Sinonasal & Nasopharynx Flashcards

1
Q

√List 9 Environmental risk factors for sinonasal malignancies

A
  1. Hardwood dust - Mahogany
  2. Nickel-refining processes
  3. Leather tanning
  4. Mineral oils
  5. Industrial fumes - Formaldehyde, Chlorophenol, Chromium
  6. Isopropyl oils, Lacquer paints, Soldering and welding, radium dial
  7. Painting
  8. Asbestos
  9. Cigarette smoke
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2
Q

√Outline a complete differential diagnosis of a sinonasal neoplastic lesion

A

BENIGN EPITHELIAL
1. Vestibular papilloma (squamous or keratotic)
2. Schneiderian papilloma (inverted, fungiform, cylindrical)
3. Adenoma

BENIGN NON-EPITHELIAL
1. Fibroma
2. Osteoma
3. Chondroma
4. Hemangioma

MALIGNANT EPITHELIAL
1. SCC (most common)
2. Adenocarcinoma
3. Adenoid cystic
4. Olfactory neuroblastoma
5. Sinonasal undifferentiated carcinoma (SNUC)
6. Melanoma (Nasal > maxillary > ethmoid > frontal)

MALIGNANT NON-EPITHELIAL
1. Liposarcoma
2. Fibrosarcoma
3. Rhabdomyosarcoma
4. Leiomyosarcoma
5. Osteogenic sarcoma
6. Chondrosarcoma
7. Hemangiopericytoma
8. Neurogenic sarcoma
9. Lymphoma
10. Extramedullary plasmacytoma
11. Giant cell tumor

OTHER:
1. Metastasis (renal cell)
2. Infectious lesions

Non-epithelial tissues are: Fat, fibrous, muscle, bone, cartilage, vessels, nerve, and white cells

Epithelial tissues are anything that covers body surfaces, mucosa, glands, etc

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3
Q

√Regarding sinonasal SCC, discuss:
1. What is the epidemiology?
2. What is the rate of occurence in each sinus?
3. Incidence of regional and distant metastasis?
4. Location of regional lymph nodes?
5. Treatment?
6. Prognosis?

A

EPIDEMIOLOGY:
- Most common sinonasal malignancy (85%)
- Incidence of regional mets = 10%
- Incidence of distant mets = 18%

RATE OF OCCURRENCE:
1. Maxillary (70-75%)
2. Ethmoid (20%)
3. Sphenoid (3%)
4. Frontal (1%)

LOCATION OF REGIONAL LN:
1. Facial
2. Parotid
3. Submandibular
4. Retropharyngeal

TREATMENT:
1. Surgical resection ± Chemo/XRT

PROGNOSIS:
1. DFS 50% at 5 years

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4
Q

√Regarding sinonasal adenocarcinoma, discuss:
1. Epidemiology
2. Subtypes
3. Differentiate low vs. high grade

A

EPIDEMIOLOGY:
- Second most common sinonasal malignancy
- Male predominance 6:1
- 5th-6th decade
- Usually arises in the middle meatus/ethmoids

SUBTYPES:
1. Papillary
2. Sessile
3. Alveolar mucoid
4. Intestinal/colonic (most common)
5. Mucinous
6. Low-grade
7. Mixed

LOW GRADE:
- Uniform glandular architecture

HIGH GRADE:
- Solid
- ++ mitosis
- 1/3 distant metastasis

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5
Q

√Regarding sinonasal adenoid cystic carcinoma, discuss:
1. Epidemiology
2. Pathology types. What is the histopathology of each?
3. Treatment
4. Prognosis

A
  • 10% of all salivary gland tumors
  • Arise from minor salivary glands in PNS
  • 5 to 15% of tumors in the PNS

Require long term follow up as can present with very
late (20 years) recurrence
- Propensity for perineural spread
- Neck mets rare

Path: tubular, cribriform, solid
- < 30% solid component = low grade
- >30% solid component = high grade

Treatment: Aggressive surgical resection and postop XRT

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6
Q

√Regarding Sinonasal Undifferentiated Carcinoma (SNUC), discuss:
1. Epidemiology
2. Incidence of regional and distant metastasis
3. Treatment
4. Prognosis

A

EPIDEMIOLOGY:
- Rare aggressive malignancy
- Commonly affecting the anterior skull base
- Incidence of regional mets = 20%
- Incidence of distant mets = 10%

TREATMENT:
1. Surgical resection + Chemo-XRT

PROGNOSIS:
1. Disease free survival up to 29% at 2 years, 9% long term

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7
Q

√Regarding Esthesioneuroblastoma/Olfactory Neuroblastoma, diiscuss:
1. Rates of metastasis
2. Imaging findings
3. Treatment

A

RATES OF METASTASIS:
- Metastasis rare at presentation
- Regional and distant mets may arise in 15-30%

IMAGING:
1. MRI: Iso or hypointense to muscle and mucosa on T1 images; heterogeneously hyperintense on T2 images

TREATMENT:
1. Craniofacial resection + adjuvant RT (= bifrontal craniotomy and lateral rhinotomy)

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8
Q

√Describe the Histopathology of Esthesioneuroblastoma (Olfactory Neuroblastoma). 6

A
  • Sheets of small round blue cells, scant cytoplasm and hyperchromatic nuclei
  • Neurofibrils
  • Flexner-Wintersteiner Rosettes (lumen)
  • Homer-Wright Pseudorosettes (no actual lumen)
  • Neurosecretory Granules on electron microscopy
  • Immunohistochemistry typically positive for neural markers: Neuron Specific Enolase (NSE most consistent), S100, Synaptophysin, ± Chromogranin
  • Grade based on Hyam’s histologic grading system (4 grades)

Rosettes = rose shaped, round assemblage of cells, cells that make a circle or “halo” surrounding a central lumen (or not)

https://www.pathologyoutlines.com/topic/nasalolfactoryneuroblastoma.html

Vancouver Page 97

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9
Q

√Outline the Kadish staging for Esthesioneuroblastoma and 5-year survivals for each stage

A

Kadish staging has the best prognosticating power for esthesioblastoma

A: Confined to nasal cavity (85%)
B: Extension into paranasal sinuses (70%)
C: Spread beyond nasal cavity and sinuses - e.g. orbit, skull base, intracranial, neck (45%)
D: Cervical or distant metastasis (Morita modification) - 10%

Kilty says Hyams

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10
Q

√What are different staging systems for staging Esthesioneuroblastoma?

A
  1. Kadish (best prognosticating power)
  2. UCLA (Modified biller staging)
  3. Biller staging
  4. Hyams
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11
Q

√Define Ohngren’s line

A
  • Line between medial canthus and angle of mandible that divides maxillary sinus
  • Used for staging purposes
  • Prognosis is poorer if posterosuperior to this line (suprastructure)

Vancouver Page 97

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12
Q

Outline the AJCC T-staging for Maxillary sinus cancer

A

Mucosal melanoma is not included

TX: Primary tumor cannot be assessed

Tis: Carcinoma in situ

T1: Tumor limited to maxillary sinus mucosa with no erosion or destruction of bone

T2: Tumor causing bone erosion or destruction including extension into the hard palate and/or middle nasal meatus, except extension to posterio wall of maxillary sinus and pterygoid plates

T3: Tumor invades any of the following: bone of the posterior wall of maxillary sinus, subcutaneous tissues, floor or medial wall of orbit, pterygoid fossa, ethmoid sinuses

T4: Moderately advanced or very advanced local disease

T4a: Moderate advanced local disease
- Tumor invades anterior orbital contents, skin of cheek, pterygoid plates, infratemporal fossa, cribriform plate, sphenoid or frontal sinuses

T4b: Very advanced local disease
- Tumor invades any of the following: orbital apex, dura, brain, middle cranial fossa, cranial nerves other than maxillary division of trigeminal nerve (V2), nasopharynx, or clivus

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13
Q

Outline the AJCC T-staging for Nasal Cavity and Ethmoid sinus cancer

A

Mucosal melanoma is not included

TX: Primary tumor cannot be assessed

Tis: Carcinoma in situ

T1: Tumor restricted to any one subsite, with or without bony invasion

T2: Tumor invading two subsites in a single region or extending to involve an adjacent region within the nasoethmoidal complex, with or without bony invasion

T3: Tumor extends to invade the medial wall or floor of the orbit, maxillary sinus, palate, or cribriform plate

T4: Moderate advanced or very advanced local disease

T4a: Moderately advanced local disease
- Tumor invades any of the following: anterior orbital contents, skin of nose or cheek, minimal extension to anterior cranial fossa, pterygoid plates, sphenoid or frontal sinuses

T4b: Very advanced local disease
- Tumor invades any of the following: orbital apex, dura, brain, middle cranial fossa, cranial nerves other than V2, nasopharynx, or clivus

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14
Q

√List the approaches to sinonasal malignancy resection 7

A

A. TRANSFACIAL OPEN APPROACHES
1. Lateral Rhinotomy
2. Midface degloving
3. Facial translocations
4. Transpalatal approach
5. Weber-Ferguson (lateral rhinotomy with an upper lip split)
6. Infratemporal fossa approach
7. Combined craniofacial approach

B. CRANIAL APPROACHES
1. Wide frontal craniotomy
2. Narrow frontal craniotomy
3. Subfrontal craniotomy

https://media.springernature.com/lw685/springer-static/image/chp%3A10.1007%2F978-3-031-22483-6_51-1/MediaObjects/533579_1_En_51-1_Fig3_HTML.png

Hussain lecture image

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15
Q

√What are the different resection types for sinonasal malignancy? 7

A
  1. Medial maxillectomy
  2. Inferior maxillectomy (infrastructure maxillectomy)
  3. Total maxillectomy
  4. Suprastructure maxillectomy
  5. Subtotal maxillectomy
  6. Radical maxillectomy - total + orbital exenteration
  7. Craniofacial resection
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16
Q

√What are 6 absolute contraindications and 2 relative contraindications for craniofacial resection?

A

ABSOLUTE: (T4b)
1. Medical or nutritionally unfit
2. Distant metastasis
3. Prevertebral fascia invasion
4. Invasion of optic chiasm or both optic nerves or frontal lobe
5. Cavernous sinus invasion by high grade lesion
6. Carotid invasion in a high-risk patient

RELATIVE:
1. Dural invasion
2. Intracranial nerve involvement by adenoid cystic

17
Q

√What are 4 ways that sinonasal cancer is considered unresectable?

A
  1. Superior: Frontal lobe involvement
  2. Lateral: Cavernous sinus involvement
  3. Posterior: Prevertebral fascia involvement
  4. Posterior: Bilateral optic nerve involvement/optic chiasm
18
Q

√What are the indications for an open medial maxillectomy? What are the bony cuts that are made?

A

INDICATIONS:
1. Involvement of the lateral nasal wall with no extensive maxillary sinus involvement

Incision: via lateral rhinotomy incision

BONY CUTS:
1. Lateral: Medial to infraorbital foramen
2. Inferiorly: Can take orbital floor and lateral nasal wall to the inferior alveolus (taking the inferior turbinate)
3. Medially: Lamina papyracea and along lateral nasal bone
4. Anterior: Through lacrimal bone
5. Posterior: Along posterior wall of maxillar sinus

Vancouver Pg 99

19
Q

√What are the indications 2 of a total maxillectomy? What are the incisions and bony cuts that are made?

A

INDICATIONS:
1. Tumors originating from maxillary sinus
2. Tumors with significant extension into the maxillary sinus

Incisions: Weber Ferguson incision ± subciliary incision

BONY CUTS:
1. Laterally: along orbital floor, zygoma
2. Medially: Along nasal bone, around pyriform aperture
3. Hard palate
4. Posteriorly ± including pterygoid plates

Vancouver Pg 99

20
Q

√Outline the Okay/Urken classification of maxillectomy defects, and the possible reconstructive options for each

A

OKAY/URKEN Classification (most common)

  1. Class IA - hard palate (but not the tooth-bearing alveolus) with no teeth involved
    - Obturator best option
    - Skin only free flap vs. Local island flap
  2. Class IB - premaxilla only or teeth (maxillary alveolus) posterior to canine
    - Obturator - will still work very well
    - Skin only free flap (usually RFFF)
  3. Class II - alveolar defect involving one canine, or transverse < 50% (alveolar transverse palatectomy)
    - Obturator less of an option, not enough teeth to clasp onto
    - Bone/skin free flap (fibula, scapula)
    - Occ muscle and skin free flap (Rectus)
  4. Class III - alveolar defect with removal of both canines, or transverse > 50%, or total palatectomy
    - No chance for obturator
    - Bone and skin free flap
    - If orbital floor removed, Orbital floor reconstruction with bone from free flap, or non-vascularized bone and a muscle free flap (e.g. rectus)

Vancouver Pg 99

21
Q

√Describe the epithelium of the nasopharynx

A
  1. Pseudostratified columnar majority at birth, slowly replaced by stratified squamous in adolescence
  2. Transitional cell - found at junction of the two epithelial linings
22
Q

√What are the borders of the fossa of rossenmuller? What is the importance of this?

A

Fossa of Rosenmuller = Lateral pharyngeal recess; Superior most side of the lateral surface of the nasopharynx

Borders:
1. Superior: Cavernous sinus and foramen lacerum (ICA - cavernous sinus)
2. Lateral: Sinus of morgagni
3. Anterior: Foramen Ovale
4. Posterior: Jugular Foramen
5. Inferior: Parapharyngeal space

Importance:
- Location where 3 different fascial planes converge - easy area for tumor spread
- Apex of parapharyngeal space: pre-styloid, post-styloid, and retropharygneal fascias

23
Q

√Describe the different histologies and their clinical features/prognosis of Nasopharyngeal Carcinoma

A

A. KERATINIZING SQUAMOUS CELL CARCINOMA (WHO Type 1)
- 25% in non-endemic areas
- 1-2% in endemic areas
- Distinct intercellular bridges
- Keratin production
- Worst prognosis = 10% 5-year survival

B. NON-KERATINIZING SQUAMOUS CELL CARCINOMA
1. Differentiated (WHO Type 2)
- 2% in non-endemic areas
- Little/no keratinization present
- Papillary morphology
- ~60% 5-year survival

  1. Undifferentiated (WHO Type 3)
    - Strong EBV association
    - Lymphoepithelioma
    - 63% in non-endemic areas
    - 95% in endemic areas
    - Clear, enlarged nuclei
    - ~60% 5-year survival

C. BASALOID SQUAMOUS CELL CARCINOMA
- Rare, aggressive clinical course, poor survival

24
Q

√What are 4 main risk factors for nasopharyngeal carcinoma?

A

A. GENETICS:
1. HLA-A2
2. B17
3. Bw46 (only in asians)

B. GEOGRAPHIC:
1. Southeast asia (Kwantung/Guangdong, Hong kong, taiwan)
2. Inuit
3. North Africa

C. ENVIRONMENTAL:
1. Nitrosamines
2. Salted preserved foods
3. Chemical fumes
4. Wood dust

D. INFECTIOUS:
1. EBV (inactivates tumor suppressor genes and increases cell signalling cascades)

25
Q

√What are the top 8 signs and symptoms of nasopharyngeal carcinoma?

A
  1. Neck mass (60%)
  2. Ear plugging/fullness (41%)
  3. Conductive hearing loss (37%)
  4. Epistaxis (30%)
  5. Nasal obstruction (29%)
  6. Cranial nerve palsy (18%) - usually VI, or V initially; if III or IV - think cavernous sinus involvement (in order: VI, III, V1, V2, IV)
  7. Decreased soft palate movement
  8. Mandibular nerve neuralgia
26
Q

√What is Trotter’s triad?

A

Conductive hearing loss, due to MEE, from Nasopharyngea, carcinoma

27
Q

√What role do EBV autoantibodies play in nasopharyngeal carcinoma diagnosis and therapy?

A

Diagnosis: Work-up of NPC may include EBV testing of the tumor and serum, particularly in the presence of nonkeratinizing and undifferentiated histology

TUMOR:
- EBV detected in tumor tissue
- In-situ hybridization for EBV-encoded-RNA (EBER)
- Immuno-histochemical staining for LMP1

SERUM:
- PCR for DNA viral load
- Persistently elevated levels of viral DNA near or at the end of radiation therapy are associated with a significantly poorer outcome
- EBV DNA load ≥ 4000 pre-treatment = high risk of distal metastasis

28
Q

√What are the sites and subsites of the nasopharynx? 4

A
  • Posterosuperior wall: extends from the level of the hard and soft palates to the base of the skull
  • Lateral wall: Including the fossa of Rosenmuller, ET, pharyngobasilar fascia, superior constrictor
  • Inferior wall: Consists of the superior surface of the soft palate
  • Anterior: Choanae
29
Q

√What are the most common patterns of spread for nasopharyngeal carcinoma? 4

A
  • Superior - foramen lacerum - cavernous sinus (III/IV/V1-2/VI)
  • Anterior - Foramen Rotundum and Ovale - V2/V3
  • Posterior - Jugular Foramen (IX-XI)
  • Submucosal extension
30
Q

√Describe the AJCC 8th edition TNM staging for Nasopharyngeal Carcinoma

A

TUMOR:
- Tx: Primary tumor cannot be assessed
- T0: No tumor identified, but EBV-positive cervical node involvement
- Tis: Carcinoma in situ
- T1: Confined to nasopharynx, or extension to oropharynx and/or nasal cavity without parapharyngeal involvement
- T2: Extension to parapharyngeal space, and/or adjacent soft tissue involvement (medial pterygoid, lateral pterygoid, prevertebral muscles)
- T3: Infiltration of bony structures at skull base, cervical vertebra, ptergyoid structures, and/or paranasal sinuses
- T4: Intracranial extension, involvement of cranial nerves, hypopharynx, orbit, parotid gland, and/or extensive soft tissue infiltration beyond the lateral surface of the lateral pterygoid muscle

NODES:
- NX: Cannot be assessed
- N0: No regional LN
- N1: Unilateral cervical mets, and/or unilateral or bilateral mets in retropharyngeal LN, ≤6cm in greatest dimention, above caudal border of cricoid cartilage
- N2: Bilateral cervical mets, ≤6cm, above caudal border of cricoid
- N3: Unilateral or bilateral cervical mets >6cm, and/or extension below the caudal border of cricoid

DISTANT METS:
- M0: No distant metastasis
- M1: Distant metastasis

STAGING GROUPS:

Stage 0: Tis, N0, M0

Stage I: T1N0M0

Stage 2:
- T0/1, N1, M0
- T2, N0/1, M0

Stage 3:
- T0/1/2, N2, M0
- T3, N0/1/2, M0

Stage 4a:
- T4, N0/1/2, M0
- Any T, N3, M0

Stage 4b:
- Any T, Any N, M1

31
Q

√What is HO’s line?

A

The line from the lateral border of the trapezius insertion on the occiput - to the attachment of the sternum where the clavicle is.

  • Separates I-II-III from IV/V neck levels
  • Nodes below this line = poor prognosis
32
Q

√What is HO’s prognostic criteria scoring for nasopharyngeal carcinoma? 9

A

Higher score is poorer prognosis

1) Number of symptoms > 7
2) Duration of symptoms > 2 months
3) Extent of tumor (T score, beyond nasopharynx)
4) Low level of LN (IV/V)
5) Presence of metastasis
6) Histologic grade - Type 1 is bad
7) ADCC < 1:7680 or < 1:960 (antibody-dependent cellular cytotoxicity)
8) Age ≥ 40 years old
9) Low Karnofsky score

https://journals-sagepub-com.proxy.bib.uottawa.ca/doi/epdf/10.1177/000348948509400601

33
Q

√What are the Ho and Neel’s 5 adverse prognostic indicators for Nasopharyngeal carcinoma?

A
  1. Length and symptomatology of disease (>7 symptoms, >2 months)
  2. Extension beyond the nasopharynx
  3. Low neck disease (Levels IV/V)
  4. Keratinizing histology
  5. Distant metastasis (3%)

“NPC’ed”

34
Q

√How does the workup of nasopharyngeal cancer differ from other head and neck cancers? 2

A

The only site where:
1. MRI with contrast of skull base to clavicle is recommended
2. EBV testing should be considered
3. Only subsite treated with primary XRT

35
Q

√Describe the genearl treatment for nasopharyngeal carcinoma based on overall stage

A

Stage 1 & 2: RT
Stage 3 & 4: concurrent ChemoRT ± adjuvant chemo

36
Q

√How does concurrent chemo-RT regimen with cisplatin differ for advanced nasopharyngeal carcinoma compared to others?

A
  • The one location in H/N where concurrent chemo-RT followed by adjuvant chemo is used in some centres
  • The benefit of this strategy is at present uncertain (negative studies) and toxicity is substantial, but listed as an option under NCCN
  • Recommendations is for cisplatin + RT, followed by cisplatin + 5FU (adjuvant)
  • Induction chemo (Gemcitabine/cisplatin) sometimes used for advanced disease
37
Q

√What is the overall survival for nasopharyngeal carcinoma post-radiotherapy?

A
  • In general, 5-year survival ~60% (Worse for Type I, better for Type II/III)
  • Survival continues to decrease past 10 years
  • Pediatric nasopharyngeal carcinoma - ~40% 5-year overall survival
38
Q

√What are 8 complications of XRT treatment of nasopharyngeal carcinoma?

A
  1. Xerostomia
  2. Eustachian tube dysfunction
  3. Dental caries
  4. Trismus
  5. VPI
  6. ORN of skull base or mandible
  7. Hypopituitary
  8. Carotid blow out
39
Q

√What are treatment options for locally recurrent Nasopharyngeal carcinoma (T1N0M0)

A

Controversial topic

Options:
A. Surgical salvage
1. Open: maxillary swing
2. Endoscopic

B. Re-irradiation
1. Salvage IMRT
2. Stereotactic radiosurgery
3. Proton beam
4. Conventional re-radiation
5. Brachytherapy

For previously treated, recurrent or metastatic nasopharyngeal carcinoma, anti-PD1 (Pembrolizumab) can be considered