Head and Neck Pathology (2)- Gomez Flashcards
Squamous Papillomas
(verruca vulgaris/wart of nasal vestibule)
Arises in squamous mucosa
More common than sinonasal (Schneiderian) tumors
Schneiderian Papillomas
Benign neoplasms (but locally destructive)
Derived from embryologic Schneiderian membrane-like epithelium
Located in sinonasal tract (nasal Schneiderian mucosa)
Three Types
Exophytic (septal, squamous) ~50-60%
Inverted (lateral, squamous) ~40-50%
Oncocytic (lateral, cylindrical/columnar) ~5-10%
Symptoms: epistaxis, nasal obstruction, asymptomatic mass
Exophytic Sinonasal Papilloma
(Septal, Squamous, Fungiform)
Occurs on septal nasal wall (less than 10% lateral)
20 – 50 yr; 4-10M:1F
HPV 6/11 in ~60%
Recurrence: ~25% if not completely excised
Rarely (almost never) develops invasive carcinoma
Inverted Sinonasal Papilloma
Occurs on lateral nasal wall near middle turbinate or sinus
40 – 70 yr; 2-5M:1F
HPV 6/11 in ~40%
Recurrence: 15% even with aggressive surgery
5-10% develop invasive carcinoma within 5 years
Nests of proliferating squamous epithelium grow inward
Downward - Inverted
Oncocytic Sinonasal Papilloma
(Cylindrical, Columnar)
Occur on Lateral nasal wall near middle turbinate
>50 yr; 1M:1F
HPV Association: None
Recurrence: 25-35% (even after aggressive surgery)
Some may develop invasive carcinoma
Oncocyte = abundant bright pink cytoplasm (from mitochondria in this tumor)
Olfactory Neuroblastoma
(Esthesioneuroblastoma)
Arises from neuroectodermal olfactory cells in olfactory mucosa
Neurosecretory (membrane bound) granules by EM,
Numerous IHC markers may be positive (NSE, SYP, CgA, CD56 [NCAM])
Average age onset bimodal- 15 and 50 years of age (Range 3-90)
Often extensive polypoid mass, obstruction, epistaxis, anosmia, visual disturbance
Rx- surgery, radiation and chemotherapy
5-year Survival 40-90%, varying biologic activity
Pharynx – Three Major Divisions
Mucosae
Nasopharynx
60% NK squamous*
40% Respiratory epithelium
Oropharynx
100% NK squamous
Laryngopharynx
100% NK squamous
*NK = “non-keratinizing”
= no cornified layer
Misnomer -All squamous epithelium makes keratin
larynx
it is not part of the laryngopharynx). The larynx is covered variably by respiratory and squamous mucosae.
Upper “Airway” Lymphoid Structures
Exposure/surveillance environmental antigens
Diffuse submucosal lymphoid aggregates (nasal cavity)
Tonsils
Palatine tonsils - covered by squamous epithelium
Lingual tonsils - covered by squamous epithelium
Adenoids (pharyngeal tonsil) - covered by respiratory epithelium
Waldeyer’s “tonsillar” ring
Nasopharynx
Airway between nasal cavity (anterior-superior) and oro- & laryngopharynx (inferior)
Subject to same infectious & inflammatory (e.g., allergic) conditions that affect nasal mucosa
Obstruction of the internal auditory canal by hypertrophic adenoidal tissue
leads to
recurrent otitis media
Obstruction in upper airway
leads to
sleep apnea
Observed episodes of sleep apnea Snoring Difficult to arouse Daytime sleepiness Poor attention span Poor school performance
Pertussis / Whooping Cough - cause, vaccine
Bordetella pertussis
Extremely small gram-negative coccobacilli
Spread via respiratory droplets
Maximal in catarrhal stage (earliest stage)
secondary attack rate up to 80% in households
Vaccine - DTaP (diphtheria/tetanus/acellular pertussis)
Tdap (tetanus toxoid, low dose diphtheria toxoid and acellular pertussis) booster
Attaches to pharygneal and tracheal surfaces
Dx - Nasopharyngeal swab for culture & PCR or serology
Whooping Cough/Pertussis Stages
Stage 1 - Catarrhal phase
Indistinguishable from common upper respiratory infections.
Nasal congestion, rhinorrhea, and sneezing
Pertussis is most infectious when patients are in the catarrhal phase
Stage 2 - Paroxysmal phase
Paroxysms of intense coughing
Posttussive vomiting and turning red are common
Coughing occasionally followed by a loud whoop as inspired air goes through a still partially closed airway
Infants younger than 6 months do not have the characteristic whoop but may have apneic episodes
Stage 3 - Convalescent phase
Chronic cough which may last for weeks
Nasopharyngeal Angiofibroma (NA)
Epidemiology: RARE –
Occurs almost exclusively in young males (often redheads)***
onset 10-20 years (rare > 30); “Juvenile NA”
Symptoms: Unilateral nasal obstruction and epistaxis
can also include swelling of face, eye, cheek
Clinical Behavior: Posterolateral wall fibromuscular stroma origin
Benign, but 10-20% are locally aggressive and 9% are fatal
Tumor cells have androgen receptors and may resolve with age
Nasopharyngeal Angiofibroma (NA) treatment and prognosis
Treatment: Surgery, hemorrhagic complications not uncommon
- requires pre-op arteriogram with pre-surgical embolization
Prognosis: Excellent after removal; local recurrence rate of 5-25%
Prognosis depends on extent of resectability
Nasopharyngeal Carcinoma subtipes and epidemiology
Three histopathologic types
Keratinizing - squamous cell carcinoma (SqCC)
Nonkeratinizing - squamous cell carcinoma
Undifferentiated/basaloid carcinoma, with lymphoid component*
Epidemiology EBV-related (+EBER-1)** environment associations diet (nitrosamines), smoking ** Africa: Common in children (not adults) ** S. China: Common in adults (not children) USA: Rare Clinical Course Freq. unresectable at diagnosis [+metastases in 70%] Treat with radiotherapy Five year survival (after Rx) 60%
NUT Midline Carcinoma
Mostly mediastinum (35% head and neck
Any age or sex
Highly aggressive (median survival 7 mo.)
Appearance similar to nasopharyngeal and squamous cell carcinoma
BRD4/BRD3-NUT fusion gene (inhibitors being developed)
Pharynx – Oropharynx
Not really part of airway; represents
posterior portion of oral cavity
Acute tonsillitis, acute pharyngitis: “beefy red”
Most of these are due to Adenovirus (some HSV, EBV, cytomegalovirus, some bacterial- Streptococcal)
Group A Streptococcus (GAS)Typical Clinical Features
age 5-15 yrs
winter-early spring
Sore throat + fever
Strawberry tongue (red swollen), petechiae on palate, erythematous pharynx
Absence of cough, coryza, hoarseness, conjunctivitis
(these suggest a virus)
Tender anterior cervical lymph nodes
Tonsils are enlarged , erythematous and have patchy exudate (follicular tonsillitis)
Fusobacterium necrophorum
~10% acute pharyngitis cases
>20% in recurring cases and in peritonsillar abscesses
May cause associated abscesses especially peritonsillar abscesses
–Jugular vein with thrombophlebitis (Lemierre syndrome)
– Thrombi break off and seed to different sites
(Filamentous, anaerobic, Gram-negative rod)
Corynebacterium diphtheriae
strains carrying tox gene cause diphtheria
Gene encoded within a lysogenic bacteriophage
Diphtheria
Sudden onset of exudative pharyngitis that gets much worse over 3 days or so
Production of pseudomembrane
Viral Pharyngitis
Rhinoviruses (~20%) - Indirect pharyngitis. Grow in nasal mucous membranes and causes swelling of membranes in the area and pharynx.
Adenoviruses –> pharyngoconjunctival fever
EBV - Infectious mononucleosis - Monospot test for heterophile antibodies
HSV types I and 2 - Gingivitis, stomatitis and pharyngitis, vesicles
Influenza - Pharyngitis is a common component
Parainfluenza and coronaviruses
Enteroviruses (certain coxsackie and echovirus)
CMV and HIV- mononucleosis-type illness
Laryngopharynx
=Hypopharynx & Larynx
Laryngopharynx & true vocal cords have squamous epithelium, most everything else covered by respiratory epithelium
Epiglottitis = Laryngoepiglottitis
Swelling of epiglottis secondary to infections (most common), chemical, and traumatic agents
Complete blockage of the airway may occur (suffocation and death)
Airflow resistance increases
H. influenzae type b main cause prior to Hib vaccine where most patients were young (~2 yr) children
Pleomorphic Gram negative coccobacilli that colonize upper airways (predominantly encapsulated variants)
Also causes pneumonia, sinusitis, otitis media, meningitis, cellulitis
Also caused by RSV & β-hemolytic strep
Nowadays more common in adults (3M:1F), but pretty rare
Acute Laryngitis defn, symptoms, common causes
Definition: Laryngitis < 3 weeks
Symptoms
Hoarseness (gradual onset, progressive)
Decreased vocal volume
Painful speech
Common Causes Infections Vocal overuse: Acute – loud yelling Vocal overuse: Subacute – lecturing Heavy smoking (acute) Direct trauma
Uncommon Causes
Acute allergic reactions
Acid reflux from the stomach (GERD)
Infectious acute laryngitis
Clinical Course: abrupt onset, self-limited, less than 3 wk duration
Age of Onset: 3 – 5 yrs* & 18–40 yrs
Symptoms: progressive hoarseness (aphonia)
often concurrent upper respiratory tract infection (URI)
Laryngoscopy: vocal cords swollen and red
Etiology
Viruses > 90% cases: Rhinoviruses, Parainfluenza, RSV, Adenoviruses
Bacteria causes: H. influenzae, S. pneumonia
*In children may lead to life-threatening laryngoepiglottitis!
Croup / Laryngotracheitis / Laryngotracheobronchitis
inspiratory stridor
seal-like barking
mainly parainfluenza
Steeple sign- subglottic narrowing, absent in about half the kids
Pathogenesis
Infection via aerosol into nasopharynx and spread to larynx and trachea
Edema and inflammation in subglottic larynx and trachea around cricoid cartilage – airway narrowing. May have endothelial damage and loss of ciliary function. Fibrinous exudate may be formed and add to airway occlusion
Edema of vocal cords can cause hoarseness
Treatment
Supportive with short term steroids to reduce inflammation
Reinke Edema (Polypoid Corditis)
Usually occurs in middle-aged females who are heavy smokers
Can also occur with heavy, recurrent voice strain
Develop husky low-pitched weak voices
Soft, gelatinous translucent expansion of cord surfaces caused by edema and expansion of Reinke space (lamina propria of vocal mucosa)
Vocal Cord Nodules and Polyps
Pathogenesis: Reaction to injury of vocal cord
Hyperkeratosis
Increased myxoid stroma
Classic location at* junction anterior and middle third of cord*
Nodules - bilateral, small (~2-3 mm)
Polyp - unilateral, larger (~4-8mm)
Occurs following * sustained injury caused by
Heavy smoking
Heavy, recurrent voice strain (**singer’s nodules)
“They virtually never give rise to cancers”
Vocal Cord Papilloma and Papillomatosis
Papillomas: Benign neoplasms located on true vocal cords
Usually single in adults, but can be recurrent
Multiple in children (juvenile laryngeal papillomatosis)
Caused by HPV types 6/11
Squamous Cell Carcinoma of Larynx
Clinical Findings Prolonged hoarseness (> 6 wks) earliest, most consistent symptom
dysphagia, palpable cervical lymph nodes
Epidemiology
: most heavy smoking (> 50 pack years)
Ethanol abuse/dependence
Location of Laryngeal Carcinoma
- Glottic carcinoma: involves the true vocal folds
50-60% of laryngeal carcinomas - Supraglottic carcinoma: confined to the supraglottic area (free border of the laryngeal epiglottis, false vocal folds and laryngeal ventricles)
30-40% of laryngeal carcinomas
Discovered later– early tumors do not cause hoarseness
Higher stage tumors at diagnosis - 2/3 Stage III or IV - Subglottic carcinoma: extend or arise more than 10mm below the free margin of the true vocal fold up to the inferior border of the cricoid cartilage.
less than 5% of laryngeal carcinomas - Transglottic carcinoma: cross the ventricle from the supraglottic area to involve the true and false vocal folds or involve the glottis and extend subglottically more than 10 mm or both.Glottic tumors: 5 yr survival ~65%
Supraglottic tumors: 5 yr survival ~45%
Squamous Cell Carcinoma of the Larynx
- terminology of extent
Intrinsic - confined to larynx
Extrinsic - beyond the larynx
Transglottic carcinoma
- the large, ulcerated, fungating lesion involving the vocal cord and pyriform sinus.
Otitis Externa
Marked tenderness after gentle traction of pinna
Peak age between 7-12
Physical Findings: erythema, swelling, moist debris +/-pus
Etiology: Traumatized ear canal
excessive use cotton-tip swabs
retained contaminated water “Swimmer’s Ear”
Bacterial - 90%
Pseudomonas Sp - many
Fungal- 10% (aspergillus, candida)
ear neoplasms
Simply skin tumors; i.e. squamous and basal cell carcinoma
Middle Ear Anatomy
lined by thin “non-keratinizing” stratified squamous epithelium
ossicles: malleus, incus, stapes
Acute Otitis Media- what we’ll see
Tympanic membrane opacity, bulging , erythema, effusion and decreased motility
Chronic Otitis Media: causes and sequellae
Bacterial agents: Pseudomonas aeruginosa, S. aureus
Long-term sequellae: Perforation tympanic membrane, scarring, mastoiditis and bone erosion, cysts; conductive hearing loss common ***
ear-o? Aer-o and aur-o
Middle ear cysts
Two types of cyst lining
Squamous epithelium (cholesteatoma): large amounts keratin produced Metaplastic columnar epithelium: mucin-secreting
Cholesteatoma
Squamous epithelium trapped within the temporal bone (middle ear or mastoid)
Otosclerosis
Autosomal dominant, variable penetrance
Progressive ankylosis/immobilization over decades → severe conductive hearing loss
Pathologic process
Callus of bone accumulates at footplate of the stapes and rim of oval window
Neck - Branchial Cleft Defects
Sinus tracts Fistulas Cysts (lymphoepithelial cyst) Circumscribed ~2 - 5 cm 20-40 y.o.
*** Lateral neck
Most arise from 2nd branchial cleft
Thyroglossal Duct Cysts
Cyst in midline location
usually lined by respiratory or squamous epithelium and usually has chronic inflammation and thyroid follicles in the wall
Neck - Carotid Body Tumor
Prototype of parasympathetic tumor
Bruit on auscultation ***
Slow growing, painless mass, pulsatile
May metastasize to lymph nodes and distant
