1. Presenter: Dr. Durga Department of Pathology, RIMS,
Imphal.
2. Anatomy & Histology
3. Roof of nasal cavity contains olfactory mucosa. In infants
extends to the mid portion of nasal septum and onto superior
turbinate. In adults its replaced by respi epithelium. Olfactory
mucosa has 3 types of cells- 1.Olfactory nerve cells(bipolar) 2.
sustentacular cells or supporting cells 3. Basal cells.
4. Classification of nasal cavity and PNS malignancies Benign
Malignant 1.Epithelial Schnederian papilloma Squamous papilloma
Minor salivary gland tumors 2.Neuroectodermal Ectopic pitutary
adenoma Paragangliomas Meningioma 3. Mesenchymal Lobular Capillary
Hemangioma Solitary fibrous tumor Fibrous histiocytoma Fibromatosis
Osteoma Lipoma, Ameloblastoma Inderminant for malignancy/low grade
malignant potential Sinonasal type hemangiopericytoma Epitheloid
haemangioendothelioma 1.Epithelial Squamous cell carcinoma
Keratininsing Non keratinising Variants of SCC Sinonasal
undifferentiated carcinoma Adenocarcinoma Intestinal type Non
intestinal type Minor salivary gland neoplasms. 2. Mesenchymal
Angiosarcoma Mucosal malignant melanoma Olfactory neuroblastoma NHL
Extra osseous Ewings MFH chondrosarcoma 3. Secondarytumors.
6. Schneiderian papilloma (sinonasal papilloma) Schneiderian
membrane; ectodermally derived lining of sinonasal tract. Represent
less than 5 % of all sinonasal tumors. Affects most commonly 5th
-8th decade and rare before 40 years Usually unilateral.
Association with HPV 6 & 11. Symptoms: airway obstruction,
epistaxis, asymptomatic mass and pain 3 morphologically distinct
benign papillomas 1. Fungiform/exophytic/septal papilloma 2.
Inverted papilloma 3. Oncocytic/cylindrical/columnar
7. Exophytic variant Papillomatous proliferation of epithelial
cells along with mucocytes seen along delicate fibrovascular core.
Bland looking epithelial cells with retention of polarity,
scattered mucocytes seen along the surface.
10. Squamous papilloma Most common benign neoplasm of UADT-oral
cavity & larynx. Less often involves nasopharynx & nasal
vestibule. Gross: Exophytic warty or cauliflower like tumors
ranging in size from few mm to 3 cm M/E: Benign squamous epithelium
in multiple finger like projection with prominent fibro vascular
core. Lacks dysplasia Treatment: Surgical excision is cuartive with
no risk of malignant transformation.
11. Sinonasal Squamous cell carcinoma
12. SCC is the most common type of malignant epithelial
neoplasm of sinonasal tract. Constitute app 3% of all head and neck
malignant neoplasms. Men> Women in 6th -7th decade. Risk
factors: Nickel exposure, textile dust, smoking , preexisting
schederian papilloma. Site(order) Maxillary antrum, nasal cavity,
ethmoid sinus, sphenoid and frontal sinuses. Clinical presentation:
Facial asymmetry, unilateral nasal obstruction epistaxis, pain with
persitent purulent rhinorrhea non healing ulcer.
13. Variants: 1.Exophytic or papillary 2.Verrucous 3. Spindle
cell squamous carcinoma 4. Basaloid squamous cell carcinoma
5.Adenosquamous carcinoma Gross: Growth varies acc to variants
& is well circumscribed, with an expansile growth.
14. Histologically keratising(MC) and non keratinising
subtypes. Keratinising: Keratinising variant characterised by
presence of keratinization and intercellular bridges. Graded as
well, moderate and poorly differentiated . Desmoplastic response is
typically found in stroma. Pic 4A.18
15. Non keratinising SCC Exophytic or endophytic growth
pattern. M/E: Nest of squamous epithelium with broad
interconnections having smooth borders.
16. Papillary Uncommon but distinct subtype showing solitary
lesion with an exophtyic growth pattern (2mm- 4cm) Hpe:filiform
growth with finger like projections & identifiable
fibrovascular core. Squamous epithelium cytologically malignant d/f
it from papillomas.
17. Verrucous Highly differentiated variant of SCC with locally
destructive but not distantly metastatic. Mc affecting oral cavity
larynx. SNT is least coomon Dyson et al- protein product of HPV can
bind the retinoblastoma gene product, removing the regulatory block
of cell cycle. Gross: tan or white, warty, fungating or exophytic,
firm to hard mass measuring upto 10 cm.
18. Histologically bland squamous cell proliferation with
uniform cells lacking dysplastic features. Retention of polarity
with no atypia. Mitotic figures can be seen only in basal layer not
elsewhere. Marked surface keratinisation and broad or bulbous rete
pegs pushing downwards into stroma. Viral associated Kolicytic
changes are seen.
19. Spindle cell squamous carcinoma/ sarcomatoid carcinoma
Occurs in sinonasal tract & nasopharynx. Appears as fungating
ulcerated masses. More aggressive and radiorestitant tumors.
21. Basaloid squamous cell carcinoma High grade SCC affecting
hypopharynx less frequently sinonasal tract. Etiology: Excessive
alcohol &/or tobacco use. Presents as a uni nasal mass lesion .
Gross: firm to hard tan white masses often with central necrosis
measuring upto 6cm Histologically: Infiltrating tumor showing
varied patterns solid ,trabecular, gland like or cystic. Foci of
squamous differentiation along with the presence of basaloid cell
component which are small closely apposed cells with hyperchromatic
nuclei, scanty cytoplasm & marked mitosis. Prone to have early
metastasis to regional lymphnode even to visceral locations.
22. WHO defined a carcinoma arising in the nasopharyngeal
mucosa that shows light microscopic or ultrastructural evidence of
squamous differentiation MC in southeast asia and north africa.
Etiological factors: multifactorial 1. strong association with EBV
nonkeratinising and un differentiated variant. 2. Diet (salted fish
high in nitrosamines) 3. poor hygeine and environmental pollutants.
4.HLA A2, HLA B17, HLA Bw46, HLA BW 58 marker for genetic
susceptibility. Non random deletions and rearragment of chromosome
3
23. Genomic wide studies have shown multiple chromosomal
abnormalities with mutation in oncogenes and tumor supressor genes.
Inactivation of p16 TSG- most common alteration. Ras associated
domain family1A. Clinical presentation: Asymptomatic cervical neck
mass(Post cervical triangle) Nasal obstruction, discharge epistaxis
with pain serous otitis media, otalgia . Site of occurrence: Fossa
of rosenmuller on the Lateral wall of nasopharynx is the most
common superior posterior wall.
24. Gross appearance Varies from a mucosal bulge with an intact
epithelium to clearly demonstrable infiltrative mass M/E: +/-
evidence keratinization Most common
25. Keratinising SCC of Nasopharynx
26. Non keratinising: (12%) shows little to no evidence of
keratinising Tumor growth pattern- stratified or pavemented
arrangment showing sharp delination from surrounding stroma
27. Undifferentiated This type represents around 60% NPC; can
be seen in pediatric age group also. 2 patterns of growth are seen
1. Regaud type characterised by well defined aggregates of tumor
cells surrounded by fibrous tissue and lymphoid cells 2. Schminkee
type-Neoplastic epithelial cells grow diffusely & are closely
intermingled with inflammatory cells. Misnomer- Lymphoepithelioma
Tumor cells characteristically show large & vesicular nuclei
with a smooth outline & a single eosinophilic nucleoli
30. Prognosis Radiotherapy is the treatment of choice. Stage at
presentation is the most important prognostic factor 5 yr survival
rate if presented stage I- 98% stage II-95% stage III-86% stage
IV-73% Worse prognosis is seen with 1. Marked anaplasia 2. High
cell proliferation rate 3. Lack of lymphocyte infiltrate. 4. High
dendritic S 100 positivity 5. Her2neu expression. Other Important
factors: Age Gender Presence of keratinisation Lymphnode
status.
31. 10-20% of all primaries SNT 2 types : Intestinal type &
Non intestinal type Intestinal: they are malignant epithelial
glandular tumors of SNT histologically resembling intestinal
adenocarcinoma or an adenoma. Males>females of age 50-70 years
Etiology: Occupational wood workers Gross: Exophytic growth with
readily identifiable mucinous qualty.
32. Low grade nasopharyngeal papillary adenocarcinoma Low grade
tumors arising from naspharyngeal surface epithelium showing
glandular differentiaion Indolent biologic behaviour. No gender
prediliction occuring in age group ranging over 20-70 years Site:
posterior nasopharyngeal wall. Gross: Exophytic, papillary, nodular
or cauliflower like with a soft to gritty consistency measuring
upto 4 cm
33. Histologically: Unencapsulated and have papillary and
glandular growth pattern. Cells vary from pseudostratified columnar
to cuboidal with eosinophilic cytoplasm Nuclei round to oval with
vesicular to optically clear chromatin
34. Sinonasal undifferentiated carcinoma Original defintion by
Freirson et al as a high grade malignant epithelial neoplasm of the
nasal cavity and PNS of uncertain histiogenesis with or without
neuroendocrine differentiation BUT with out evidence of squamous or
glandular differentiation Rare tumor less than 100 cases Male
predominance over a wide range of group 30-60 years. At
presentation SNUC are extensive & involves multiple sites nasal
cavity or PNS
35. Presents clinically most commonly as unilateral mass,
epistaxis, proptosis, diplopia, facial pain & cranial nerve
involvement Typically pt presents with multiple symptoms of short
duration. Histologically: Hypercellular proliferation with varied
growth pattern- trabecular, sheet like, ribbons, solid,
lobular.
36. Cellular infiltrates composed of polygonal cells composed
of medium to large sized , round to oval, hyperchromatic to
vesicular nuclei with varying amount of eosinophilic cytoplasm
37. IHC is non contributory to diagnosis: Epithelial mucin ()
P63 (v) Cytokeratin (v) Synaptophysin chromogranin S100 (r)
Viamentin & smooth muscle markers (-)
38. Hemangiomas Lobular capillary hemangiomas formerly known as
Pyogenic granuloma Benign vascular tumors primarly affecting skin
and mucous membrane. Affects both the genders equally. Age group:
40-50 years Location: Nasal septum over Littles area, Turbinates.
Presents clinically as epistaxis, painless obstructive mass.
Pathogenesis unclear but an association noted with pregnancy and
OCP usage. They regress after parturition
39. Gross: smooth lobulated, polypoid red mass measuring upto
1.5 cms Microscopically: submucosal vascular proliferation arranged
in lobules and clusters composed of central capillaries and
tributaries. Staghorn appearance Prominent endothelial cell lining
and show endothelial tufting. Surrounded by granulation tissue
& mixed chronic inflammatory cells.
40. Pic 4A.7
41. Nasopharyngeal angiofibroma Rare Benign mesenchymal tumor
accounting less than 1% of all head and neck tumors Tumor
exclusively affects male thought to be hormonally driven. Age group
second decade and also older ages. Site: Roof of nasal cavity
nasopalatine foramen. Symptoms: Nasal obstruction, epistaxis Late-
facial swelling and deformity, nasal dicharge headache diplopia
hearing defect Patients with FAP are 25 times more susceptible to
have angiofibroma- APC B catenin mutations are noted . Radiography
Holman miller sign. Gross: Sessile lobulated masses occasionally
polypoid or pedunculated.
42. Microscopic: unencapsulated & characterised by
fibrocollagenous stromal proliferation with admixture of variable
sized vascular spaces. Vascular component varies from small to
large sized, staghorn to inconspicuos due to marked compression by
the stromal fibrous tissue. Stroma- fibrous tissue with fine or
coarse collagen fibres & may be focally myxoid. Mitotic figures
are rare. Nuclear pleomorphism and MNGC can be seen. Tumors of
longer duration tend to be more fibrous & less vascular. IHC:
Smooth muscle actin; viamentin(+) Testosterone receptor (+) ER PR
(-)
43. pic4A.8
44. The major complication with angiofibroma is excessive
bleeding, recurrence, extension beyond nasopharynx. Nasal Biopsies
of such young male with facial deformities should be performed with
extreme caution because of the risk of excessive bleeding.
Recurrence rate varies 6 to 24%.more with the one tumor showing
intracranial extension. Good prognosis no risk of malignant
transformation.
45. Olfactory Neuroblastoma Malignant neuroectodermal neoplasm
arising from olfactory membrane of sinonasal tract. Olfactory
placode tumor, esthesioneuroblastoma, esthesioneuroepithelioma.
Basal cells are mitotically active proposed to be putative cell of
origin. Uncommon -2% SNT tumors Wide age range from 3 years to 9th
decade Clinical: anosmia headache along with nasal obstruction.
Site: cribriform plate Radiographically opacification of SNT with
calcification(speckeled pattern)
46. Gross: Glistening, mucosa covered, soft, polypoid mass
varying from a small nodule
47. 4A.31 Neuron specific enolase S-100 synaptophysin
chromogranin. High proliferation index
48. Histologically:
49. Complete surgical excision Craniofacial resectio involving
removal of cribriform plate Followed by full course radiotherapy
Prognosis depends on clinical staging defined by kadish Stage
Extent of tumor 5 year survival A Confined to nasal cavity 75-91% B
Involving nasal cavity + 1/more PNS 68-71% C Extension tumor beyond
sinonasal cavities. 41-47%
50. Mucosal Malignant melanoma Neural crest derived neoplasm
originating from melanocytes & show melanocytic
differentiation. 15-20% of all MM arise in head n neck out of which
80 % are of cutaneous origin Of the non cutaneous head n neck
melanomas most often have occular origin & few in SNT
Men>women of age group 60-80 occuring MC on nasal septum
Symptoms: airway obstruction, epistaxis Gross: polypoid or sessile,
brown , black, pink or white friable mass. ulceration is most
common
51. Histologically: Pleomorphic epitheloid or spindled cells
arranged in either solid, organoid, nested patterns cells are round
to oval with high N C ratio having vesicular to hyperchromatic
nuclei
52. Diagnosis usually confirmed by IHC: HMB 45(+) and S 100(+)
EMA (-) cytokeratin (-)
54. Sinonasal polyp Non neoplastic inflammatory swelling of
sinonasal mucosa Commonly seen in adults over 2o years age and less
common in children less than 5 years.(except the child with CF)
Location lateral wall of nose or ethmoid recess. Symptoms nasal
obstruction rhinorrhea headaches. Antrochoanal polyp: 3-6% of all
polyps. More common in men than in women usually single unilateral
lesions with associated nasal obstruction. Gross: Soft, fleshy
polypoid lesions with a mucoid appearance.
55. Microscopically polyps are composed of loose edematous
stroma with large number of eosinophils in allergic polyps.
Basement membrane is thickened and stroma is edematous AND there is
an absence of seromucinous glands. Mixed chronic inflammatory cell
infiltrate.
56. Sinonasal & nasopharyngeal infectious disease.
Clinically simulate neoplasia Fungal disease aspergillosis
&rhinosporidiosis bacterial rhinoscleroma mycobacterial disease
ICP- viral disease HSV CMV Produce ulcerative mass over nasal
cavity and nasopharynx
57. Rhinosporidiosis Large globular cystic spaces surronding
fibro vascular stroma PAS sporangia
58. HIV infection of waldeyers tonsillar tissue Presents
clinically as enlargment of lymphoid tissue of waldeyers ring
including tonsils and adenoids Clinically presents as unilateral
swelling. Histomorphology vary with progression of disease Early
lesion: Florid follicular hyperplasia with follicular fragmentation
& follicle lysis with areas of follicular involution Presence
of monocytoid B cell hyperplasia Paracortical & interfollicular
zone expansion with immunoblasts plasma cells Intrafollicular
hemorrhage Multi nucleated gaint cells clusters adjacent to
tonsillar crypt.
59. In advanced stages there is effacement of nodal
architecture, loss of normal lymphoid cell population with
replacement by benign plasma cell and presence of increased
vascularity. Less Multinucleated gaint cell in advanced
stages.
60. Heterotopic CNS tissue Glial heterotopia or nasal glioma
Considered to be a variant of encephalocele in which communication
to CNS has closed. Usually present at birth or with in first year
Site: around nasal cavity; ethmoid sinus, nasopharynx Subcutaneous
lesion over bridge of nose appears blue or red mass Clinical
symptoms: Nasal obstruction, respiratory distress epistaxis with
DNS Histologically: Astrocytes and neuroglial fibres ass with a
fibrous vascularized connective tissue. On long standing fibrous
stroma obscure the astrocytes Surgery is the treatment of choice
& curable.