
both epithelioid and spindle cell
features.
• As with all mucosal melanomas,
the prognosis is quite poor with
local recurrence and distant
metastases ocurring in up to two-
thirds of patients within 1 year.
Olfactory Neuroblastoma
• Olfactory neuroblastoma, also
known as
esthesioneuroblastoma,is a tumor
of neural crest origin derived from
olfactory epithelium.
• These tumors arise from the
superior nasal cavity and typically
form a mucosacovered, soft mass
with a congested appearance.
• These tumors occur with equal frequency
in males and females with a bimodal age
distribution; one peak occurs in a younger
population aged 11 to 20 years, and the
second peak in an older group aged 51 to
60 years.
• The histologic appearance is
marked by a characteristic rosette
pattern of round cells with fibrillary
material.
• Electron microscopy and special
stains for neurofibrils are required
to make the final diagnosis.
• A staging system proposed by
Kadish - categorizes
• stage A as tumor confined to the
nasal cavity,
• stage B as extension to the
paranasal sinuses, and
• stage C as extension beyond,
including cranial cavity, orbit, or
with distant metastases.
• The majority of these tumors are locally
aggressive and tend to invade adjacent
structures such as the orbit and cranial
cavity.
• Regional lymph node metastases may
occur in 20 to 40 percent of cases.
• Prognosis is dependent on disease extent
at presentation.
Sinonasal Undifferentiated
Carcinoma
• Sinonasal undifferentiated carcinoma
is a highly aggressive, rapidly growing
malignancy that frequently involves
multiple sinuses and the nasal cavity.
• Histologically, sinonasal
undifferentiated carcinoma is marked
by pleomorphic cells arranged in
sheets, nests, and trabeculae with a
high nuclear tocytoplasmic ratio.
• These tumors may be
histologically confused with
olfactory neuroblastoma, although
the distinction is important
because of their different clinical
behaviors.
• The poor prognosis of sinonasal
undifferentiated carcinoma is
related to its propensity for
extensive local invasion.
Other
• Other malignancies which may arise
in the sinonasal tract include a variety
of lymphomas and sarcomas.
• Kraus reviewed non-squamous cell
malignancies of the paranasal
sinuses and identified the most
common histologic sarcoma types as
fibrosarcoma,rhabdomyosarcoma,
and osteogenic sarcoma.
• The lymphoma type most
commonly involved was
histiocytic,large cell lymphoma.
• Minor salivary gland carcinomas
other than adenoid cystic
carcinoma and adenocarcinoma
may also arise in the sinonasal
tract, such as mucoepidermoid
carcinoma.

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