Neuro: Head and Neck

Question 1

• White patch or plaque, can’t be scraped off
• Gross: single or multiple, sharply demarcated, flat or raised, white patch or plaque
• Can’t be characterized as any other disease
• A/w Tobacco, Alcohol, and Ill-fitting dentures
• Any age, but usually 40-70 y/o
• Buccal mucosa, Floor of mouth, Ventral tongue, Palate, Gingiva
• ~80% are non-neoplastic; ~15% dysplastic; ~3% cancer
• Hyperkeratosis

Answer 1

Leukoplakia

Question 2

• Red patch or Plaque
• Can’t be Scraped off
• Can’t be characterized as any other disease
• 90% are pre-malignant or malignant
• Severe dysplasia, Carcinoma in situ, or Minimally-invasive SCC

Answer 2

Erythroplakia

Question 3

• Slowly developing Scaly, White-gray Plaque
• Fissuring or Cracking of the mucosa, typically at the corners of the mouth
• A/w Sun Exposure

Answer 3

Actinic Cheilitis

‘Actinic Keratosis’

Question 4

• Small Round Blue cell Tumor
• Arises from Neuroectodermal Olfactory cells
• Expresses neuroendocrine markers (chromogranin, synaptophysin, CD56)
• Bimodal age: Peaks at 15 & 50 y/o
• Nasal obstruction or Epistaxis
• Tx: Surgery + XRT + Chemo
• Prognosis: (5-year survival)
  
  • Confined to nasal cavity & fully resected → 75%
  • Extension beyond paranasal sinus → 40%

Answer 4

Esthesioneuroblastoma

(Olfactory Neuroblastoma)

Answer 5

Lymphoma

Question 6

• Benign tumor of the Nasal mucosa composed of Large Blood Vessels and Fibrous Tissue
• Classically seen in Adolescent males only (Fair skinned and Red-headed)
• Presents w/ Profuse Epistaxis (Nosebleeds) and Obstruction
• Posterior lateral wall of Nasal cavity, near roof
• Tx: Surgery

Answer 6

Nasopharyngeal Angiofibroma

Question 7

• Arise from & surface lined by respiratory mucosa
• M>F; typically 30-60 y/o
• Nasal cavity or paranasal sinuses
• (3) Types:
  
  • Exophytic / Fungating: Most Common type; **HPV 6 & 11 **
  • Endophytic / Inverted: Most Aggressive type; **HPV 6 & 11 **(Benign but locally aggressive) “gopher holes filled with squamous cells”
  • **Cylindrical **

Answer 7

Schneiderian Papilloma (Sinonasal)

Question 8

• Most occur in Adrenal medulla → **Pheochromocytoma **
• Aorticopulmonary parasympathetic chain (near great vessels of the neck) - have a “Bland” appearance but metastasize
• Zellballen pattern: Balls of Round to Oval cells (chief cells) w/ abundant Pink, Granular cytoplasm
• S-100 positive Sustentacular cells, Chromogranin
• Carotid Body: most common location
• Sporadic: Solitary; slow-growing; painless; 5th to 6th decade
• Familial: MEN 2 Syndrome, multiple; earlier
• Tx: Surgery
• Can metastasize and up to 50% are fatal

Answer 8

Paraganglioma

Question 9

• One of the most common diseases worldwide
• # 1 cause of tooth loss in patients < 35 y/o
• Pathogenesis: poor oral hygiene
  
  • Bacteria + Acidic product + Teeth = Deminiralization

Answer 9

Dental Caries

Question 10

• Inflammation of Oral Mucosa surrounding Teeth
• Most Prevalent and Severe in Adolescence
• Pathogenesis: poor oral hygiene
  
  • Accumulation of Dental Plaque
  • Mineralization
  • Tartar formation (calculus)

Answer 10

Gingivitis

Question 11

• Inflammation of supporting structures of teeth (ligaments, alveolar bone and cement)
• Can lead to infective Endocarditis and Abscesses
• A/w Actinobacillus
• Pathogenesis: poor oral hygiene & alteration of flora
  
  • Normal Facultive Gram Positive Organisms
  • Replaced by Anaerobic and Microaerophilic Gram Negatives
• ​​Tx: Fluoride and Hygiene

Answer 11

Periodontitis

Question 12

• **Painful, **superficial Ulceration of the Oral mucosa due to Stress: “Canker-sores”
• Arises in relation to Stress and Resolves Spontaneously, but often recurs
• A/w a mononuclear Infiltrate
• Characterized by a Grayish Base (granulation tissue) surrounded by Erythema

Answer 12

Apthous Ulcer

Question 13

• AKA “traumatic fibroma”
• Buccal mucosa (along bite line) or on the gingiva
• Caused by repetitive Trauma
• Tx: simple Excision curative

Answer 13

Irritation Fibroma

Question 14

• Gingiva of children, young adults & pregnant woman
• May grow rapidly; most eventually regress
• Gross: ulcerated, dark red nodule on gingival mucosa
• Histo: consists of highly vascular, granulation tissue

Answer 14

Pyogenic Granuloma

Question 15

• Vesicles involving Oral mucosa that Rupture, resulting in Shallow, Painful, **Red ulcers, **“cold sores”
• Usually HSV-1 (above the belt), HSV-2 (below the belt)
• Primary infection occurs in Childhood; Lesions heal, but virus remains dormant in the Ganglia of the Trigeminal never (CN V)
• Stress and Sunlight cause reactivation of the virus –> Leads to Vesicles that often arise on the lips

Answer 15

Oral HSV Infection

Question 16

• White membrane overlying **Erythematous base **
• Necrotic squamous epithelium w/ yeast & hyphae overlying an inflamed submucosa
• Often seen in Immunocompromised individuals as a Yellowish Plaquelike area
• Candida Albicans: normal part of oral flora in 50%
• Most common Fungal Infection in oral cavity
• White Lesion that can be Scraped off

Answer 16

Oral Candidiasis (Thrush)

Question 17

• Immunocompromised patients
• Usually on Lateral border of Tongue
• Caused by Epstein-Barr Virus (EBV)
• Confluent, fluffy white patches that can’t be Scraped off
• Hyperparakeratosis, Acanthosis, Balloon cells

Answer 17

Hairy Leukoplakia

Question 18

• Protrusion of Edematous, Inflamed Nasal mucosa
• Usually Secondary to repeated bouts of Rhinitis
• A/w Allergic rhinitis, Type 1 Hypersensitivity –> “Hay Fever” –> HIGH IgE lvls in the Serum
• A/w Cystic fibrosis and Aspirin-Intoleratnt-Asthma
• ASA-intolerant characterized by the triad of Asthma, Aspirin-induced Bronchospasms, and Nasal polyps (10% asthmatic kids)
• A consequence of repeated bouts of Rhinitis

Answer 18

Nasal Polyp

Question 19

• Benign neoplasm on True vocal cord
• Caused by HPV types 6 & 11
• May recur but DOES NOT become Malignant
• Usually Single in Adults; often Multiple in Children
• Often spontaneously Regress at Puberty

Answer 19

Squamous Papilloma

Question 20

• Nodule that arises on the True vocal cord
• Due to Excessive use of vocal cords; usually Bilateral
• Produces “Reactive nodules”
• Composed of Degenerative (Myxoid) Connective Tissue
• Wear and Tear issue and resolves with Rest of Voice

Answer 20

Singer’s Nodules

Question 21

• Anterior Lateral (along sternocleidomastoid muscle)
• Lymphoid tissue with prominent germinal centers
• Lymphoepithelial cysts
• Remnants of an Embryonic Branchial arch or Salivary gland inclusion in a Cervical lymph node - absence of thyroid tissue
• Tx: Surgical Cystectomy

Answer 21

Branchial Cleft Cyst

Question 22

• Cyst of the Anterior Midline (foramen cecum to middle of neck)
• Thyroid tissue
• Tx: Cystectomy plus middle 3rd of hyoid bone

Answer 22

Thyroglossal Duct Cyst

Question 23

• Inflammation of the Nasal Mucosa (resp. epithelium)
• Adenovirus is the most common cause
• Presents w/ Sneezing, Congestion, and Runny nose (common cold)
• Allergic subtype is due to Type I Hypersensitivity rxn
  
  • Inflammation Infiltrate w/ Eosinophils
  • A/w Asthma and Eczema
• Repeated bouts can lead to Nasal polyps

Answer 23

Rhinitis

Question 24

• Extremely common; “Sore throat”
• Edematous, Congested nasopharyngeal mucosa (+/- exudate)
• Viral culprits a/w Rhinitis, plus RSV, Influenza, and bacteria
• Rule out Group A, β-hemolytic strep to avoid complications: (Rheumatic Fever & Post-Strep Glomerulonephritis)
• Rapid Strep Antigen test +/- Culture

Answer 24

Pharynginitis

Question 25

• Inflammation of the Upper Airway
• Parainfluenza virus is the most common cause
• Presents w/ Hoarse, ‘Barking cough’ and Inspiratory stridor

Answer 25

Laryngotracheo Bronchitis (‘Croup’)

Question 26

• Inflammation of the Epiglottis of the Larynx
• H influenzae Type B is the most common; in both Immunized and Non-immunized children
• High fever, Sore throat, Drooling w/ Dysphagia, Muffled voice, and Inspiratory Stridor (airway compromise)
• Risk of Airway obstruction –> Medical emergency

Answer 26

Acute Epiglottitis

Question 27

• Malignant tumor of Nasopharyngeal Epithelium
• A/w EBV
• Classically seen in African Children and Chinese Adults (young)
• Biopsy usually reveals Pleomorphic Keratin-positive** epithelial cells (poorly differentiated SCC) ** in a background ofLymphocytes
• Often presents w/ involvement of Cervical Lymph nodes
• (3) Histologic variants:
  • Keratinizing: well-diff SCC; worst prognosis
  • Non-Keratinizing: poorly-diff SCC
  • Undifferentiated: syncytium of cells w/ lymphoid background; best prognosis; radiosensitive

Answer 27

Nasopharyngeal Carcinoma

Question 28

• Full-thickness defect of Lip and Palate
• Due to Failure of Facial Prominences to Fuse
• During early pregnancy, Facial prominences (One from Superior, Two from the Sides, Two from Inferior) grow and fuse together to form the face (5 total)
• Lip and Palate usually occur together, isolated version is less common

Answer 28

Cleft Lip and Palate

Question 29

• Recurrent Aphtous ulcers, Genital ulcers and Uveitis (visual disturbances)
• Due to Immune complex Vasculitis involving Small Blood Vessels
• Can be seen after Viral Infection, but Etiology is unknown

Answer 29

Behcet Syndrome

Question 30

• Malignant neoplasm of the Oral mucosa
• Tobacco and ** Alcohol** are major Risk Factors
• Floor of Mouth is most common
• Desmosomes and Keratin
• Oral Leukoplakia and Erythroplakia are Precursor Lesions
• Leukoplakia vs. Oral Candidiasis = is a white deposition which is easily scraped away, usually a/w immunocompromised states
• Hairy leukoplakia is white, rough ‘hairy’ patch that arises on the Lateral tongue - AIDS, EBV-induced SCHyperplasia - not pre-maliginant

Answer 30

Squamous Cell Carcinoma

Answer 31

Pyogenic Granuloma

Answer 32

Erythroplakia

Question 33

• Congenital, Non-inherited (somatic), Developmental anomaly of Neural Crest derivatives (Mesoderm / Ectoderm) due to activating mutation of GNAQ gene
• Affects small (capillary-sized) blood vessels
• Port-wine stain of the Face (non-neoplastic “birthmark” in CN V₁, V₂ Distribution)
• Ipsilateral Leptomeningeal Angioma –> Seizures / Epilepsy
• Intellectual Disability and Episcleral Hemangioma –> Increase IOP –> Early-onse Glaucoma

Answer 33

Sturge-Weber Syndrome

• SSTURGGE-Weber
  
  • Sporadic, port-wine Stain
  • Tram track Ca²⁺ (opposing gyri)
  • Unilateral
  • Retardation
  • Glaucoma, GNAQ gene
  • Epilepsy

Question 34

• HAMARTOMAS-S
• Hamartomas in CNS and Skin
• Angiofibromas
• Mitral regurgitation
• Ash-leaf spots
• Rhabdomyoma - cardiac
• Tuberous sclerosis
• autosomal dOminant
• Mental retardation
• Angiomyolipoma - renal
• Seizures, Shagreen patches
• Increase Incidence of Subependymal Astrocytomas and Ungual Fibromas

Answer 34

Tuberous Sclerosis

Question 35

• Cafe-au-lait spots
• Lisch nodules (pigmented iris hamartomas)
• Neurofibromas in Skin, Optic gliomas, Pheochromocytomas (PCC)
• Mutated NF-1 tumor suppressor gene (neurofibromin, a negative regulator of Ras) on Chrom. 17
• Skin tumors of NF-1 are derived from Neural crest cells

Answer 35

Neurofibromatosis Type 1

(von Recklinghausen Disease)

Question 36

• Cavernous Hemangiomas in Skin, Mucosa, Organs, Bilateral Renal Cell Carcinoma
• Hemangioblastoma (High vascularity w/ Hyperchromatic nuclei) in Retina, Brain stem, Cerebellum and Pheochromocytomas (PCC medulla of Adrenal Glands)
• Autosomal Dominant
• Mutated VHL tumor suppressor gene on Chrom. 3
• Results in Constitutive expression of HIF (Transcription factor) and activation of Angiogenic growth factors

Answer 36

von Hippel-Lindau Disease